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Multicentric carpotarsal osteolysis syndrome with variants of MAFB gene: a case report and literature review Pediatr Rheumatol. (IF 2.5) Pub Date : 2024-03-13 Xianfei Gao, Xiang Fang, Danping Huang, Song Zhang, Huasong Zeng
Multicentric carpotarsal osteolysis (MCTO) is a rare genetic disorder characterized by the progressive loss of bone in the hands, feet, and other skeletal structures. It presents with symptoms that may resemble those of juvenile idiopathic arthritis, making diagnosis challenging for clinicians. The identification of MAF BZIP Transcription Factor B (MAFB) mutations as significant contributors to MCTO
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Serum and salivary inflammatory biomarkers in juvenile idiopathic arthritis—an explorative cross-sectional study Pediatr Rheumatol. (IF 2.5) Pub Date : 2024-03-09 Lena Cetrelli, Anette Lundestad, Elisabet G. Gil, Johannes Fischer, Josefine Halbig, Paula Frid, Oskar Angenete, Annika Rosén, Karin B. Tylleskär, Keijo Luukko, Ellen Nordal, Anne N. Åstrøm, Marit S. Skeie, Astrid Kamilla Stunes, Athanasia Bletsa, Abhijit Sen, Astrid J. Feuerherm, Marite Rygg
Biomarkers may be useful in monitoring disease activity in juvenile idiopathic arthritis (JIA). With new treatment options and treatment goals in JIA, there is an urgent need for more sensitive and responsive biomarkers. We aimed to investigate the patterns of 92 inflammation-related biomarkers in serum and saliva in a group of Norwegian children and adolescents with JIA and controls and in active
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Gene association analysis to determine the causal relationship between immune cells and juvenile idiopathic arthritis Pediatr Rheumatol. (IF 2.5) Pub Date : 2024-03-08 Longhao Chen, Xingchen Zhou, Chao Yang, Hong Jiao Wu, Yu Tian, Shuangwei Hong, Huijie Hu, Kaizheng Wang, Shuang Wu, Zicheng Wei, Tao Li, Yuanshen Huang, Zihan Hua, Qiong Xia, Xiao Jie Chen, Zhizhen Lv, Lijiang Lv
Juvenile idiopathic arthritis (JIA) is a type of chronic childhood arthritis with complex pathogenesis. Immunological studies have shown that JIA is an acquired self-inflammatory disease, involving a variety of immune cells, and it is also affected by genetic and environmental susceptibility. However, the precise causative relationship between the phenotype of immune cells and JIA remains unclear to
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Musculoskeletal symptoms and their impact on health-related quality of life in chronic nonbacterial osteomyelitis patients Pediatr Rheumatol. (IF 2.5) Pub Date : 2024-03-06 Samar Tharwat, Mohammed Kamal Nassar
Chronic non-bacterial osteomyelitis (CNO) is a rare, non-infection- related inflammatory disorder that affects children and teens. Clinical manifestations of CNO range widely from moderate, time-limited, monofocal inflammation of the bone to extreme multifocal or chronically active inflammation of the bone. The main aim of this study was to explore the correlation between musculoskeletal (MSK) symptoms
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Effect of exercise training on heath, quality of life, exercise capacity in juvenile idiopathic arthritis: a meta-analysis of randomized controlled trials Pediatr Rheumatol. (IF 2.5) Pub Date : 2024-03-04 Wen-yu Liu, Hui-min Li, Hao Jiang, Wen-kui Zhang
Little is known about the efficacy and safety of exercise training on juvenile idiopathic arthritis (JIA). This study aims to investigate the effect of exercise on health, quality of life, and different exercise capacities in individuals with JIA. A comprehensive search of Medline, Embase, Web of Science, and the Cochrane Library was conducted from database inception to October, 2023. Included studies
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Th17/1 and ex-Th17 cells are detected in patients with polyarticular juvenile arthritis and increase following treatment Pediatr Rheumatol. (IF 2.5) Pub Date : 2024-03-02 Stephanie Wood, Justin Branch, Priscilla Vasquez, Marietta M. DeGuzman, Amanda Brown, Anna Carmela Sagcal-Gironella, Saimun Singla, Andrea Ramirez, Tiphanie P. Vogel
