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  • Prevalence of axial spondyloarthritis in Poland
    Rheumatol. Int. (IF 2.200) Pub Date : 2019-12-04
    Małgorzata Tłustochowicz, Melania Brzozowska, Waldemar Wierzba, Filip Raciborski, Brygida Kwiatkowska, Witold Tłustochowicz, Andrzej Jacyna, Michał Marczak, Bartłomiej Kisiel, Andrzej Śliwczyński

    The prevalence of axial spondyloarthritis (axSpA) in the published data varies significantly. Two types of axSpA can be distinguished depending upon the presence of abnormalities consistent with sacroiliitis on plain radiography: ankylosing spondylitis (AS) and nonradiographic axial SpA (nr-axSpA). The aim of this study is to perform a retrospective analysis of axSpA prevalence in Poland in the years 2008–2017. The National Health Fund (NHF) database for the period 2008–2017 was analysed. Data of all patients with the ICD-10 codes M46 (M46.1, M46.8, M46.9) or M45 (further named other inflammatory spondylopathies—OIS and AS, respectively) as the main or co-existing diagnosis were extracted and analysed. The AS prevalence was stable during the period under examination amounting to approximately 0.083%, while the OIS prevalence increased from 0.036 to 0.059%. For both men and women, the AS prevalence increased with age, reaching a maximum around the age of 70; however, in men, a marked increase in prevalence was observed earlier as compared to women (20–24 vs. 40–44 years, respectively). The OIS prevalence also increased with age; however, the maximum was reached earlier as in case of AS. Moreover, a sharp increase in OIS prevalence occurred earlier than in AS (15–19 years) with no difference between sexes. In Poland, approximately 0.1% of the population suffers from AS—the prevalence remained stable over the last decade. The prevalence of OIS increased markedly over the studied period which presumably reflects an increasing prevalence of nr-axSpA as the effect of the introduction of ASAS classification criteria for axSpA.

    更新日期:2020-01-14
  • Immunology and social networks: an approach towards impact assessment.
    Rheumatol. Int. (IF 2.200) Pub Date : 2019-10-14
    José Andrés Ordoñez-Gutiérrez,Juan Manuel Oviedo-Moreno,Daniela Patino-Hernandez,Daniel Gerardo Fernández-Ávila

    Scientific journals have changed the mechanisms they use for distribution and dissemination of information. Different approaches towards determining impact have emerged and among these, metrics derived from activity on social media are an emerging trend. This article aims to assess whether a correlation exists between the traditional impact factor and activity on social media. We assessed journals categorized within the area of "immunology" on the SCImago Journal and Country Rank website. Variables reflecting traditional and alternative measures of impact were collected. Differences between journals with and without social networks were assessed using non-parametric Mann-Whitney U tests. Correlation was assessed through Spearman tests. 156 journals were analyzed, 17% had at least one social network. 48.2% of journals with social networks were classified within SJR's quartile 1. An almost perfect correlation was found between the SJR and the number of followers on Twitter, this correlation remained statistically significant after adjusting for time since creation of the account [Spearman's correlation (rs) = 0.83]. We propose the use of Twitter as a mechanism for dissemination of information by immunology journals, as well as other social networks for their potential to increase their audience, as well as the dissemination and impact of their publications.

    更新日期:2020-01-14
  • Dilemma in management of hemorrhagic myositis in dermatomyositis
    Rheumatol. Int. (IF 2.200) Pub Date : 2019-12-23
    Julia M. Chandler, Yoo Jung Kim, Justin L. Bauer, Irene L. Wapnir

    Dermatomyositis (DM) is a rare inflammatory disorder affecting the muscle and skin. DM patients can present with spontaneous muscle hemorrhage, a potentially fatal complication. The best practice for management of hemorrhagic myositis in these patients remains unclear. Here we discuss the case of a patient who presented with progressive muscle weakness and intermittent rash that was diagnosed with dermatomyositis. During admission, she developed spontaneous hemorrhagic myositis of the right pectoralis major treated with surgical evacuation. She also developed a spontaneous left anterior thigh hematoma which was treated conservatively. She recovered and showed no evidence of recurrent bleeding at either location. We performed a literature review and identified ten cases of spontaneous hemorrhage in DM patients, with a 60% mortality rate among reported cases. Given the high mortality rate associated with spontaneous hemorrhage in DM patients, it is important for physicians to be aware of the diagnosis, workup, and management strategies.

    更新日期:2020-01-14
  • Profile of new referrals to a single pediatric rheumatology center in Turkey.
    Rheumatol. Int. (IF 2.200) Pub Date : null
    Şerife Gül Karadağ,Hafize Emine Sönmez,Ayşe Tanatar,Figen Çakmak,Mustafa Çakan,Nuray Aktay Ayaz

    To describe the demographic characteristics and clinical features of patients referred to a pediatric rheumatology outpatient clinic in Turkey and to compare the final diagnoses with the previous literature data. All new patients referred to pediatric rheumatology outpatient clinic of Kanuni Sultan Süleyman Research and Training Hospital between March 2018 and March 2019 were enrolled to the study. Demographic data, referral patterns, disease related features, physical examination findings and final diagnoses of new referrals were collected prospectively. A total of 2982 new referrals were evaluated in 1-year period. Among them 1561 (52%) had a diagnosis of a rheumatic disease. The frequencies of most common rheumatic diseases were; periodic fever syndromes (47.3%), juvenile idiopathic arthritis (18%) and vasculitis (14.4%), respectively. Non-rheumatic conditions were diagnosed in 1243 patients, among them orthopedic/mechanic problems (27.4%) were the most frequent ones followed by vitamin D deficiency (17.5%) and dermatological problems (9.8%). Patients with non-rheumatic conditions comprised a large part of the pediatric rheumatology outpatient clinic. National registries are required to establish the frequencies of pediatric rheumatic diseases in Turkey.

    更新日期:2020-01-14
  • Enthesitis and its relationship with disease activity, functional status, and quality of life in psoriatic arthritis: a multi-center study.
    Rheumatol. Int. (IF 2.200) Pub Date : null
    Ismihan Sunar,Sebnem Ataman,Kemal Nas,Erkan Kilic,Betul Sargin,Sevtap Acer Kasman,Hakan Alkan,Nilay Sahin,Gizem Cengiz,Nihan Cuzdan,Ilknur Albayrak Gezer,Dilek Keskin,Cevriye Mülkoğlu,Hatice Resorlu,Ajda Bal,Mehmet Tuncay Duruöz,Okan Küçükakkaş,Ozan Volkan Yurdakul,Meltem Alkan Melikoglu,Yıldıray Aydın,F Figen Ayhan,Hatice Bodur,Mustafa Calis,Erhan Capkın,Gul Devrimsel,Kevser Gok,Sami Hizmetli,Ayhan Kamanlı,Yaşar Keskin,Hilal Kocabas,Oznur Kutluk,Nesrin Şen,Omer Faruk Şendur,Ibrahim Tekeoğlu,Sena Tolu,Murat Toprak,Tiraje Tuncer

    Psoriatic arthritis (PsA) is an inflammatory arthritis with distinct phenotypic subtypes. Enthesitis is assigned as a hallmark of the disease, given its significant relations to disease activity and quality of life. Our objective is to evaluate the prevalence of enthesitis and its association with some clinical parameters, particularly quality of life, using data from a national registry. Patients with PsA meeting ClASsification criteria for Psoriatic Arthritis (CASPAR) were enrolled by means of a multi-centre Turkish League Against Rheumatism (TLAR) Network Project. The following information was recorded in web-based case report forms: demographic, clinical and radiographic data; physical examination findings, including tender and swollen joint counts (TJC and SJC); nail and skin involvement; Disease Activity Score-28 for Rheumatoid Arthritis with Erythrocyte Sedimentation Rate (DAS 28-ESR); Bath Ankylosing Spondylitis Disease Activity Index (BASDAI); Maastricht Ankylosing Spondylitis Enthesitis Score (MASES); Psoriasis Area Severity Index (PASI); Bath Ankylosing Spondylitis Radiology Index for the spine (BASRI-s); Health Assessment Questionnaire (HAQ); Bath Ankylosing Spondylitis Functional Index (BASFI); Health Assessment Questionnaire for the spondyloarthropathies (HAQ-s); Psoriatic arthritis quality of Life scale (PsAQoL); Short Form 36 (SF-36); Hospital Anxiety Depression Scale (HADS); Functional Assessment of Chronic Illness Therapy-Fatigue (FACIT-F); and Fibromyalgia Rapid Screening Tool (FiRST) scores. The patients were divided into two groups, namely with and without enthesitis, based on the triple Likert-type physician-reported statement of 'active enthesitis', 'history of enthesitis' or 'none' in the case report forms. Patients with active enthesitis were compared to others in terms of these clinical parameters. A total of 1130 patients were enrolled in this observational study. Of these patients, 251 (22.2%) had active enthesitis according to the clinical assessment. TJC, HAQ-s, BASDAI, FiRST and PsAQoL were significantly higher whereas the SF-36 scores were lower in patients with enthesitis (p < 0.05). Chronic back pain, dactylitis, and tenosynovitis were more frequent in the enthesopathy group (59.4%/39%, 13.1%/6.5% and 24.7%/3.4%, respectively). Significant positive correlations between the MASES score and the TJC, HAQ, DAS 28-ESR, BASDAI, FiRST and PsAQoL scores, and a negative correlation with the SF-36 score were found. When linear regression analysis was performed, the SF-36 MCS and PCS scores decreased by - 9.740 and - 11.795 units, and the FiRST scores increased by 1.223 units in patients with enthesitis. Enthesitis is an important involvement of PsA with significant relations to quality of life determined with PsAQoL and SF-36 scores. Our study found higher frequency of dactylitis and chronic back pain, and worse quality of life determined with SF-36 and PsAQoL scores in patients with enthesitis.

    更新日期:2020-01-14
  • The family history of rheumatoid arthritis in anti-cyclic citrullinated peptide antibody-positive patient is not a predictor of poor clinical presentation and treatment response with modern classification criteria and treatment strategy: the ANSWER cohort study.
    Rheumatol. Int. (IF 2.200) Pub Date : null
    Koichi Murata,Motomu Hashimoto,Wataru Yamamoto,Yonsu Son,Hideki Amuro,Koji Nagai,Tohru Takeuchi,Masaki Katayama,Yuichi Maeda,Kosuke Ebina,Ryota Hara,Sadao Jinno,Akira Onishi,Kosaku Murakami,Masao Tanaka,Hiromu Ito,Tsuneyo Mimori,Shuichi Matsuda

    A family history of rheumatoid arthritis (RA) is a strong risk factor for developing RA, affecting both genetically and environmentally. However, whether family history provides clinically relevant information in the modern classification and treatment remains largely unknown. This study aimed to determine whether a family history of RA is associated with a different clinical presentation of RA and treatment response. We retrospectively evaluated the demographic data and disease activity of newly diagnosed RA patients at baseline, 1 year, and 2 years after onset, using the ANSWER (Kansai consortium for the well-being of rheumatic disease patients) cohort data. Thirty-one patients (11.9%) among 260 newly diagnosed RA patients had a family history of RA up to second degree. There was no significant difference in the age at onset, time from onset to first visit, sex, positivity or value of rheumatoid factor or anti-cyclic citrullinated peptide antibody (ACPA), or disease activity between patients with and without a family history of RA. However, patients who had a family history of RA and were ACPA positive showed significantly lower erythrocyte sedimentation rate, and C-reactive protein. Disease activity in patients with a family history was not worse at baseline, after 1 year or 2 years of treatment. The Larsen score 2 years after onset was equivalent between the patients with and without a family history of RA in ACPA-positive patients. Family history of RA in ACPA-positive patients is not associated with high disease activity at baseline and is not a predictor of poor outcome 2 years after onset.

    更新日期:2020-01-14
  • Nailfold capillaroscopy and autoimmune connective tissue diseases in patients from a Portuguese nailfold capillaroscopy clinic.
    Rheumatol. Int. (IF 2.200) Pub Date : null
    Vera Bernardino,Ana Rodrigues,Ana Lladó,António Panarra

    Raynaud's phenomenon (RP) is frequent in autoimmune connective tissue diseases (AICTD) and its approach includes nailfold capillaroscopy (NFC), as it is a non-invasive technique that permits direct visualization of the microcirculation. The aim of this study is to analyze and establish clinical correlations between NFC findings and particular aspects of autoimmune disorders. This is a retrospective study. Clinical data from patients attending our NFC clinic were reviewed. Inclusion criteria included AICTD previous diagnosis, which included systemic sclerosis (SSc), mixed connective tissue disease (MCTD), systemic lupus erythematosus (SLE), Sjögren syndrome, inflammatory idiopathic myopathies (IIM), rheumatoid arthritis, undifferentiated connective tissue disease and antiphospholipid syndrome (APS). Videocap® version 3.0 biomicroscope was used. NFC score was determined. For statistics, SPSS software was utilized. 384 patients were included; most of them were women, with mean age of 47 years. RP was present in 91% of the patients, with greater prevalence in SSc and MCTD. Scleroderma pattern was the most prevalent NFC pattern, mainly in SSc, MCTD and IIM. Mean capillary density was reduced in IIM, SSc and MCTD. NFC score was worse in SSc, IIM and MCTD. In patients with AICTD, RP is related to microvascular damage and worse NFC score. NFC scleroderma pattern correlates with SSc classification criteria score. In MCTD, scleroderma pattern relates to myositis. SLE and APS reveal significant hemorrhages, but not related to APS antibodies. This study highlights the possible role of NFC as biomarker of AICTD, particularly in SSc and IIM.