A better understanding of the pathogenesis of polyarticular juvenile idiopathic arthritis (polyJIA) is needed to aide in the development of data-driven approaches to guide selection between therapeutic options. One inflammatory pathway of interest is JAK-STAT signaling. STAT3 is a transcription factor critical to the differentiation of inflammatory T helper 17 cells (Th17s). Previous studies have demonstrated
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Profile of HLA-B27-positive enthesitis/spondylitis-related arthritis in Senegal, West Africa Pediatr Rheumatol. (IF 2.5) Pub Date : 2024-02-29 Mounib M. Sabounji, Aïssatou Ndiaye, Saïdou Diallo
Enthesitis/spondylitis-related arthritis (ERA) is a type of juvenile idiopathic arthritis (JIA) frequently associated with HLA-B27. In sub-Saharan Africa, HLA-B27-positive ERA hasn’t been the subject of a specific study. We aimed to describe the clinical features, disease activity, functional disability and treatment of HLA-B27-positive ERA at diagnosis in Senegal and compare the findings to other
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Increase in stress contributes to impaired jaw function in juvenile idiopathic arthritis: a two-year prospective study Pediatr Rheumatol. (IF 2.5) Pub Date : 2024-02-26 Alexandra Dimitrijevic Carlsson, Kerstin Wahlund, Erik Kindgren, Martina Frodlund, Per Alstergren
Stress in patients with Juvenile Idiopathic Arthritis (JIA) has been found to be associated with orofacial pain, psychological distress, jaw dysfunction and loss of daily activities in a cross-sectional study. The aim of this study was to investigate the relations between stress and change of stress over time versus changes in orofacial pain, psychosocial factors and jaw function over a two-year period
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Juvenile eosinophilic fasciitis: a single center case series Pediatr Rheumatol. (IF 2.5) Pub Date : 2024-02-24 Leigh A. Stubbs, Oluwaseun Ogunbona, Emily Beil, Vibha Szafron, Adekunle Adesina, Sara Anvari, Jamie Lai, Andrea Ramirez, Matthew G. Ditzler, Marietta DeGuzman
Eosinophilic fasciitis (EF) is a rare disease characterized by skin induration and musculoskeletal abnormalities. Diagnostic criteria for EF are based on adult populations. There is a need to expand the literature on EF in children due to limited reported cases and potential differences compared to adults. We conducted a retrospective review of medical records for six pediatric patients diagnosed with
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Resource utilization and costs of transitioning from pediatric to adult care for patients with chronic autoinflammatory and autoimmune disorders Pediatr Rheumatol. (IF 2.5) Pub Date : 2024-02-23 Daniela Choukair, Christian Patry, Ronny Lehmann, Dorothea Treiber, Georg F. Hoffmann, Corinna Grasemann, Normi Bruck, Reinhard Berner, Peter Burgard, Hanns-Martin Lorenz, Burkhard Tönshoff
A structured transition of adolescents and young adults with chronic autoinflammatory and autoimmune disorders from the pediatric to the adult health care system is important. To date, data on the time, processes, outcome, resources required for the necessary components of the transition process and the associated costs are lacking. Evaluation of resource use and costs in a prospective cohort study
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Correction: Engaging patients and parents to improve mental health intervention for youth with rheumatological disease Pediatr Rheumatol. (IF 2.5) Pub Date : 2024-02-16 Oluwatunmise A. Fawole, Michelle V. Reed, Julia G. Harris, Aimee Hersh, Martha Rodriguez, Karen Onel, Erica Lawson, Tamar Rubinstein, Kaveh Ardalan, Esi Morgan, Anne Paul, Judy Barlin, R. Paola Daly, Mitali Dave, Shannon Malloy, Shari Hume, Suzanne Schrandt, Laura Marrow, Angela Chapson, Donna Napoli, Michael Napoli, Miranda Moyer, Vincent Delgaizo, Ashley Danguecan, Emily von Scheven, Andrea Knight
Following publication of the original article [1], we have been notified that the third column heading in Tables 1, 2 and 3 was incorrect. Initially published heading: Patient Report. Corrected heading: Parent Report. The original article has been corrected. Fawole et al. Pediatric Rheumatology (2021) 19:19 https://doi.org/10.1186/s12969-021-00503-7 Download references Authors and Affiliations Children’s
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Algorithmic approach in the diagnosis of pediatric uveitis Pediatr Rheumatol. (IF 2.5) Pub Date : 2024-02-14 Farhad Salehzadeh
Uveitis is defined as an intraocular inflammation involving the iris, ciliary body, or choroid [1]. Uveitis is caused by disorders of diverse etiologies including wide spectrum of infectious and non-infectious causes. These entities can be categorized into three groups based on their etiologies: infectious, non-infectious, and masquerade syndrome [2]. “Infectious uveitis” including viruses, bacteria
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Body mass index is associated with health-related quality of life and disease characteristics in young adults with juvenile idiopathic arthritis Pediatr Rheumatol. (IF 2.5) Pub Date : 2024-02-02 Anna-Kaisa Tuomi, Katariina Rebane, Ellen Dalen Arnstad, Lillemor Berntson, Anders Fasth, Mia Glerup, Troels Herlin, Hannu Kautiainen, Ellen Nordal, Suvi Peltoniemi, Marite Rygg, Veronika Rypdal, Marek Zak, Kristiina Aalto
There is a growing interest concerning the relationship between obesity and several medical conditions and inflammation. Nevertheless, there is a lack of studies regarding body mass index (BMI) among patients with juvenile idiopathic arthritis (JIA). Our aim was to investigate the impact of BMI on health-related quality of life (HRQoL) measured with a 36-Item Short Form Survey (SF-36), disease activity
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Activated phosphoinositide 3-kinase δ syndrome caused by PIK3CD mutations: expanding the phenotype Pediatr Rheumatol. (IF 2.5) Pub Date : 2024-01-29 Peiwei Zhao, Juan Huang, Huicong Fu, Jiali Xu, Tianhong Li, Xiankai Zhang, Qingjie Meng, Lei Zhang, Li Tan, Wen Zhang, Hebin Chen, Xiaoxia Lu, Yan Ding, Xuelian He
Germline heterozygous gain-of-function (GOF) mutations in the PIK3CD gene lead to a rare primary immunodeficiency disease known as activated phosphoinositide 3-kinase (PI3K) δ syndrome type 1(APDS1). Affected patients present a spectrum of clinical manifestations, particularly recurrent respiratory infections and lymphoproliferation, increased levels of serum immunoglobulin (Ig) M, Epstein-Barr virus
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Kawasaki disease in neonates: a case report and literature review Pediatr Rheumatol. (IF 2.5) Pub Date : 2024-01-29 Mingjun Shen, Die Liu, Fang Ye, Jing Zhang, Jun Wang
Kawasaki disease (KD) is an acute systemic vasculitis of unknown etiology that affects infants and young children but is extremely rare in neonates, especially afebrile KD. We present a case of KD without fever in a neonate and review the literature on KD in neonates. A newborn female was hospitalized because her peripheral blood leukocytes increased for half a day. The admission diagnosis was considered
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Screening for juvenile idiopathic arthritis associated uveitis with laser flare photometry in the pediatric rheumatology office: a prospective observational study Pediatr Rheumatol. (IF 2.5) Pub Date : 2024-01-26 Kaleo Ede, Michael Shishov, Elisa Wershba, Nikita Goswami, Sabrina Gorry, Malin Joseph, Lucia Mirea, James O’Neil
Juvenile Idiopathic Arthritis (JIA) Associated Uveitis (JIA-U) remains one of the most serious complications of JIA in children. Historically, pediatric JIA is diagnosed by an Optometrist or Ophthalmologist; however, barriers to scheduling increase wait times that may delay diagnosis and treatment. The purpose of this study was to evaluate laser flare photometry (LFP) use to diagnose JIA-U in the Pediatric
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Rheumatological complaints in H syndrome: from inflammatory profiling to target treatment in a case study Pediatr Rheumatol. (IF 2.5) Pub Date : 2024-01-23 Alessandra Tesser, Erica Valencic, Valentina Boz, Gianluca Tornese, Serena Pastore, Manuela Zanatta, Alberto Tommasini