    更新日期:2020-01-14
  • Biologic therapy in the idiopathic inflammatory myopathies.
    Rheumatol. Int. (IF 2.200) Pub Date : null
    Thomas Khoo,Vidya Limaye

    The idiopathic inflammatory myopathies (IIM) are a group of autoimmune diseases resulting from inflammation of muscle and manifesting as weakness, though a range of extra-muscular manifestations are observed. These are often correlated closely with disease subtype and the presence of myositis-specific/myositis-associated antibodies. IIM are notoriously difficult to treat and often refractory to glucocorticoid therapy and synthetic immunosuppressants. Both the innate and adaptive immune systems are implicated in the pathogenesis of IIM. A growing understanding of the key cytokines as well as the cell-mediated and antibody effectors of disease has identified multiple potential targets for biologic therapy. The most widely used of these is B-cell depletion via rituximab though the tumour necrosis factor inhibitors and other biologic therapies used in diseases such as rheumatoid arthritis, systemic lupus erythematosus and multiple sclerosis have also been trialled. This review summarises the literature thus far on biologic therapy in IIM, highlighting both the significant trials that influence current treatment regimens and also the continuing need for further research to inform more effective therapies.

    更新日期:2020-01-14
  • Re-conceptualizing functional status through experiences of young adults with inflammatory arthritis.
    Rheumatol. Int. (IF 2.200) Pub Date : null
    Kristine Carandang,Cheryl L P Vigen,Elizabeth Ortiz,Elizabeth A Pyatak

    The objective of this study is to assess the impact of inflammatory arthritis on young adults' activity participation using quantitative and qualitative methods to advance the field's conceptualization of functional status. Young adults diagnosed with juvenile idiopathic arthritis or rheumatoid arthritis completed (1) the Health Assessment Questionnaire-Disability Index to determine functional status and (2) the day reconstruction method to explore experiential dimensions of function, including functional performance, functional satisfaction, and severity of arthritis symptoms during activities on the previous day. Bivariate analyses were conducted to examine relationships between functional status, experiential variables, and demographic variables. Open-ended questions were provided for participants to report ways that arthritis affected their participation that were not otherwise reflected within survey questions; responses were numerically coded using summative content analysis. Among 37 participants (24.8 ± 3.3 years old), 70% reported moderate-to-severe disability. On average, participants experienced pain, stiffness, or fatigue for more than 50% of their waking hours. Functional status significantly correlated with functional performance (r = - 0.39, p = 0.02) and satisfaction (r = - 0.39, p = 0.02), yet did not correlate with stiffness or fatigue severity or duration of symptoms throughout the day. Participants described strategies that improved their ability to participate in certain activities but reduced their overall quality of activity engagement and caused emotional distress. Young adults with arthritis may experience more significant functional limitations than previously reported. Traditional measures of functional assessment may not capture experiential components of activity that affect participation, such as severity of stiffness or fatigue or the duration of symptoms throughout the day.

    更新日期:2020-01-14
  • Social media for research, education and practice in rheumatology
    Rheumatol. Int. (IF 2.200) Pub Date : 2019-12-20
    Olena Zimba, Olena Radchenko, Larysa Strilchuk

    Online social networking offers numerous opportunities for continuing medical education, professional development, and scholarly collaboration. Available social media channels proved useful for expanding education and research perspectives, particularly in rapidly developing academic disciplines such as rheumatology. Although there are numerous advantages of social media, busy clinicians should be aware of some drawbacks related to misinformation, unethical promotion, and unprofessional behavior in globally expanding platforms. Filtering credible and expert-proven information by skilled users is, therefore, increasingly important. Enforcing ethical norms and advancing professional etiquette in the field is strongly advisable. This article overviews the advantages and shortcomings of social media and reflects on available platforms for education and research in rheumatology.

    更新日期:2020-01-14
  • Effect of mycophenolate mofetil (MMF) on systemic sclerosis-related interstitial lung disease with mildly impaired lung function: a double-blind, placebo-controlled, randomized trial
    Rheumatol. Int. (IF 2.200) Pub Date : 2019-12-07
    G. S. R. S. N. K. Naidu, Shefali Khanna Sharma, M. B. Adarsh, Varun Dhir, Anindita Sinha, Sahajal Dhooria, Sanjay Jain

    The efficacy and safety of mycophenolate mofetil (MMF) has been studied in patients with systemic sclerosis (SSc)-related interstitial lung disease (ILD) with moderate–severe impairment. There is no study on its use in patients with mildly impaired lung function. The objective of this study is to determine the efficacy and safety of MMF for treating mild SSc-ILD (forced vital capacity (FVC) ≥ 70% predicted). This was a double-blind, randomized, placebo-controlled pilot trial. The subjects with SSc-ILD with FVC ≥ 70% were randomized to receive either MMF (2 g/day) or placebo for 6 months. FVC, diffusing capacity of lungs for carbon monoxide (DLCO), modified Rodnan skin score (mRSS), Short Form-36 (SF36v2), Mahler’s Dyspnoea Index (MDI), and 6-min walk distance (6MWD) were recorded at baseline and at 6 months. Forty-one subjects were included in the study (MMF: 20, placebo: 21). FVC decreased by a median of 2.7% (range − 21 to 9) in MMF arm and increased by 1% (range − 6 to 10) in placebo arm (p = 0.131). SF36v2 scores improved in both the groups. Median change in MDI (3 vs 3), DLCO (1% vs 1.5%), and 6MWD (0 m vs 0 m) was similar between the study groups. MMF was effective in improving mRSS (− 5 vs − 1, p = 0.045) compared to placebo. Adverse events occurred with similar frequency in both the study groups. In this pilot study, MMF did not result in significant improvement in lung function in SSc-ILD with minimally impaired lung function, but was effective in reducing the skin tightness. Larger studies are needed to confirm these findings. This study was registered at ClinicalTrials.gov (NCT02896205).

    更新日期:2020-01-14
  • The complexity of classifying ANCA-associated small-vessel vasculitis in actual clinical practice: data from a multicenter retrospective survey.
    Rheumatol. Int. (IF 2.200) Pub Date : null
    Luis Corral-Gudino,Elvira González-Vázquez,Ismael Calero-Paniagua,Laura Pérez-Garrido,Ivan Cusacovich,Alicia Rivas-Lamazares,Alba Quesada-Moreno,Ana González-Fernández,Damian Mora-Peña,Jose Luis Lerma-Márquez,Javier Del-Pino-Montes

    The different sets of criteria for diagnosis or classification of anti-neutrophil cytoplasmic antibody-associated vasculitis (AAV) lead to numerous overlapping and reclassified diagnoses in clinical practice. We designed this study to assess the difficulties in classifying patients with AAV. As a secondary objective, different variables were tested to predict prognosis. We conducted a retrospective chart review in a Western Spain multicentre survey. A total of 115 adult patients diagnosed with AAV from 2002 to 2013 and followed for at least 3 years were included. They were classified according to (1) Chapel Hill Consensus Conference (CHCC), (2) European Medicines Agency algorithm and (3) French Vasculitis Study Group/European Vasculitis Society phenotypes. Fifty-three patients (46%) had neither distinctive histopathological data of a single AAV definition nor any surrogate markers for granulomatous inflammation and thus did not fulfill any diagnostic criteria. Ocular, ear, nose, throat, skin, and lung involvement were more frequent with proteinase 3 (PR3) antibodies, whereas peripheral neuropathy was more frequent with myeloperoxidase (MPO) antibodies. When the disease was severe at diagnosis, the HR for mortality was 10.44. When induction treatment was not given in accordance with the guidelines, the HR for mortality was 4.00. For maintenance treatment, the HR was 5.49 for mortality and 2.48 for relapse. AAV classification is difficult because many patients had neither specific clinical data nor distinctive histological features of a single CHCC definition. A structured clinical assessment of patient severity is the best tool to guide the management of AAV.

    更新日期:2020-01-14
  • Comparison of an administrative algorithm for SLE disease severity to clinical SLE Disease Activity Index scores.
    Rheumatol. Int. (IF 2.200) Pub Date : null
    Cameron B Speyer,Daniel Li,Hongshu Guan,Kazuki Yoshida,Emma Stevens,April M Jorge,Brendan M Everett,Candace H Feldman,Karen H Costenbader

    Systemic lupus erythematosus (SLE) severity, reflecting both disease intensity and duration, is heterogeneous making it challenging to study in administrative databases where severity may confound or mediate associations with outcomes. Garris et al. developed an administrative claims-based algorithm employing claims over a 1-year period to classify SLE severity as mild, moderate or severe. We sought to compare this administrative algorithm to a measure of SLE activity, the SLE Disease Activity Index-2000 (SLEDAI-2K) score at clinical visits. We identified 100 SLE patients followed in the Brigham and Women's Hospital (BWH) Lupus Center (in 2008-2010) with SLEDAI-2K scores at each visit over a 1-year period per person. We obtained data for the Garris algorithm for the same year per subject. We compared Garris SLE severity to the highest SLEDAI-2K in that year, with SLEDAI-2K categories of mild < 3, moderate 3-6, and severe > 6. We compared classification using weighted kappa statistics, and positive and negative predictive values (PPV, NPV). We also assessed the binary comparison of mild vs. moderate/severe. We calculated sensitivity, specificity, and McNemar's test. We analyzed 377 SLEDAI-2K assessments (mean 3.8 [SD 2.6] per subject/year). For classifying moderate/severe vs. mild SLE severity, the sensitivity was 85.7%, specificity 67.6%, PPV 81.8% and NPV 73.5%. The Garris algorithm for classifying SLE severity in administrative datasets had moderate agreement for classification of mild vs. moderate/severe SLE activity assessed by SLEDAI-2K assessments in an academic lupus center. It may be a useful tool for classifying SLE severity in administrative database studies.

    更新日期:2020-01-14
  • Measurement properties of Portuguese–Brazil Western Ontario and McMaster Universities osteoarthritis index (WOMAC) for the assessment of knee complaints in Brazilian adults: ELSA-Brasil Musculoskeletal cohort
    Rheumatol. Int. (IF 2.200) Pub Date : 2019-12-19
    Poliane T. S. Lage, Luciana A. C. Machado, Sandhi M. Barreto, Roberta C. de Figueiredo, Rosa W. Telles

    Abstract Information on measurement properties of translated versions of the Western Ontario and McMaster Universities (WOMAC) osteoarthritis index is still limited. This study investigated the internal consistency, test–retest reliability/agreement, construct validity, and floor and ceiling effects of Portuguese–Brazil WOMAC applied to civil servants at baseline of ELSA-Brasil Musculoskeletal cohort. Each measurement property was evaluated in the overall sample, in the subgroup reporting knee symptoms, and across different sociodemographic strata (except factorial analyses). Separate analyses were performed for pain, stiffness and function dimensions, considering the knee with the worst score (or right knee if same score in both knees). A total of 1740 participants were included (319 completed WOMAC on 2 occasions), mean age 56.0 (standard deviation = 8.9) years, 46.8% male, 42.1% had knee symptoms. In the overall sample, the range of results for WOMAC’s dimensions were: internal consistency = cronbach alpha 0.92–0.98; test–retest reliability = intraclass correlation coefficient 0.85–0.97; standard error of measurement (SEM) = 1.38–5.86; smallest detectable change (SDC) = 3.84–16.25; lowest possible score = 38.8%-61.1% (floor effect present); highest possible score = 0.2%-0.9% (ceiling effect absent). Construct validity was confirmed by hypothesis testing and factorial analysis. Results were similar in the symptomatic group, except for higher SEM and SDC, and the absence of floor effects in pain and function dimensions. Portuguese–Brazil WOMAC showed good overall quality in a nonclinical setting. Variability in measurement properties across different strata of the population should be taken into consideration for the design of future studies using WOMAC.

    更新日期:2020-01-14
  • Obesity is related to incidence of patellofemoral osteoarthritis: the Cohort Hip and Cohort Knee (CHECK) study.
    Rheumatol. Int. (IF 2.200) Pub Date : null
    Harvi F Hart,Marienke van Middelkoop,Joshua J Stefanik,Kay M Crossley,Sita Bierma-Zeinstra

    To determine the longitudinal association of baseline body mass index and change in body mass index over 8 years to incident of patellofemoral osteoarthritis at the 8-year follow-up. A sample of 528 women and men, aged 45-65 years, with knee complaints and without radiographic evidence of patellofemoral and tibiofemoral osteoarthritis at baseline, were selected from the Cohort Hip and Cohort Knee cohort. Incidence of patellofemoral osteoarthritis was defined as presence of radiographic patellofemoral osteoarthritis (with or without tibiofemoral osteoarthritis) at the 8-year follow-up. Baseline body mass index data were categorized into normal, overweight, and obese weight-categories. Logistic regression analyses, adjusted for age and sex, were conducted to determine the association of baseline body mass index and change in body mass index to patellofemoral osteoarthritis incidence 8 years later. Obesity was associated with greater odds of radiographic patellofemoral osteoarthritis incident (odds ratio: 1.8 [95% CI 1.1, 3.1]) 8 years later. There were no significant associations observed between body mass index change over 8 years and incidence of radiographic patellofemoral osteoarthritis in overweight and obese individuals. Obesity is associated with increased odds of developing radiographic patellofemoral osteoarthritis 8 years later.