H Syndrome is a rare genetic condition caused by biallelic pathogenic variants in the SLC29A3 gene. It is characterized by a wide range of clinical manifestations, many of which are related to the immune-rheumatological field. These include scleroderma-like skin changes, deforming arthritis, and enlarged lymph nodes. The condition also features cardiac and endocrine defects, as well as hearing loss
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Lymphocyte apoptosis and its association with the inflammatory markers and disease severity in juvenile-onset systemic lupus erythematosus patients Pediatr Rheumatol. (IF 2.5) Pub Date : 2024-01-19 Eman Eissa, Rania Kandil, Dalia Dorgham, Raghda Ghorab, Naglaa Kholoussi
The defective clearance of apoptotic bodies in juvenile-onset systemic lupus erythematosus (jSLE) potentially leads to the persistence of autoreactive lymphocytes and the perpetuation of the autoimmune response. These factors contribute to the disturbance in lymphocyte apoptosis and show potential as key determinants in the clinical course and severity of jSLE. This study evaluates the role of peripheral
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Hypertension as a prominent manifestation secondary to renal artery lesions in pediatric Behcet’s disease Pediatr Rheumatol. (IF 2.5) Pub Date : 2024-01-19 Xinning Wang, Zhixuan Zhou, Jianguo Li, Gaixiu Su, Xiaohui Li
Hypertension caused by vascular Behcet’s disease (BD) is an important prognostic factor of paediatric BD. However, much less is known about its clinical features. The objective of this study was to investigate the clinical characteristics of paediatric vascular BD complicated by hypertension. A retrospective study was carried out in paediatric BD patients complicated by hypertension treated in the
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Establishment and analysis of a novel diagnostic model for systemic juvenile idiopathic arthritis based on machine learning Pediatr Rheumatol. (IF 2.5) Pub Date : 2024-01-19 Pan Ding, Yi Du, Xinyue Jiang, Huajian Chen, Li Huang
Systemic juvenile idiopathic arthritis (SJIA) is a form of childhood arthritis with clinical features such as fever, lymphadenopathy, arthritis, rash, and serositis. It seriously affects the growth and development of children and has a high rate of disability and mortality. SJIA may result from genetic, infectious, or autoimmune factors since the precise source of the disease is unknown. Our study
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Monogenic systemic lupus erythematosus onset in a 13-year-old boy with Noonan like-syndrome: a case report and literature review Pediatr Rheumatol. (IF 2.5) Pub Date : 2024-01-18 Patricia Morán-Álvarez, Alessandra Gianviti, Francesca Diomedi-Camassei, Monia Ginevrino, Fabrizio de Benedetti, Claudia Bracaglia
Childhood systemic lupus erythematosus (cSLE) has been considered as a polygenic autoimmune disease; however, a monogenic lupus-like phenotype is emerging with the recent recognition of several related novel high-penetrance genetic variants. RASopathies, a group of disorders caused by mutations in the RAS/MAPK pathway, have been recently described as a cause of monogenic lupus. We present a 13-year-old
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Psoriatic arthritis and COVID-19: a new challenge for rheumatologists and dermatologists Pediatr Rheumatol. (IF 2.5) Pub Date : 2024-01-18 Zohreh Jadali
COVID-19 has changed the global health system and has great impact on different types of medical specialties including, dermatology and rheumatology. This point is important because although these two specialties are distinct subfields of medicine, there is some overlap between them. The overlap can be described by a number of rheumatic diseases that have cutaneous manifestations and vice versa. A
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Shaping the future of pediatric rheumatology Pediatr Rheumatol. (IF 2.5) Pub Date : 2024-01-16 Tadej Avčin, Angelo Ravelli
Dear colleagues, It is with great pleasure and honor that we address you as the newly appointed Editors-in-Chief of Pediatric Rheumatology, an open access journal published by BioMed Central, a member of the Springer Nature publishing group. The journal has been serving the worldwide pediatric rheumatology community for more than two decades and was originally launched as the Pediatric Rheumatology