    更新日期:2020-01-14
  • Efficacy and retention rate of adalimumab in rheumatoid arthritis and psoriatic arthritis patients after first-line etanercept failure: the FEARLESS cohort.
    Rheumatol. Int. (IF 2.200) Pub Date : null
    Ennio G Favalli,Andrea Becciolini,Antonio Carletto,Fabrizio Conti,Giorgio Amato,Enrico Fusaro,Luca Quartuccio,Colin Gerard Egan,Andrea Lo Monaco,Maurizio Benucci,Fausto Salaffi,Angelo Semeraro,Simone Parisi,Fulvia Ceccarelli,Ilaria Piazza,Rosario Foti

    Few studies have compared the efficacy of switching from etanercept to adalimumab in the real-life setting in rheumatoid arthritis (RA) and psoriatic arthritis (PsA). This study evaluated the 2-year retention rate and 12-month efficacy of adalimumab in RA and PsA patients, previously treated with etanercept. RA and PsA patients from 11 Italian Rheumatology Units received adalimumab after first-line etanercept failure. Two-year adalimumab retention rate was calculated by the Kaplan-Meier method and Cox proportional hazard models were developed to examine predictors of drug persistence. Univariate and multivariate logistic regression analyses were developed to examine potential predictors of 12-month DAS-28 remission. The study population included 117 RA (disease duration of 10.1 ± 7.7 years and baseline DAS28-ESR of 4.97 ± 1.3) and 102 PsA (disease duration of 7.1 ± 5.1 years and baseline DAPSA of 24.6 ± 11.8). The 2-year retention rate was 48.2% in RA and 56.5% in PsA patients. Concomitant methotrexate treatment was not associated with increased drug survival in both groups. Similarly, cause of etanercept discontinuation or treatment duration was not associated with retention rate. 12-month remission and low disease activity were achieved, respectively, in 27.3% and 23.9% of RA patients and 27.4% and 23.5% PsA of patients. In multivariate models, etanercept discontinuation due to inefficacy (OR 0.27, 95% CI 1.03-0.73; p = 0.009) and baseline DAS-28 (OR 0.45, 95% CI 0.29-0.69; p < 0.001) remained significant negative predictors of remission in RA patients. No variable was associated with remission in PsA patients. Adalimumab after etanercept failure was highly effective and safe in both RA and PsA patients.

    更新日期:2020-01-14
  • Risk prediction of degenerative scoliosis combined with lumbar spinal stenosis in patients with rheumatoid arthritis: a case–control study
    Rheumatol. Int. (IF 2.200) Pub Date : 2020-01-09
    Shuai Xu, Yan Liang, Fanqi Meng, Zhenqi Zhu, Haiying Liu

    The purpose of this study is to compare incidence of degenerative scoliosis (DS) in patients who diagnosed lumbar spinal stenosis (LSS) with or without rheumatoid arthritis (RA) and identify the risk factors of DS severity in RA patients. 61 LSS patients with RA (RA group) and 87 demographic-matched LSS patients without RA (NoRA group) from January 2013 to April 2018 were enrolled. The extracted information includes RA-related parameters such as Steinbrocker classification, disease-modifying anti-rheumatic drugs (DMARDs), and DS-related information such as Cobb angle, apical vertebra, along with osteoporosis and history of total knee arthroplasty (TKA). Comparisons between RA and NoRA group and between DS and non-DS subgroup with RA were performed, as well as the risk factors on DS severity in RA patients. The incidence of DS in RA group was 42.6%, larger than that of NoRA group (P = 0.002). The mean Cobb angle between the two groups was of no difference (P = 0.076). The apical vertebrae were both mainly focused on L3 and L4 vertebrae in both groups with no significant difference on the distribution of apical vertebrae (P = 0.786). Female took a larger proportion in DS subgroup than that of NoDS subgroup in patients with RA (P = 0.039), while Steinbrocker classification was irrelevant to the occurrence of DS and Cobb angle. Multiple regression analysis showed that TKA was a risk factor for the severity of Cobb angle (P = 0.040). The incidence of DS in LSS patients with RA is higher than non-RA patients. RA patients performed TKA sustained less severity of DS.

    更新日期:2020-01-11
  • Positive conversion of interferon-γ release assay in patients with rheumatic diseases treated with biologics
    Rheumatol. Int. (IF 2.200) Pub Date : 2020-01-09
    Hye Won Kim, Oh Chan Kwon, Sang Hoon Han, Min-Chan Park

    The objective of this study is to investigate whether the type of biologics (TNFi or others) or type of rheumatic diseases (RA or AS) influence the conversion rate of initially negative tuberculosis (TB) screening test results. A total of 119 patients with RA or AS who had negative baseline interferon-γ release assay (IGRA) results assessed by QuantiFERON-TB Gold in tube (QTF-GIT) were included. All patients received biologic agents, and rescreening with QTF-GIT was performed after a median of 25.9 months from the baseline test. Clinical characteristics and IFN-γ levels were compared between converters and non-converters. Logistic regression analysis was performed to identify factors associated with positive conversion. IGRA conversion was found in 14 of 119 patients (11.8%). The converters were older (53.4 ± 14.2 vs 44.4 ± 15.5 years, p = 0.040), had higher baseline TB-specific IFN-γ responses (0.105 [0.018–0.205] vs 0.010 [0.000–0.035] IU/ml, p = 0.001) and higher incidence of active TB (14.3% vs 0.0%, p = 0.013). The number of patients with RA or AS was 9 (64.3%) or 5 (35.7%) in converters, and 45 (42.9%) or 60 (57.1%) in non-converters. In terms of use of biologics, TNFi of monoclonal antibody form was less commonly used in the converters (p = 0.024). In the logistic regression analysis, type of disease and type of biologics used were not associated with IGRA conversion, whereas baseline TB-specific IFN-γ response was significantly associated with IGRA conversion (OR 1.083, 95% CI 1.019–1.151, p = 0.011). Serial monitoring of LTBI with IGRA retesting is needed during biologic treatment, regardless of the type of rheumatic diseases or type biologics used.

    更新日期:2020-01-09
  • Correction to: A prospective, longitudinal monocentric study on laser Doppler imaging of microcirculation: comparison with macrovascular pathophysiology and effect of adalimumab treatment in early rheumatoid arthritis
    Rheumatol. Int. (IF 2.200) Pub Date : 2020-01-06
    László Dávida, Vanda Pongrácz, Emir Awad Mohamed, Szilvia Szamosi, Gabriella Szücs, Andrea Váncsa, Orsolya Tímár, Zoltán Csiki, Edit Végh, Pál Soltész, Zoltán Szekanecz, György Kerekes

    In the Original article, the legend of Figures 3 and 4 are interchanged and line number 387 is incomplete.

    更新日期:2020-01-06
  • Fibromyalgia interferes with disease activity and biological therapy response in inflammatory rheumatic diseases
    Rheumatol. Int. (IF 2.200) Pub Date : 2020-01-03
    Ilke Coskun Benlidayi

    Fibromyalgia is one of the numerous comorbidities that may accompany inflammatory rheumatic diseases. Concomitant fibromyalgia in inflammatory rheumatic conditions can interfere with symptomatology, disease activity and overall management plan. The aim of the present narrative review article was to discuss the current evidence on (i) the prevalence/frequency of comorbid fibromyalgia in inflammatory rheumatic conditions, (ii) the role of fibromyalgia on disease activity, (iii) the impact of concomitant fibromyalgia on biological disease-modifying antirheumatic treatment outcomes and (iv) potential effectiveness of biological disease-modifying antirheumatic drugs on fibromyalgia-related symptoms among patients with inflammatory rheumatic diseases. A literature search was conducted through PubMed/MEDLINE Cochrane and Web of Science databases by using relevant keywords and their combinations. Studies representing different geographical areas of the world revealed that frequency rates of fibromyalgia are higher in inflammatory rheumatic diseases than those in the general population. Comorbid fibromyalgia interferes not only with the disease activity scores but also with the treatment outcomes and management plan. Further evidence is warranted in order to determine the potential benefits of biological disease-modifying antirheumatic drugs on fibromyalgia-related symptoms in patients with inflammatory rheumatic diseases.

    更新日期:2020-01-04
  • Juvenile dermatomyositis resembling late-stage Degos disease with gastrointestinal perforations successfully treated with combination of cyclophosphamide and rituximab: case-based review
    Rheumatol. Int. (IF 2.200) Pub Date : 2020-01-03
    William Day, Christos Gabriel, Robert E. Kelly, Cynthia M. Magro, Judith V. Williams, Alice Werner, Laura Gifford, Sameer P. Lapsia, Cassyanne L. Aguiar

    Abstract Dermatomyositis (DM) is a multi-system disease that results in chronic inflammation principally of the skin and striated muscle. Small blood vessel injury in the GI tract has been described in dermatomyositis, manifesting as bleeding, ulceration, pneumatosis intestinalis, and ultimately perforation. Recent histopathological studies have shown deposits in the capillaries of the skin, gastrointestinal tract, and brain of patients with dermatomyositis similar to that found in patients with Degos disease, suggesting these disease processes are closely related or represent varying degrees of severity on the same pathologic spectrum. We report a case of juvenile dermatomyositis (JDM) resembling late-stage Degos disease with gastrointestinal perforations successfully treated with combination rituximab and cyclophosphamide therapy. We systematically reviewed the literature detailing the medical and surgical treatments for gastrointestinal perforation in dermatomyositis, Degos-like dermatomyositis, and Degos disease. In addition to our case, as of October 2019, we identified 36 cases describing gastrointestinal perforation in patients with underlying dermatomyositis, 5 cases of Degos-like dermatomyositis and 17 cases of idiopathic Degos disease. Corticosteroid therapy was used widely for dermatomyositis and Degos-like dermatomyositis, while antiplatelet and anticoagulant medications were chiefly used for patients with idiopathic Degos disease. However, there were no cases that detailed the successful treatment of dermatomyositis or Degos disease with gastrointestinal perforation with rituximab alone or combined with cyclophosphamide. We report that rituximab, in combination with cyclophosphamide, can be used as a novel adjunctive therapy to successfully treat dermatomyositis with Degos-like gastrointestinal perforation.

    更新日期:2020-01-04
  • Rheumatology in Ukraine
    Rheumatol. Int. (IF 2.200) Pub Date : 2020-01-02
    Bohdana Doskaliuk, Olena Zimba, Roman Yatsyshyn, Volodymyr Kovalenko

    Rheumatology in Ukraine is based on established research and clinical infrastructure that enable the integration with regional and global societies. This article overviews current state of Ukrainian rheumatology, important steps toward expanding clinical settings, and opportunities for strengthening cooperation of local rheumatologists and patients with rheumatic diseases with related international societies. The main achievements and some challenges encountered by the Association of Rheumatologists of Ukraine are outlined. The Association supports continuing medical education of local specialists and explores options for advancing research and publishing in rheumatology. One of the main challenges still remains the creation of registries of patients with rheumatic diseases and expansion of activities of public societies with interest in combating rheumatic diseases. The issue of inadequate access to immunobiological therapies is also highlighted.

    更新日期:2020-01-04
  • Recurrent myelitis and asymptomatic hypophysitis in IgG4-related disease: case-based review
    Rheumatol. Int. (IF 2.200) Pub Date : 2020-01-02
    Aigli G. Vakrakou, Maria-Eleptheria Evangelopoulos, Georgios Boutzios, Dimitrios Tzanetakos, John Tzartos, Georgios Velonakis, Panagiotis Toulas, Maria Anagnostouli, Elissavet Andreadou, Georgios Koutsis, Leonidas Stefanis, George E. Fragoulis, Constantinos Kilidireas

    IgG4-related disease (IgG4-RD) is a disorder with various clinical manifestations. Central nervous system (CNS) involvement is well recognized, with hypertrophic pachymeningitis and hypophysitis being the most common manifestations. Spinal cord involvement is an extremely rare manifestation. We present the first case of an IgG4-RD patient with spinal cord parenchymal disease and concurrent hypophysitis. We review also the current literature about CNS parenchymal involvement in the context of IgG4-RD. A young female presented with clinical symptoms of myelitis. Cervical spinal cord magnetic resonance imaging (MRI) displayed features of longitudinally extensive transverse myelitis (LETM). Brain MRI showed a small number of high-intensity lesions in the deep white matter and enlargement of hypophysis with homogeneous gadolinium enhancement (asymptomatic hypophysitis). Diagnostic workup revealed elevated IgG4 serum levels (146 mg/dL). Our patient fulfilled the organ-specific diagnostic criteria of IgG4-hypophysitis. Treatment with intravenous glucocorticoids led to rapid clinical response, and to the substantial resolution of imaging findings. Azathioprine was used as a maintenance treatment. One relapse occurred 2 years after the initial diagnosis and patient was re-treated with glucocorticoids. Three years after relapse, patient is in remission with azathioprine. We present the first case of myelitis with radiological features of LETM associated with increased IgG4 serum levels and the simultaneous presence of asymptomatic IgG4-related hypophysitis.

    更新日期:2020-01-04
  • Description of the resources for rheumatology training and speciality certification and recertification requirements in Argentina
    Rheumatol. Int. (IF 2.200) Pub Date : 2020-01-02
    Marina Laura Micelli, Gabriel Sequeira, Nauan Fara, Leila Abbas, Natalia Estrella, Magdalena Acevedo, Eduardo Kerzberg

    The objective of the study was to analyse resources for rheumatology training, and speciality certification and recertification requirements in Argentina. Information was gathered regarding vacancies, entry requirements, duration and validity of the specialist degree in every residency and postgraduate course in adult rheumatology. The following aspects were analysed: monitoring authority, certification and recertification requirements, and mandatory recertification. Six out of 36 universities offer rheumatology postgraduate courses. Out of 65 vacancies, 36 (55%) are implemented by a National Public University in the Autonomous City of Buenos Aires (CABA), and 46 (70%) are in CABA. There are 32 vacancies for rheumatology residencies in 7 out of 24 districts, 16 of them (50%) in CABA. There are 2- to 3-year postgraduate courses; entry requirements range from 1-year experience in internal medicine to either complete residency or specialist degree in internal medicine. Training formats vary from full-time university residency to either university-based courses with part-time dedication in a rheumatology service without residency or non-university courses with part-time dedication. Not every specialist degree is automatically homologated in every jurisdiction. Provincial governments and colleges of physicians are the certification and recertification authorities; medical school was included in one district. Recertification is mandatory in only 8 districts; 40–50% of the process is achieved by merely practising as a rheumatologist. Most of the training resources are concentrated in CABA. Although there are various options, not all of them are automatically homologated. Recertification is not mandatory nationwide, and a significant part of the process involves practising as a rheumatologist.

    更新日期:2020-01-04
  • Prevalence and demographic characteristics of Behcet disease in Colombia: data from the national health registry 2012–2016
    Rheumatol. Int. (IF 2.200) Pub Date : 2019-10-29
    Daniel G. Fernández-Ávila, Diana N. Rincón-Riaño, Santiago Bernal-Macías, Juan M. Gutiérrez Dávila, Diego Rosselli

    To estimate prevalence and describe the main demographic characteristics of Behcet disease in Colombia. Cross-sectional study, based on official Ministry of Health registry data. 523 cases of Behcet disease (ICD-10 code: M352) were reported between 2012 and 2016, for a prevalence in people over 18 years old of 1.10 per 100,000 inhabitants, of which 68% are women, with a female-to-male ratio of 2.2:1, with greater prevalence within the 45–49 age group. This is the first study that shows demographic and epidemiological information on Behcet disease in Colombia. Prevalence seems to be low when compared with other similar studies in the region.

    更新日期:2020-01-04
  • Pattern and diagnostic evaluation of systemic autoinflammatory diseases other than familial Mediterranean fever among Arab children: a multicenter study from the Pediatric Rheumatology Arab Group (PRAG)
    Rheumatol. Int. (IF 2.200) Pub Date : 2019-11-18
    Sulaiman M. Al-Mayouf, Abdulaziz Almutairi, Safiya Albrawi, Basil M. Fathalla, Raed Alzyoud, Abdullatif AlEnazi, Mohammed Abu-shukair, Adel Alwahadneh, Abdullah Alsonbul, Mabruka Zlenti, Ebtisam Khawaja, Awatif Abushhaiwia, Khulood Khawaja, Zakiya AlMosawi, Wafa Madan, Muna Almuatiri, Nora Almuatiri, for Pediatric Arab Rheumatology Group (PRAG)

    To define the spectrum and phenotypic characteristics of systemic autoinflammatory diseases (SAIDs) other than familial Mediterranean fever (FMF) in Arab children and to delineate diagnostic evaluation. Data retrospectively collected on patients with clinical and/or genetically proven SAIDs other than FMF at 10 tertiary Arab pediatric rheumatology clinics from 1990 to 2018. The collected data comprised the clinical findings and diagnostic evaluation including genetic testing, the provided treatment and the accrual damage related to SAIDs. A total of 144 patients (93 female) with a median age at onset of 2.5 (range 0.1–12) years were enrolled. The initial diagnosis was inaccurate in 49.3%. Consanguinity rate among parents was 74.6%. The median time-to-diagnosis for all SAIDs was 2.5 (range 0.1–10) years. There were 104 patients (72.2%) with a confirmed diagnosis and 40 patients with suspected SAIDs. Seventy-two had monogenic and 66 patients with multifactorial SAIDs while six patients had undifferentiated SAIDs. The most frequent monogenic SAIDs were LACC1 mediated monogenic disorders (n = 23) followed by CAPS (12), TRAPS (12), HIDS (12), and Majeed’s syndrome (6). The most frequent multifactorial SAIDs was CRMO (34), followed by PFAPA (18), and early onset sarcoidosis (EOS) (14). Genetic analysis was performed in 69 patients; 50 patients had genetically confirmed disease. Corticosteroid used for 93 patients while biologic agents for 96 patients. Overall, growth failure was the most frequent accrual damage (36%), followed by cognitive impairment (13%). There were three deaths because of infection. This study shows a heterogenous spectrum of SAIDs with a high number of genetically confirmed monogenic diseases; notably, LACC1 associated diseases. Hopefully, this work will be the first step for a prospective registry for SAIDs in Arab countries.

    更新日期:2020-01-04
  • Prescribing motivations and patients’ characteristics related to the use of biologic drugs in adult-onset Still’s disease: analysis of a multicentre “real-life” cohort
    Rheumatol. Int. (IF 2.200) Pub Date : 2019-07-01
    Piero Ruscitti, Paola Cipriani, Vasiliki Liakouli, Daniela Iacono, Ilenia Pantano, Francesco Caso, Federico Perosa, Fabiola Atzeni, Francesco Paolo Cantatore, Raffaele Scarpa, Francesco Ciccia, Roberto Giacomelli

    A growing body of evidence suggests the usability of biologic disease-modifying anti-rheumatic drugs (bDMARDs) in treating adult-onset Still’s disease (AOSD). In a multicentre “real-life” cohort, the physicians’ prescribing motivations and patients’ predictive characteristics of being treated with bDMARDs were assessed. Patients with AOSD, who were included in GIRRCS (Gruppo Italiano di Ricerca in Reumatologia Clinica e Sperimentale) cohort and treated with bDMARDs, were retrospectively assessed. Relevant data were collected by a review of clinical charts. Forty-four patients treated with bDMARDs were analysed, with slight male preponderance (52.3%) and a mean age of 39.3 ± 15.2 years. All patients were treated with corticosteroids (CCSs) (38.6% with low dosage) and 93.2% were treated with synthetic DMARDs (sDMARDs). Regarding the effectiveness of the first-line bDMARD, 65.6% of patients experienced a complete remission, defined as complete disappearance of both systemic and joint symptoms and normalisation of laboratory evidence of disease. The physicians’ prescribing motivations for bDMARDs were inadequate response to CCSs and/or sDMARDs, CCS-sparing effect and occurrence of macrophage activation syndrome (MAS). Analysing patients’ characteristics, chronic disease course (OR 3.09; 95%CI 1.22–7.80, p = 0.017), defined as disease with persistent symptoms, was predictive of being treated with bDMARDs, whereas age (OR 0.97, 95%CI 0.93–0.99, p = 0.048) was negatively associated, suggesting younger age as a further predictive factor. Patients with AOSD were treated with bDMARDs for inadequate response to CCSs and/or sDMARDs, CCS-sparing effect and MAS occurrence. Younger age and chronic disease course were patients’ predictive characteristics of being treated with bDMARDs.

    更新日期:2020-01-04
  • Peripheral blood mononuclear cell proteome profile in Behçet’s syndrome
    Rheumatol. Int. (IF 2.200) Pub Date : 2019-08-14
    Asli Kirectepe Aydin, Yeşim Özgüler, Didar Uçar, Murat Kasap, Gürler Akpınar, Emire Seyahi, Eda Tahir Turanli

    Abstract Behçet’s syndrome (BS) is a systemic inflammatory disorder with unknown etiology. Investigation of proteome profiles of disease specific cells facilitates our understanding of the processes and related molecular pathways, especially in disorders like BS with complex inheritance pattern and clinical heterogeneity. In the current study, we evaluated the peripheral blood mononuclear cells (PBMCs) proteome of 59 patients with BS (33 in active and 26 in inactive phases) and of 28 healthy controls using two-dimensional fluorescence difference gel electrophoresis (2D-DIGE). Differentially expressed protein spots with at least twofold and/or statistically significant change (p ≤ 0.05) between active BS vs inactive BS, and also active BS vs healthy controls were identified by mass spectrometry (MALDI-TOF/TOF). Bioinformatic analyses revealed 16 differentially expressed proteins (12 of them in active vs inactive BS comparison, whereas 11 of them for active BS vs healthy control comparison) belonging to glycolysis, cytoskeleton organization, protein folding, and regulation of blood coagulation pathways. Stathmin (active BS vs inactive BS; fourfold, active BS vs healthy control; 4.7-fold) and WD repeat-containing protein-1 (active BS vs inactive BS; 2.7-fold, active BS vs healthy control; 2.7-fold), which are cytoskeleton-related proteins, were found to be lower in active patients compared to inactive patients and healthy control. Decreased levels of calreticulin (active BS vs inactive BS; 1.29-fold) and heat shock 70 kDa protein 8 (active BS vs healthy control; 1.5-fold) which are involved in protein folding and endoplasmic reticulum (ER) stress process, were observed in patients with active phase of BS. Down-regulation of protein folding and ER stress process proteins in BS patients may further support the involvement of ER stress in BS.

    更新日期:2020-01-04
  • Chronic nonbacterial osteomyelitis in children: a multicenter case series
    Rheumatol. Int. (IF 2.200) Pub Date : 2019-08-02
    Sara Concha, Alfonso Hernández-Ojeda, Oscar Contreras, Cecilia Mendez, Eduardo Talesnik, Arturo Borzutzky

    Chronic nonbacterial osteomyelitis (CNO) is a primary autoinflammatory bone disease that presents more frequently in children and is characterized by inflammatory bone lesions in the absence of an infectious etiology. There is little information of this disease in Latin America. The objective of the study was to evaluate demographic, clinical, laboratory, imaging, histopathology characteristics, and treatment responses of pediatric CNO patients. The clinical records of 19 patients with CNO diagnosed between 2007 and 2019 at three tertiary centers in Santiago, Chile were reviewed. The median age of onset was 10 years and 47% were female. Median delay in diagnosis was 12 months. All patients had a pattern of recurrent multifocal disease. 37% of patients had positive antinuclear antibodies and 16% HLA-B27 positivity. 21% of patients presented arthritis or other rheumatologic comorbidity, although no association with psoriasis, inflammatory bowel disease (IBD) or palmoplantar pustulosis (PPP) was observed. Eighteen patients received treatment with nonsteroidal anti-inflammatory drugs with partial response. Twelve patients received methotrexate, and half of them received steroids at the same time reaching remission in 50%. Of the five patients who received bisphosphonates, 60% achieved remission. All four patients who received adalimumab had comorbid arthritis and 75% achieved remission. In a series of Chilean children with CNO, all patients presented with multifocal lesions. Comorbid autoimmune diseases including arthritis were frequent, but no association was observed with psoriasis, IBD, or PPP.

    更新日期:2020-01-04
  • Identification of physical and psychosocial problems based on symptoms in patients with Behçet’s disease
    Rheumatol. Int. (IF 2.200) Pub Date : 2019-12-04
    Hideyo Tsutsui, Hirotoshi Kikuchi, Hiroko Oguchi, Kyoko Nomura, Takayoshi Ohkubo

    Abstract The purpose of this study was to use the International Classification of Functioning, Disability and Health (ICF) to identify physical and psychosocial problems associated with symptoms of Behçet’s disease (BD) in Japanese patients. Thirty patients with BD were interviewed in a pilot study using the “ICF Checklist”, and a team of medical experts selected categories related to physical and psychosocial aspects of BD. To identify specific physical and psychosocial problems of Japanese patients with BD, 100 new patients were interviewed using the selected categories. Among the 128 categories in the original ICF Checklist, 80 categories were identified as impaired, and another 12 ICF categories were added based on expert discussion of patients input. The number of problem categories was significantly greater in patients with BD with eye involvement and fatigue (eye involvement, 25.7 categories; fatigue, 25.2 categories; both P < 0.001). Specifically, patients with eye involvement had more difficulties with problems in daily life, such as writing (odds ratio 4.2), understanding such nonverbal messages as gestures and facial expressions (13.7), moving (5.7), walking in intense sunlight and bright light (17.6), and patients with fatigue had more difficulties with climate problems such as symptoms getting worse at the turn of the seasons or on cold days (2.5), compared to those without these symptoms. This study demonstrated that support focusing not only on physical symptoms but on other aspects of life as well is necessary for patients with BD, particularly patients with eye involvement and fatigue.

    更新日期:2020-01-04
  • Digital ischemia in Behçet’s disease: case-based review
    Rheumatol. Int. (IF 2.200) Pub Date : 2019-10-09
    Gopika Hari, Nedaa Skeik

    Behçet’s disease is a rare chronic autoimmune disease affecting primarily Middle and East Asian populations between the ages of 20 and 40 years. Behçet’s disease manifests with oral and genital mucocutaneous lesions, ocular disease, venous thrombosis, and central nervous system degradation. Treatment can be challenging and may require immunosuppressive agents and/or topical wound-care. While larger vascular involvement has been reported, digital ischemia due to small-vessel involvement has not been well described in the literature. Based on a systematic literature review, we were only able to find seven published cases of limb ischemia, none of which reported digital involvement. We present a unique case of Behçet’s disease with severe digital ischemia and ulceration caused by small-vessel involvement. The patient was managed successfully with antiplatelet, immunosuppressants, and anti-inflammatories with complete resolution of the ischemic symptoms. By focusing on small-vessel involvement and digital ischemia, we provide insight into clinical presentation and treatment for this very rare vascular manifestation of Behçet’s disease.

    更新日期:2020-01-04
  • Familial mediterranean fever: assessment of clinical manifestations, pregnancy, genetic mutational analyses, and disease severity in a national cohort
    Rheumatol. Int. (IF 2.200) Pub Date : 2019-09-14
    Hatice Bodur, Fatma Gül Yurdakul, Hasan Fatih Çay, Ülkü Uçar, Yaşar Keskin, Betül Sargın, Gülcan Gürer, Ozan Volkan Yurdakul, Mustafa Çalış, Hülya Deveci, Yıldıray Aydın, Sami Hizmetli, Remzi Çevik, Ali Yavuz Karahan, İsmihan Sunar, Mehmet Tuncay Duruöz, Hilal Ecesoy, Zafer Günendi, Murat Toprak, Nesrin Şen, Duygu Altıntaş, Ahmet Kıvanç Cengiz, Gökhan Çağlayan, Ali Nail Demir, Hüseyin Kaplan, Sertaç Ketenci, Meltem Alkan Melikoğlu, Mehmet Nayimoğlu, Kemal Nas, Ayşe Banu Sarıfakıoğlu, İlhan Sezer

    Abstract The aims of this study were to investigate the main clinical and laboratory features, including pregnancy and genetic analysis, of Turkish Familial Mediterranean Fever (FMF) patients and to analyze the relationships between genotypic features, age of disease onset, clinical findings, and disease severity. A study was planned within a national network of 22 different centers. Demographics, clinical and laboratory findings, attack characteristics, drugs, pregnancy and birth history, disease severity, and gene mutation analyses were evaluated. Disease severity, assessed using a scoring system developed by Pras et al., was evaluated in relation to gene mutations and age of disease onset. A total of 979 patients (643 females and 336 males; mean age: 35.92 ± 11.97 years) with FMF were included in the study. Of a total of 585 pregnancies, 7% of them resulted in preterm birth and 18.1% resulted in abortions. During pregnancy, there was no FMF attack in 61.4% of patients. Of the MEditerranean FeVer (MEFV) mutations, 150 (24.3%) cases were homozygous, 292 (47.3%) cases were heterozygous, and 175 (28.4%) were compound heterozygous. Patients with homozygous gene mutations had more severe disease activity, earlier age of disease onset, higher rates of joint and skin involvement, sacroiliitis, and amyloidosis. Patients with compound heterozygous genotype displayed severe disease activity in close resemblance to patients with homozygous mutation. In addition, patients with compound heterozygous mutations had higher rates of protracted febrile myalgia and elevated fibrinogen levels. In 63.9% of compound heterozygous patients, age of onset was < 20 years, with greater disease severity, and high rates of attack frequency and colchicine resistance. Our results suggest that indicators for disease severity include early onset of disease and homozygous gene mutations. Furthermore, patients with compound heterozygous mutations displayed significant presentations of severe disease activity.

    更新日期:2020-01-04
  • Familial Mediterranean fever and atherosclerosis in childhood and adolescence
    Rheumatol. Int. (IF 2.200) Pub Date : 2019-11-08
    Olga Vampertzi, Kyriaki Papadopoulou-Legbelou, Areti Triantafyllou, Stella Douma, Efimia Papadopoulou-Alataki

    Familial Mediterranean fever is a chronic inflammatory disease characterized by periodic and self-limited episodes of fever and aseptic polyserositis. Although colchicine treatment has altered the course of the disease, it is believed that subclinical inflammation is still present, leading to endothelial dysfunction and atherosclerosis in the course of time. In this review, following the published recommendations, we queried online databases such as MEDLINE Pubmed, Scopus, and Web of science for peer-reviewed studies and reviews written in English language, using the following keywords: familial Mediterranean fever, children, endothelial dysfunction, atherosclerosis, cardiovascular disease. The objective of this review is to highlight the correlation between familial Mediterranean fever and atherosclerosis, and moreover to describe new serum inflammatory markers and non-invasive methods of endothelial dysfunction, to detect the atherosclerosis process early starting from childhood.

    更新日期:2020-01-04
  • Familial Mediterranean fever is associated with a wide spectrum of inflammatory disorders: results from a large cohort study
    Rheumatol. Int. (IF 2.200) Pub Date : 2019-08-07
    Nuh Atas, Berkan Armagan, Erdal Bodakci, Hasan Satis, Alper Sari, Nazife Sule Yasar Bilge, Reyhan Bilici Salman, Gozde Kubra Yardımcı, Hakan Babaoglu, Aslihan Avanoglu Guler, Hazan Karadeniz, Levent Kilic, Mehmet Akif Ozturk, Berna Goker, Seminur Haznedaroglu, Umut Kalyoncu, Timucin Kasifoglu, Abdurrahman Tufan

    Abstract Familial Mediterranean fever (FMF) is characterized by recurrent short-lived/self-limiting inflammatory attacks. Besides these, a substantial number of patients with FMF present with a variety of other inflammatory diseases; however, this issue has not been systematically studied previously. Hence, we aimed to investigate the frequency of inflammatory comorbid diseases in a large FMF cohort. All patients were recruited from “FMF in Central Anatolia (FiCA) Cohort”, comprising 971 (mean age 35.3 ± 12 years, 61.5% female) adult subjects. All patients fulfilled Tel Hashomer criteria. Demographic data, FMF disease characteristics, MEFV gene mutations, and comorbid inflammatory diseases were meticulously questioned, and laboratory features and genotype data were retrieved from hospital records. There were comorbid inflammatory diseases in 205 (21.1%) patients. The most common inflammatory disease was spondyloarthritis (12.9%). Other remarkable inflammatory disorders were psoriasis, immunoglobulin A vasculitis/Henoch–Schönlein purpura, Behçet’s disease and inflammatory bowel diseases. Cryptogenic organizing pneumonia is a newly defined entity in our cohort which is seemed to be associated with FMF (0.3%). Number of patients with persistent inflammation was higher in those with comorbid diseases (p < 0.001). Our results suggest that FMF is commonly associated with other inflammatory diseases. Therefore, clinicians should be cautious about comorbid inflammatory diseases in FMF patients, particularly in those with persistent inflammation. Identification of pathogenic pathways linking FMF to these diseases warrants further investigations.

    更新日期:2020-01-04
  • Sexual dysfunction in Behçet’s syndrome
    Rheumatol. Int. (IF 2.200) Pub Date : 2019-10-08
    Rosaria Talarico, Elena Elefante, Alice Parma, Fabio Taponeco, Tommaso Simoncini, Marta Mosca

    Behçet’s syndrome (BS) is an autoimmune, rare, and severe multisystemic inflammatory disease characterized by recurrent oral aphthous ulcers, genital ulcers, skin lesions, and both anterior and posterior uveitis; articular, vascular, gastroenteric and neurological involvement may also occur. The multi-organ involvement and the wide range of clinical spectrum make the diagnosis of BS challenging. As other systemic chronic diseases, BS can affect all aspects of patients’ life, including sexual dysfunction (SD). So far, SD has been deeply studied among patients affected by many rheumatic diseases; however, little is known about the prevalence and the characteristics of SD among BS patients. The present work was aimed at providing a systematic literature review of the literature published on SD and BS. We carried out a systematic search in PubMed and Scopus based on controlled terms (MeSH) and keywords to identify literature data on SD in BS. The systematic search was independently performed by two clinicians based on the controlled medical subject heading terms for Behcet syndrome and SD. Systematic database search identified 32 records. Screening by title and abstract was then conducted and a total of 10 articles were eligible for full text assessment, five studies explored SD in male patients with BS, 3 studies in females and 2 works reported data on both genders. Globally, the systematic literature review results have underlined that SD seems frequent in BS patients. Both female and male patients experienced a significant correlation between SD and depression. The studies investigating sexuality in BS seem to demonstrate that in these patients SD may be mainly related to depression rather than to active organic manifestations.

    更新日期:2020-01-04
  • The peculiarities and treatment outcomes of the spinal form of chronic non-bacterial osteomyelitis in children: a retrospective cohort study
    Rheumatol. Int. (IF 2.200) Pub Date : 2019-11-21
    Mikhail M. Kostik, Olga L. Kopchak, Alexey S. Maletin, Alexander Yu. Mushkin

    Chronic non-bacterial osteomyelitis (CNO) is a group of immune-mediated diseases which appears in bone inflammation, destruction and some orthopaedic consequences, especially in the cases of spinal involvement. This study is to compare characteristics and treatment outcomes of CNO patients with spinal involvement. The retrospective cohort study included data from 91 pediatric patients with CNO. The diagnosis is based on Jannson’s criteria with morphological confirmation (nonspecific chronic inflammation). Spine involvement detected by X-ray, computed tomography, magnetic resonance imaging, and bone scan in 29 (31.9%) patients. No differences in the family history, concomitant immune-mediated diseases between spinal (SpCNO) and peripheral (pCNO) forms of CNO have been revealed. Only 5 (10.2%) SpCNO patients (10.2%) had monofocal monovertebral involvement. The main risk factors of spinal involvement were female sex: RR = 2.0 (1.1; 3.9), sensitivity (Se) = 0.66, specificity (Sp) = 0.6; multifocal involvement: RR = 2.1 (0.9; 5.0), Se = 0.83, Sp = 0.37; no foot bones involvement: RR = 3.1 (1.3; 7.5), Se = 0.83, Sp = 0.5; sternum involvement RR = 2.3 (1.3; 4.1), Se = 0.24, Sp = 0.94. In the linear regression analysis only female sex (p = 0.005), multifocal involvement (p = 0.000001) and absence of foot bones involvement (p = 0.000001) were independent risk factors of spinal involvement (p = 0.000001). The response rate on bisphosphonates and tumor necrosis factor-a inhibitors was 90.9% and 66.7%, consequently. Only 4/29 (13.8%) SpCNO patients underwent surgery due to severe spinal instability or deformities. The spinal involvement is frequent in CNO and could be crucial for choosing a treatment strategy. Bisphosphonates and TNFa-inhibitors could be effective treatment options for severe SpCNO.

    更新日期:2020-01-04
  • Tofacitinib for the treatment for colchicine-resistant familial Mediterranean fever: case-based review
    Rheumatol. Int. (IF 2.200) Pub Date : 2019-12-07
    Hazan Karadeniz, Aslıhan Avanoğlu Güler, Nuh Atas, Hasan Satış, Reyhan Bilici Salman, Hakan Babaoglu, Abdurrahman Tufan

    Abstract Familial Mediterranean fever is characterized by self-limited attacks of serositis and arthritis. However, substantial number of patients suffer from chronic complications of this disease, primarily involving musculoskeletal system. Treatment for these complications is challenging due to limited evidence. Interleukin-1 (IL-1) antagonists, tocilizumab and anti-tumor necrosis factor (anti-TNF) agents are off-label treatment options for the management of chronic manifestations of FMF, such as secondary (AA) amyloidosis, chronic arthritis and sacroiliitis. This paper presents a case series of four FMF patients who are refractory to IL-1 antagonists, anti-TNF agents and tocilizumab, who responded well to tofacitinib. The authors also conducted a comprehensive literature search for studies investigating tofacitinib use in FMF patients. Although still limited, current data suggest that tofacitinib could be a useful treatment option for FMF patients with associated inflammatory comorbid conditions and chronic manifestations of disease.

    更新日期:2020-01-04
  • Evaluation of co-existing diseases in children with familial Mediterranean fever
    Rheumatol. Int. (IF 2.200) Pub Date : 2019-07-27
    Mehmet Yildiz, Amra Adrovic, Emre Tasdemir, Khanim Baba-zada, Muhammed Aydin, Oya Koker, Sezgin Sahin, Kenan Barut, Ozgur Kasapcopur

    Abstract Familial Mediterranean fever (FMF) is A common periodic fever syndrome. The causative gene of the FMF is named Mediterranean Fever gene (MEFV). Increased inflammation in FMF may play a role as a trigger for the development of some diseases. The objective of the study is to evaluate the frequency of comorbid disorders in children followed up with diagnosis of FMF. Additionally, we aimed to assess the association between FMF and other inflammatory conditions in a large pediatric FMF cohort. A total of 686 FMF patients were included in the cross-sectional study. A questionnaire including questions about characteristics of fever episodes, presence of arthralgia, arthritis, abdominal pain, chest pain during and co-existence of any other disease diagnosed by a physician was filled out by face-to-face interviews with patients or their parents. Female–male ratio was 0.85. Median age at the time of study, age at disease onset and at the time of diagnosis were 12.9 (1.7–22.3), 3 (0.08–17), and 6 (0.75–17) years, respectively. In 130 (18.9%) FMF patients we detected co-existing inflammatory condition. The most common co-existing diseases were: juvenile idiopathic arthritis 42 (6.1%), asthma/reactive airway disease 29 (4.2%), Henoch–Schönlein purpura 20 (2.9%), uveitis 12 (1.7%) and inflammatory bowel disease 10 (1.4%). Except for asthma/reactive airway disease and inflammatory bowel disease, there was no significant difference regarding the type of MEFV gene mutation. We have reported increased frequencies of various inflammatory conditions and decreased frequency of asthma in patients with FMF.

    更新日期:2020-01-04
  • Successful use of tocilizumab in amyloidosis secondary to systemic juvenile idiopathic arthritis
    Rheumatol. Int. (IF 2.200) Pub Date : 2019-07-04
    Ayush Gupta, Narendra Kumar Bagri, Saroj Kumar Tripathy, Adarsh Barwad, Ravi Hari Phulware, Pankaj Hari

    Abstract Amyloidosis secondary to juvenile idiopathic arthritis is a known complication of poorly controlled systemic juvenile idiopathic arthritis (SJIA), occurring in 1–2% of the patients. The IL-6 inhibitor tocilizumab is effective in controlling systemic signs and symptoms of sJIA and may be of therapeutic benefit in secondary amyloidosis. Herein, we report the clinical timeline of a 10-year boy with sJIA and secondary amyloidosis, who showed a sustained improvement of systemic symptoms and a reduction in proteinuria with tocilizumab. Compared to the data on adult patients affected with the secondary amyloidosis, there are very few reports on therapeutic options for the children affected with SJIA and secondary amyloidosis in the paediatric population. While doing a systematic literature search for writing this review, we could only retrieve nine case reports and one case series of the children affected with SJIA and secondary amyloidosis, including five cases which were treated with tocilizumab. We also looked into the clinical and biochemical response to various agents that have been used in the previous cases, including tocilizumab. The available literature and the present case report suggest that tocilizumab may be considered as a safe and effective option to treat SJIA-related secondary amyloidosis.

    更新日期:2020-01-04
  • Serum KL-6 level as a biomarker of interstitial lung disease in childhood connective tissue diseases: a pilot study.
    Rheumatol. Int. (IF 2.200) Pub Date : null
    Ayse Ayzit Kilinc,Asli Arslan,Mehmet Yildiz,Mine Kucur,Amra Adrovic,Kenan Barut,Sezgin Sahin,Haluk Cokugras,Ozgur Kasapcopur

    Krebs von den Lungen-6 (KL-6) has been described as a promising biomarker in the diagnosis and determining the severity of interstitial lung disease in adults with connective tissue disease. This study was performed to determine whether the serum KL-6 level is useful as a biomarker for detecting the interstitial lung disease (ILD) in pediatric cases of connective tissue disease (CTD). In total, 88 patients [36 patients with systemic juvenile idiopathic arthritis (sJIA), 27 patients with juvenile systemic lupus erythematosus (JSLE), 14 patients with juvenile dermatomyositis (JDM), and 11 patients with juvenile systemic sclerosis (JSSc)] and 68 healthy controls were included in this study. Age, sex, and duration of CTD and ILD (if any) were recorded. Blood samples from all the patients and controls were examined by ELISA. Eleven of the 88 patients with CTD (12.5%) had ILD and all of them were symptomatic. Subgroup analysis indicated that eight patients had JSSc, two had JSLE, and one had systemic JIA. The median serum KL-6 level was 1450.5 U/mL (interquartile range (IQR) 742.9-2603.2) in the CTD with ILD group, 415.9 U/mL (IQR 233.4-748.4) in the CTD without ILD group, and 465.9 U/mL (IQR 273.6-1036) in the control group. KL-6 levels were significantly higher in the CTD with ILD group than the CTD without ILD group and the control group (p = 0.003). At a cut-off of 712.5 U/ml identified by ROC curve, serum KL-6 yielded a sensitivity of 81% and specificity of 72% for CTD with ILD group. There was no significant difference in serum KL-6 level among the disease subgroups (sJIA, JSLE, JSSc, JDM), in either the CTD with ILD group or the CTD without ILD group (p > 0.05). In conclusion, KL-6 is a useful biomarker of CTD with ILD in pediatric patients.

    更新日期:2019-11-01
  • Platelet-rich plasma for thumb carpometacarpal joint osteoarthritis in a professional pianist: case-based review.
    Rheumatol. Int. (IF 2.200) Pub Date : 2019-10-15
    Ivan Medina-Porqueres,Pablo Martin-Garcia,Sofia Sanz-De Diego,Marcelo Reyes-Eldblom,Raquel Cantero-Tellez

    Thumb carpometacarpal osteoarthritis (TCMC-OA) is a progressively disabling, debilitating condition presenting with thumb base pain and hand functional impairment. Platelet-rich plasma has been used widely for the management of musculoskeletal pathologies, OA being among them. To our knowledge, only a few cases have been previously reported on this topic until now. A 59-year-old male professional pianist presented with chronic, mild onset of right thumb base pain involving a progressive lack of pinch strength in his right hand, and severe difficulties with playing. Three PRP injections were administered to the TCMC joint on a 1-week interval regime. Clinical outcomes were assessed by using the visual analog scale (VAS) for pain, grip and pinch strength, and the Quick-DASH Questionnaire. Functional outcome was excellent according to patient's capability with daily living activities and specific playing demands. At 12 months follow-up, no recurrences or complications were identified, with the musician returning to his previous level of performance 2 weeks before the end of this period. Patient self-reported satisfaction was high and he reported to return to his routine piano activity with no limitations. This case-based review study documents the clinical efficacy of PRP treatment from both functional and perceived-pain perspectives in a professional pianist. Presenting this case, our aim is to draw attention of healthcare providers dealing with TCMC-OA to PRP as a safe, beneficial therapy for this condition which needs further assessment in randomized controlled trials.

    更新日期:2019-11-01
  • Changing trends in mortality in systemic lupus erythematosus? An analysis of SLE inpatient mortality at University Hospital Coventry and Warwickshire NHS Trust from 2007 to 2016.
    Rheumatol. Int. (IF 2.200) Pub Date : 2019-10-02
    Himashi Anver,Shirish Dubey,James Fox

    The aim of this study was to determine the causes of mortality in patients with systemic lupus erythematosus (SLE) at the University Hospital Coventry and Warwickshire (UHCW) NHS Trust over a 10 year period. This was a retrospective study of patients who had died in UHCW NHS Trust between 2007 and 2016, where SLE or lupus was mentioned on the death certificate. Ethics approval was obtained from the Research and Development. We identified 22 patients out of 1979 admissions with SLE who had died during the period between 2007 and 2016, 7 of these patients were under 50 years of age. The leading cause of death was infection with pneumococcus being associated with two deaths. Active disease was associated with younger age at death. Median age at death was 58.5 years, with median duration of disease of 14.5 years. Constitutional and mucocutaneous features were the most common items scoring on disease activity, seen in 68.2% and 45.45%, respectively. We identified three patients with biopsy proven lupus nephritis and one patient with CNS lupus. Surprisingly, none of the patients died because of vascular problems. The study suggests a changing trend in SLE mortality with none of the deaths in this cohort being due to cardiovascular or cerebrovascular disease. Infection continues to be the biggest reason for mortality in this cohort and greater emphasis is needed on vaccination for preventable infections like pneumococcus.

    更新日期:2019-11-01
  • Magnetic resonance imaging assessment in patients with axial spondyloarthritis: development of checklists for use in clinical practice.
    Rheumatol. Int. (IF 2.200) Pub Date : 2019-09-20
    Raquel Almodóvar,Ángel Bueno,Enrique Batlle,Emma Beltrán-Catalán,Daniel Bernabeu,Carmen Castro Copete,Angela Cepero,Concha Crespo,Fernando Díez,Cristina Fernández-Carballido,Fran García Lorente,Angel Gil De Miguel,Xavier Juanola,Luis Linares,Rafael Montero Pérez-Barquero,Carmen Castro,Manuel José Moreno Ramos,Mireia Moreno,Victoria Navarro-Compán,Christopher Pack,Carlos Quiles,Maite Veintemillas,Pedro Zarco

    The objective of our study was to standardize magnetic resonance imaging (MRI) assessment of spine and sacroiliac joints in patients with axial spondyloarthritis (axSpA) and/or inflammatory spinal pain, by creating checklists and templates based on the opinions of rheumatologists and radiologists. A scientific committee developed a series of questionnaires with multiple items regarding MRI in patients with axial inflammatory pain and/or axSpA. Then an expert panel of rheumatologists and radiologists rated all items in a 9-point Likert scale. Finally, the scientific committee and the expert panel met to create the definitive documents. Several definitive checklists and templates were generated for rheumatologist-requested MRI and for radiologist-requested MRI reports of sacroiliac joint and spinal examinations. A technical requirement protocol was also agreed on. Our results could be useful in increasing understanding between rheumatologists and radiologists regarding MRI in axSpA diagnosis and follow-up.

    更新日期:2019-11-01
  • Rheumatologists' awareness of hepatitis B reactivation before immunosuppressive therapy.
    Rheumatol. Int. (IF 2.200) Pub Date : 2019-09-15
    Bilal Toka,Ahmet Tarik Eminler,Emel Gönüllü,Mukaddes Tozlu,Mustafa Ihsan Uslan,Erkan Parlak,Oğuz Karabay,Aydin Seref Koksal

    HBV reactivation (HBVr) is a well-known complication of immunosuppressive (IS) treatment. The aim of this study was to evaluate the awareness of rheumatologists about the risk of HBVr. A survey was sent via e-mail to 270 members of the Turkish Society for Rheumatology. It consisted of fourteen questions on their awareness of the major society guidelines, approach to hepatitis B virus (HBV) screening according to different IS regimens, decision process in screening patients for HBV, knowledge of antiviral treatments for HBV, follow-up strategies, experience and postgraduate training on HBVr. Forty-eight (17.8%) rheumatologists responded to the survey. Of the respondents, 93.8% reported that they screened all patients before IS treatment, while 6.2% screened patients with a high risk of HBV infection only. The screening rate was 95.8% (46/48) in patients undergoing high-risk IS treatment and 35.4% (17/48) in those undergoing low-risk treatment. All respondents screened for HBsAg, and 83.3% (40/48) screened for anti-HBc IgG and anti-HBs. Forty-four (91.7%) rheumatologists had previously initiated antiviral prophylaxis, and 14 (29.2%) had detected HBVr in at least one patient. Rheumatologists had a high awareness of the necessity for HBV screening before IS treatment. However, the screening rates were still lower than desired, especially in patients receiving IS treatments with moderate or low risk of reactivation.

    更新日期:2019-11-01
  • The relation between upper extremity joint involvement and grip force in early rheumatoid arthritis: a retrospective study.
    Rheumatol. Int. (IF 2.200) Pub Date : 2019-09-09
    Maria Rydholm,Ingegerd Wikström,Sofia Hagel,Lennart T H Jacobsson,Carl Turesson

    To investigate the relation between joint involvement in the upper extremities and grip force in patients with early rheumatoid arthritis (RA). An inception cohort of 225 patients with early RA was followed according to a structured protocol. The same rheumatologist assessed all patients for swollen joints and joint tenderness. Grip force was measured (Grippit; AB Detektor, Gothenburg, Sweden) at the same visit. Average grip force values for the dominant hand were expressed as % of expected, based on age- and sex-specific reference values from the literature. Associations between grip force and current synovitis or tenderness of individual joints, and other disease parameters measured at the same visit, were examined. Patients with current synovitis of the wrist joint or ≥ 1 metacarpophalangeal (MCP) joint of the dominant hand had a significantly lower grip force at inclusion, at 1 year and at 5 years. Proximal interphalangeal joint tenderness and MCP joint tenderness were consistently associated with reduced grip force. In multivariate analysis, extensive MCP joint synovitis was associated with lower grip force at inclusion (β - 2.8% per joint; 95% CI - 5.3 to - 0.4), and also at the 1-year follow-up. Patient reported pain scores and erythrocyte sedimentation rates had independent negative associations with grip force at all time points. In patients with early RA, extensive synovitis of the MCP joints was associated with reduced grip force, independently of other upper extremity joint involvement. Pain and inflammation have effects on hand function beyond those mediated by local synovitis.

    更新日期:2019-11-01
  • Computed diffusion-weighted imaging for differentiating synovial proliferation from joint effusion in hand arthritis.
    Rheumatol. Int. (IF 2.200) Pub Date : 2019-08-29
    Yuki Tanaka,Motoshi Fujimori,Koichi Murakami,Hiroyuki Sugimori,Nozomi Oki,Takatoshi Aoki,Tamotsu Kamishima

    The objective of this study is to investigate computed DWI (cDWI) as an alternative method to contrast-enhanced MRI in comparison with directory measured DWI (mDWI) and apparent diffusion coefficient (ADC) for differentiating synovial proliferation from joint effusion. Nine patients suspected with RA (5 women) were included in this study. A radiologist identified region of interest (ROI) based on STIR, and evaluated using a 5-point grading scale of 0 (fluid) to 4 (synovial proliferation) according to the degree of contrast enhancement within the ROI. cDWI was synthesized for b values from 1000 to 2000 at 200 s/mm2 intervals using the combination of b values at mDWI. In addition to ADC values, contrast ratios were calculated using signal intensity for each ROI on the mDWI and cDWI. Visual assessment by a radiologist was conducted between pairs of STIR image and mDWI or cDWI. ROI grades were most significantly correlated with cDWI2000 based on b values of 400-1000 s/mm2 (rs = 0.405, p < 0.01). The area under the curve of cDWI2000 based on b values of 400-1000 s/mm2 (0.762) was larger than that of ADC values (0.570-0.608) when comparing low versus high contrast enhancement grades. Both cDWI1800 (200-1000) and cDWI2000 (400-1000) demonstrated high sensitivity and specificity in visual assessment (84.6% and 66.7%, respectively). The cDWI2000 based on b values of 400-1000 s/mm2 may be useful for noninvasive differentiation of synovial proliferation from joint effusion in hand arthritis.

    更新日期:2019-11-01
  • Isotretinoin-induced sacroiliitis in patients with hidradenitis suppurativa: a case-based review.
    Rheumatol. Int. (IF 2.200) Pub Date : 2019-08-29
    Belkıs Nihan Coskun,Burcu Yagiz,Yavuz Pehlivan,Ediz Dalkilic

    Hidradenitis suppurativa (HS) is a chronic, suppurative skin disease characterized by painful nodules, particularly in the axillae and groin. Isotretinoin can be used in the treatment of HS; however, it may paradoxically lead to skin lesions or worsen the existing lesions. Isotretinoin, which is commonly used in the treatment of severe acne, is associated with several side effects, including rheumatic side effects and rarely sacroiliitis. In this study, we discussed two cases who presented with low back pain after isotretinoin was used for the treatment of acne vulgaris. The patients did not have low back pain before isotretinoin use and did not have enthesitis, dactylitis, uveitis, psoriasis, recent infection, trauma, and family history spondylitis. HLA-B27 was negative. Bone-marrow edema was detected at the sacroiliac joint on magnetic resonance imaging. Because of these findings, sacroiliitis related to the drug was considered in our patients and isotretinoin treatments were discontinued. Because the patients' low back pain continued when they administered non-steroidal anti-inflammatory drugs, biological drug treatments were started. Both cases presented had a simultaneous HS lesion. After the treatment, both low back pain and HS lesions regressed. Patients with isotretinoin therapy should be alerted for inflammatory low back pain and HS lesions that may develop. We should note that biologic agents should be considered in the treatment of resistant cases.

    更新日期:2019-11-01
  • Association of biomarkers of inflammation and HLA-DRB1 gene locus with risk of developing rheumatoid arthritis in females.
    Rheumatol. Int. (IF 2.200) Pub Date : 2019-08-28
    Biljana Klimenta,Hilada Nefic,Nenad Prodanovic,Radivoj Jadric,Fatima Hukic

    Rheumatoid arthritis (RA) is an autoimmune disease causing chronic inflammation of the joints. Multiple factors, including HLA-DRB1 gene variants, influence the susceptibility to RA. The HLA-DRB1 gene is part of a family of genes called the human leukocyte antigen (HLA) complex. In this study, we compared the inflammatory biomarkers values, including erythrocyte sedimentation rate (ESR) and C-reactive protein (CRP), between patients with RA and healthy control group of females of the Public Institution Health Centre of Sarajevo Canton. In addition, we estimated the frequencies of the HLA-DRB1 gene variants and their association with the risk for RA development in females. The haematological and biochemical tests were completed on automated analyzers. To assess the association between the HLA-DRB genes and the risk of RA in females, low-resolution genotyping of the HLA-DRB1, DRB3, DRB4, and DRB5 gene loci was performed by the sequence-specific polymerase chain reaction method (PCR-SSP). ESR and CRP were the most sensitive acute-phase reactants in females with RA and there was a correlation between ESR and CRP values in RA patients. There was significantly positive association between of the HLA-DRB1*03, *04, *08, *10, *11, and *14 variants and elevated values of ESR in RA patients, but negative between HLA-DRB1*03, *13 and *15 alleles and elevated CRP values. Furthermore, our results confirm genetic susceptibility to RA in a female population to the members of the HLA-DRB1*04 and *03 allelic groups, the DRB1*04/DRB1*04 and DRB1*03/DRB1*04 genotypes, and the DRB1*04-DRB4* or DRB1*03-DRB3* haplotypes, which, therefore, represent risk factors for the development of this disease. According to our results, the DRB1*01/DRB1*15 and DRB1*07/DRB1*16 genotypes and the HLA-DRB5 gene locus represent a protective factor for RA. The presence of specific HLA-DRB1 gene variants increases the risk of developing RA, while other variants provide protection against disease. Therefore, HLA typing could be helpful in the prediction of RA development and establishing and confirming a definitive diagnosis of autoimmune diseases in some subjects. A strong association with the higher levels of ESR and CRP could be used to establish definitive diagnosis and introduce of early treatment of RA to prevent the occurrence of RA symptoms.

    更新日期:2019-11-01
  • Potential clinical utility of a novel optical tomographic imaging for the quantitative assessment of hand rheumatoid arthritis.
    Rheumatol. Int. (IF 2.200) Pub Date : 2019-08-23
    Dong Jin Go,Sang Jin Lee,Sang Hyun Joo,Gi Jeong Cheon,Sung Hwan Hong,Yeong Wook Song

    Optical tomographic imaging (OTI) was reported to be a novel technique for the early diagnosis and disease activity assessment of rheumatoid arthritis (RA). This study aimed to evaluate the clinical utility of OTI for the detection of hand synovitis of RA patients. Manu-scan was used to perform imaging targeting the proximal interphalangeal (PIP) and metacarpophalangeal (MCP) joints in 12 RA patients and three controls. The enrolled RA patients also underwent magnetic resonance imaging (MRI) and bone scintigraphy (BS) to provide reference images. Of the 181 joints feasible for OTI analysis, 140 joints (111 in RA patients and 29 in controls, 77.3%) in which the difference of the OTI indices in the two measurements was within 20% were evaluated. The OTI indices in RA joints were significantly lower than those in control joints (p < 0.001). Overall, the OTI indices in RA joints decreased as the synovitis grades on MRI or BS increased. Moreover, OTI was able to discriminate between RA and control joints (AUC = 0.815, 95% CI 0.739-0.891), even if RA joints were normal on physical examination (AUC = 0.714, 95% CI 0.594-0.834). OTI was in good agreement (kappa = 0.60) with MRI for evaluating synovitis in RA patients and showed positive results in 11.4% of clinically asymptomatic joints. OTI in this study showed the potential to be a supplementary imaging modality for the quantification of synovial inflammation in PIP and MCP joints of RA patients. Further large-scale trials are needed to confirm these findings.

    更新日期:2019-11-01
  • Cervical proprioceptive impairment in patients with rheumatoid arthritis.
    Rheumatol. Int. (IF 2.200) Pub Date : 2019-08-23
    Firat Ulutatar,Cagri Unal-Ulutatar,Mehmet Tuncay Duruoz

    Rheumatoid arthritis (RA) involving the cervical spine can lead to various neurologic defects and impairment of proprioception is just one of them. The aim of this study was the assessment of cervical proprioception and its relation with radiographic, clinical, and functional characteristics of patients with RA. One hundred and six rheumatoid arthritis patients who diagnosed according to the 2010 American College of Rheumatology/European League Against Rheumatism criteria and age, gender, educational status matched one hundred and six healthy volunteers were enrolled in this study. Cervical joint position error test (CJPET) was applied to healthy volunteers and RA patients for cervical proprioception assessment. Fatigue, depression, balance, quality of life and balance scales were administered to all patients. Cervical radiography was used to assess cervical subluxations. Regression analysis was used for grading the factors which had relations with cervical proprioception. Mean age of patients and healthy volunteers was 51 ± 11.1 and 48.9 ± 9.2, respectively. Scores of CJPET were statistically significantly higher in RA group than healthy volunteers (p = 0.001). CJPET scores were negatively correlated with Berg balance scale findings in right rotation, left rotation, flexion and extension (rho = - 0.421,- 0.473,- 0.448,- 0.515). There was weak or not significant correlation between the scores of CJPET and fatigue, depression, and quality of life scales. Scores of CJPET in patients with atlantoaxial subluxations (AAS) were statistically significantly higher than those without AAS (p < 0.05). Regression analysis results showed that the AAS was related to impaired cervical proprioception on right and left rotations. There was no correlation between CJPET scores and functional parameters. Cervical proprioception impaired in RA patients. This impairment was related to the existence of AAS and balance problems.

    更新日期:2019-11-01
  • Measuring inflammation in rheumatoid arthritis with a new clinical and ultrasound index: development and initial validation.
    Rheumatol. Int. (IF 2.200) Pub Date : 2019-08-10
    Juan José de Agustín,Alba Erra,Andrés Ponce,Carmen Moragues,Cesar Díaz-Torné,Delia Reina,Estefanía Moreno,Julio Ramírez,Lourdes Mateo,Manel Pujol Busquets,Patricia Moya,Pilar Santo-Panero,Sergi Ros-Expósito,Javier Narváez,Raimon Sanmartí,Mª Jesús García de Yébenes,Loreto Carmona

    With the aim to develop and validate a clinical + ultrasound (US) inflammation score in rheumatoid arthritis (RA) for use in clinical practice, a mixed-method study was conducted. The theoretical development of the index was achieved with qualitative methodology (discussion group and Delphi survey). Subsequently, a cross-sectional study was carried out to analyse issues related to scoring and validation of the new index. RA patients underwent clinical [28 swollen and tender joints count, patient and physician global assessment (PhGA), erythrocyte sedimentation rate (ESR), C-reactive protein (CRP)], and US assessments [synovitis or tenosynovitis by grey-scale (GS) and power Doppler (PD) of 42 structures]. An index was created based on statistical models and expert interaction. Construct validity was tested by correlation with DAS28, SDAI, CDAI, and PhGA. Reliability was evaluated in a subgroup of patients with the intraclass correlation coefficient (ICC). US assessment, CRP, and swollen joints were the items that passed the prioritization phase (Delphi study). For the cross-sectional study, 281 patients were randomly divided into design (n = 141) and validation samples (n = 140). The combination of US sites chosen (7 bilaterally) detected the maximum proportion of synovitis and PD present. Three scoring methods were tested: semiquantitative (0-3 GS + 0-3 PD), dichotomous (0/1 GS + 0/1 PD), and qualitative (0/1 based on algorithm). All showed strong correlation with activity measures (ρ ≥ 0.60), and reliability (ICC 0.89-0.93). The index with best parameters of validity, feasibility, and reliability was the qualitative. The final index chosen was the sum of swollen joint count, US qualitative score, and CRP. The UltraSound Activity score is a valid and reliable measure of inflammation in RA equal to the sum of 28 SJC, a simplified (0/1) US assessment of 11 structures and CRP. It is necessary further investigation to demonstrate additional value over existing indices.

    更新日期:2019-11-01
  • Clinical and therapeutic management of rheumatoid arthritis with biological disease-modifying antirheumatic drugs: RADAR study.
    Rheumatol. Int. (IF 2.200) Pub Date : 2019-08-10
    Antonio Gomez-Centeno,Esteban Rubio-Romero,Juan Gabriel Ovalles,Sara Manrique-Arija,Sara Marsal-Barril,Juan Amarelo-Ramos,Javier Del Pino-Montes,Santiago Muñoz-Fernández,Sagrario Bustabad,Ceferino Barbazán-Álvarez

    To describe the clinical and therapeutic management of rheumatoid arthritis (RA) patients with biological disease-modifying antirheumatic drugs (bDMARDs), alone or in combination with conventional synthetic DMARDs (csDMARDs), as well as analysing changes over time in bDMARD use. An observational, retrospective, multicentre study was conducted in the rheumatology departments of 10 public Spanish hospitals. Patients with RA treated with bDMARDs at baseline who had medical records available in the data collection period 2013-2016 were included. All visits to rheumatology departments recording any type of bDMARD modification (dose, etc.) were collected. Clinical characteristics, concomitant treatment, resource use, work productivity and quality of life (QoL) were recorded. 128 patients were included: 81 received first-line bDMARD treatment, 28 second-line bDMARD treatment and 19 received third or later lines. Mean study follow-up was 4.1 years. Assessment of DAS28 was available in 54.6% of visits. At baseline, 48.7% of patients had moderate-high disease activity. At final observation, 69.5% of patients continued with the first bDMARD. Tumour necrosis factor blockers were administered to 85.2% of patients in first line, 45.7% in second line and 18.1% in third or later lines. At final observation, 80.2% of patients still felt pain/discomfort. As expected, those with higher disease activity had higher loss of work productivity and lower QoL, as assessed by DAS28, than patients with lower disease activity. Drugs represented 82.6% of the total cost. In this Spanish cohort of 128 patients, most patients remained on the first prescribed bDMARD, despite remaining signs and symptoms.

    更新日期:2019-11-01
  • Brucellosis as a rare cause of olecranon bursitis: case-based review.
    Rheumatol. Int. (IF 2.200) Pub Date : 2019-08-08
    Seyed Mokhtar Esmaeilnejad-Ganji,Mohammad Reza Hasanjani Roushan,Soheil Ebrahimpour,Arefeh Babazadeh

    A 51-year-old man shepherd presented with mild pain and swelling of the right posterior aspect of his right elbow. In ultrasonography, the affected bursal space had swelling and effusion. Moreover, the aspiration of the affected bursa revealed an inflammatory profile. Brucella melitensis was detected in aspirated fluid and blood cultures. The serum agglutination test (SAT) and 2-mercaptoethanol test for brucellosis were also positive. Therefore, the diagnosis of brucellar olecranon was confirmed. Treatment was initiated using gentamicin for the first 7 days and doxycycline plus rifampicin for 2 months. After treatment, all clinical signs and symptoms were resolved. No relapse was seen after 1 year of the completion of treatment. Clinicians should pay attention to the symptoms of olecranon brucellar bursitis that is similar to that of pyogenic bursitis.

    更新日期:2019-11-01
  • Trunk position sense, postural stability, and spine posture in fibromyalgia.
    Rheumatol. Int. (IF 2.200) Pub Date : 2019-08-02
    Seyda Toprak Celenay,Oguzhan Mete,Ozge Coban,Deran Oskay,Sukran Erten

    This study aimed to investigate trunk position sense, postural stability, and spine posture in women with fibromyalgia syndrome (FMS). Fifteen (15) women with FMS and age- and gender-matched fifteen (15) healthy controls were included. Trunk position sense as indicated by trunk reposition errors (TRE) and spine posture (thoracic and lumbar curvature) was measured with a digital inclinometer. Postural stability [eyes open (EO) and eyes closed (EC) on bipedal stance (BS), EO on monopedal stance (MS), and limits of stability (LOS)] was assessed with a computerized stabilometer (Prokin, TecnoBody S.R.L., Dalmine, 24044 Bergamo, Italy). TRE (p = 0.002) and the angle of thoracic curvature (p = 0.009) were found higher in women with FMS compared to healthy controls; however, the angle of lumbar curvature was similar (p = 0.467). It was seen that women with FMS had higher anterior-posterior sway in EO-BS (p = 0.009) and EC-BS (p = 0.001), ellipse area in EC-BS (p = 0.015), EO-MS of the dominant side (p = 0.021), and EO-MS of the non-dominant side (p = 0.007), and medial-lateral sway in EO-MS of the dominant (DM) side (p = 0.004) and the non-dominant (NDM) side (p = 0.002). Ellipse area in EO-BS (p = 0.054), medial-lateral sway in EO-BS (p = 0.983) and EC-BS (p = 0.290), anterior-posterior sway in EO-MS of the DM (p = 0.059) and the NDM side (p = 0.065), and LOS did not differ between groups (p = 0.274). Women with FMS had poor trunk position sense and postural instability, and alterations in spine posture. Therefore, the training of trunk position sense, postural stability, and posture might be beneficial and, thus, should be considered while planning an optimal treatment.

    更新日期:2019-11-01
  • Intermetatarsal bursitis as first disease manifestation in different rheumatological disorders and related MR-imaging findings.
    Rheumatol. Int. (IF 2.200) Pub Date : 2019-07-19
    Omar M Albtoush,Theodoros Xenitidis,Marius Horger

    Metatarsalgia defined as pain at the plantar aspect of the forefoot. Intermetatarsal bursitis is considered one potential soft-tissue cause of metatarsalgia that is presumably under-estimated, under-investigated, and, consequently, often misdiagnosed. To assess the role of MRI in the elucidation of the cause of metatarsalgia in patients with different autoimmune disorders presenting primarily with this symptom and to present the accompanying clinical and radiological findings of intermetatarsal bursitis. Retrospective evaluation of the medical records of patients with different rheumatological conditions claiming primarily of pedal pains suggests metatarsalgia and who underwent, therefore, all magnetic resonance imaging between March 2010 and April 2018. Of them, six patients fulfilled these criteria and were diagnosed subsequently with intermetatarsal bursitis. Several underlying autoimmune conditions were diagnosed. All patients were clinically assessed by the squeeze test and radiologically investigated with MRI; three patients underwent additional sonography. All patients presented intermetatarsal bursitis as first disease manifestation. The number of involved bursae ranged from one to three on one side. The main MR findings were distension of the intermetatarsal bursa with increased signal intensity on T2-weighted and post-contrast fat saturation T1-weighted images. Most frequent locations were the second and third intermetatarsal spaces. The size of the intermetatarsal bursitis and its plantar extension were correlated in all patients. Intermetatarsal bursitis can potentially be the first manifestation of different rheumatological diseases. Awareness of this potential association as well as cognizance of its imaging findings can help for making a more accurate and prompt earlier diagnosis of the underlying disease changing also the therapeutic approach.

    更新日期:2019-11-01
  • Renal flare in class V lupus nephritis: increased risk in patients with tubulointerstitial lesions.
    Rheumatol. Int. (IF 2.200) Pub Date : 2019-07-08
    Oh Chan Kwon,Yong Mee Cho,Ji Seon Oh,Seokchan Hong,Chang-Keun Lee,Bin Yoo,Yong-Gil Kim

    The objective of this study is to investigate the risk factors of renal flare in patients with membranous lupus nephritis (class V lupus nephritis). Biopsy-proven pure membranous lupus nephritis patients diagnosed between January 1997 and September 2017 were studied. We assessed and compared the clinical and pathological parameters between patients who experienced renal flare and those who did not. To identify risk factors of renal flare, multivariable Cox proportional hazard regression analysis was performed. Out of the 53 patients with pure membranous lupus nephritis, 17 patients (32.1%) experienced renal flare during a median follow-up of 121.5 months (range 44.4-196.9). Patients who experienced renal flare had significantly higher proportion of tubulointerstitial inflammation (76.5% vs. 36.1%, p = 0.006) and tubular atrophy/interstitial fibrosis (70.6% vs. 27.8%, p = 0.003) at baseline. In multivariable Cox proportional hazard regression analysis, the presence of tubulointerstitial inflammation [adjusted hazard ratio (HR) 5.532, 95% confidence interval (CI) 1.722-17.776, p = 0.004] and tubular atrophy/interstitial fibrosis (adjusted HR 4.328, 95% CI 1.450-12.916, p = 0.009) at baseline was significantly associated with increased risk of renal flare. The presence of tubulointerstitial inflammation and tubular atrophy/interstitial fibrosis is associated with increased risk of renal flare in patients with membranous lupus nephritis.

    更新日期:2019-11-01
  • Study of familial aggregation of autoimmune rheumatic diseases in Asian Indian patients with systemic lupus erythematosus.
    Rheumatol. Int. (IF 2.200) Pub Date : 2019-07-03
    Arvind Ganapati,Gautham Arunachal,Suvrat Arya,Devika Shanmugasundaram,Lakshmanan Jeyaseelan,Sathish Kumar,Sumita Danda,Debashish Danda

    Systemic lupus erythematosus (SLE) and other autoimmune rheumatic diseases (AIRD) tend to co-aggregate in families, making positive familial history a risk factor. We aimed to estimate familial aggregation of AIRD in SLE patients and to compare between ones having a positive and negative family history of autoimmunity in our cohort. We included families of 157 consecutive SLE patients in a hospital-based, cross-sectional design for a three-generation pedigree study. Clinical and laboratory parameters of these patients were recorded. AIRD was seen in families of 39 SLE patients amounting to a familial prevalence of 24.8% [95% confidence interval (CI) 18.1, 31.6] with a relative risk (λ) of 4.3 for first-degree relatives (FDRs) and 1.1 for second-degree relatives (SDRs). SLE was the commonest AIRD seen in families of 19 patients with a familial prevalence of 12.1% (95% CI 7.0, 17.2) and λ of 78.2 for FDRs and 18.1 for SDRs. AIRD as a whole and SLE alone were seen more commonly with parental consanguinity (p < 0.05). Familial aggregation in SLE patients also showed a relatively higher percentage of affected males and lesser presentation with constitutional features (p < 0.05) than sporadic SLE patients. Rheumatoid arthritis (RA) was the second most common AIRD seen in 16/39 (41%) families with a RR of 3.1 in FDRs of SLE patients. In conclusion, Asian Indian SLE patients seem to have a high familial aggregation of AIRD, which is more pronounced in the background of parental consanguinity. SLE is the commonest AIRD seen amongst FDRs and SDRs of SLE patients, followed by RA, with FDRs being at highest risk.

    更新日期:2019-11-01
  • Consider the wrist: a retrospective study on pediatric connective tissue disease with MRI.
    Rheumatol. Int. (IF 2.200) Pub Date : 2019-06-22
    Charlotte M Nusman,J Merlijn van den Berg,Amara Nassar-Sheikh Rashid,Katerina Ntailiani,Apostolos Karantanas,Taco W Kuijpers,Mario Maas,Dieneke Schonenberg-Meinema

    The aim of this study is to describe the clinical characteristics and MRI findings of the wrist in a cohort of children suffering from connective tissue disease with musculoskeletal involvement. Ten patients with pediatric connective tissue disease [median age 14.7 years (IQR 12.7-16.6 years), 70% female] were identified from a large MRI database. Clinical findings during the disease course were retrospectively obtained from patient charts and findings at the time of MRI were prospectively registered in the MRI database. MRI wrist datasets were evaluated by three readers in consensus for synovitis, tenosynovitis, bone marrow changes, bone erosions and myositis. Patients suffered from connective tissue disease with clinical overlap of subtypes systemic lupus erythematosus, Sjögren syndrome and dermatomyositis. Median onset of disease was at 12.3 years (IQR 7.8-14.8 years). Clinical arthritis activity was scored low (median visual analogue scale physician 19, IQR 7-31). Notwithstanding, extensive inflammatory abnormalities such as synovitis and tenosynovitis were found in the wrist of 7/10 patients. Osteochondral involvement was detected in 3/10 patients. In a small cohort of children with connective tissue disease and musculoskeletal symptoms, severe inflammatory abnormalities of the involved wrist were present in the MRI, while clinical disease scores suggested mild disease activity. Therefore, clinicians should consider the wrist as vulnerable for joint damage and can add MRI as a helpful tool in the management of patients with pediatric connective tissue disease and musculoskeletal involvement.

    更新日期:2019-11-01
  • Successful arthroscopic treatment of refractory and complicated popliteal cyst associated with rheumatoid arthritis in combination with osteoarthritis: case series and literature review.
    Rheumatol. Int. (IF 2.200) Pub Date : 2019-04-13
    Jae-Hyuk Yang,Hyuk-Hee Kwon,Jin Kyu Lee,So Young Bang,Hye-Soon Lee

    Although popliteal cysts are most frequently identified in patients with osteoarthritis (OA), they may occur in patients with rheumatoid arthritis (RA), in which serious complicated cases such as cyst rupture can be developed. The objective of this study was to report four patients with RA (six knees) in combination with OA with a brief review of literature of previous similar published cases. This is a retrospective review of case records of patients with refractory and/or complicated popliteal cysts, who have successfully treated with arthroscopic intervention. We suggest that arthroscopic interventions such as radical debridement, synovectomy, biomechanical valve excision, and/or cystectomy should be considered in patients with refractory and complicated popliteal cysts associated with RA or RA in combination with OA.

    更新日期:2019-11-01
  • The impact of gender on time to rheumatoid arthritis classification: a retrospective analysis of a population-based cohort.
    Rheumatol. Int. (IF 2.200) Pub Date : 2019-07-25
    Caitrin M Coffey,John M Davis,Cynthia S Crowson

    The objective is to compare the time to fulfillment of 1987 and 2010 ACR/EULAR classification criteria between female and male patients with rheumatoid arthritis (RA) and to assess the potential impact of gender on the time to DMARD therapy. Time from first provider-documented joint swelling to fulfillment of 1987 and 2010 ACR/EULAR criteria was measured in a population-based cohort of adults with incident RA, who were stratified by male or female gender. Disease characteristics, time to start of DMARD therapy, and choice of initial DMARD were compared between groups. The study included 214 patients with RA (148 females and 66 males). Median times from first joint swelling to fulfillment of 1987 (6.5 vs 2.5 days, p = 0.48) and 2010 (1 vs 0 days, p = 0.34) classification criteria were not different between female and male patients overall. There was no difference in time to first DMARD therapy in female vs male patients (15.5 vs 16 days, p = 0.90), and methotrexate was used most frequently as first DMARD in both genders (61% female vs 64% male, p = 0.76). Among the 49 female and 20 male RF/ACPA-negative patients, females experienced a higher median time from first joint swelling to fulfillment of the 1987 (65 vs 11 days, p = 0.063) and 2010 (65 vs 0 days, p = 0.035) classification criteria. Overall, there was no significant delay in meeting 1987 and 2010 ACR/EULAR classification criteria between female and male RA patients, though time to meeting both 1987 and 2010 criteria was slightly longer in males compared with females. Among seronegative patients, females experienced a significant delay to meeting 2010 criteria from first clinically detected synovitis.

    更新日期:2019-11-01
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