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Correction: Transition readiness among finnish adolescents with juvenile idiopathic arthritis Pediatr Rheumatol. (IF 2.5) Pub Date : 2024-01-15 Katriina Mikola, Katariina Rebane, Hannu Kautiainen, Kristiina Aalto
Following publication of the original article [1], we have been notified that the first and last author names have been swapped. Originally published names: Mikola Katriina, Rebane Katariina, Kautiainen Hannu and Aalto. Kristiina. Correct name order: Katriina Mikola, Katariina Rebane, Hannu Kautiainen and Kristiina Aalto. The original article has been corrected. Kohli, et al. Pediatr Rheumatol. 2023;21:149
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Longitudinal follow-up of mixed connective tissue disease and overlapping autoimmune diseases of childhood onset in the Afro-descendant population of the French West Indies Pediatr Rheumatol. (IF 2.5) Pub Date : 2024-01-11 Arthur Felix, Lindsay Osei, Frederique Delion, Benoit Suzon, Aurore Abel, Moustapha Drame, Yves Hatchuel, Christophe Deligny, Fabienne Louis-Sidney
Overlap autoimmune syndromes (OAS) and mixed connective tissue disease (MCTD) are rare in children. We performed a retrospective, longitudinal and descriptive study of Afro-Caribbean patients from the French West Indies followed for MCTD and OAS to describe their characteristics and outcomes during childhood. Retrospective study from January 2000 to 2023. Listings of patients were obtained from multiple
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The 4th NextGen Therapies for SJIA and MAS: part 1 the elephant in the room: diagnostic/classification criteria for systemic juvenile idiopathic arthritis and adult-onset still’s disease Pediatr Rheumatol. (IF 2.5) Pub Date : 2024-01-05 Peter A. Nigrovic, Fabrizio de Benedetti, Yukiko Kimura, Daniel J. Lovell, Sebastiaan J. Vastert
Currently, the criteria used to classify patients with SJIA are different from those used for AOSD. However, it has been recognized that the existing terms are too narrow, subdividing the Still’s population unnecessarily between pediatric-onset and adult-onset disease and excluding an appreciable group of children in whom overt arthritis is delayed or absent. Government regulators and insurers rely
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Proceedings from the 4th NextGen Therapies for SJIA and MAS virtual symposium held February 13–14, 2022 Pediatr Rheumatol. (IF 2.5) Pub Date : 2024-01-05 Rashmi Sinha, Fabrizio De Benedetti, Alexei A. Grom
Refractory Systemic Juvenile Idiopathic Arthritis (SJIA) remains a major source of morbidity and mortality in children with pediatric rheumatic disease [1, 2]. Although several potential therapeutic targets have been recently identified, only few patients have been enrolled in ongoing clinical trials. The 4th “NextGen Therapies for SJIA and MAS” virtual symposium organized by the “SJIA Foundation”
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The 4th NextGen therapies for SJIA and MAS: part 2 phenotypes of refractory SJIA and the landscape for clinical trials in refractory SJIA Pediatr Rheumatol. (IF 2.5) Pub Date : 2024-01-05 Grant Schulert, Sebastiaan J. Vastert, Alexei A. Grom
Although the introduction of the IL-1 and IL-6 inhibiting biologics in 2012 has revolutionized the treatment and markedly improved outcomes for many patients with SJIA, about 20% of these patients continue to have active disease, have markedly decreased quality of life and high disease activity as well as treatment-related morbidity and mortality. There is a clear need to define these disease states
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Part 5: Allogeneic HSCT in refractory SJIA with lung disease; recent cases from centers in North America & Europe Pediatr Rheumatol. (IF 2.5) Pub Date : 2024-01-05 Alexei A. Grom, Scott W. Canna, Rolla F. Abu-Arja, Rashmi Sinha, Luciana Peixoto, Elvira Cannizzaro, Shanmuganathan Chandrakasan, Kyla Driest, Rebecca Marsh, Bénédicte Neven, Karen Onel, Sampath Prahalad, Susan Prockop, Pierre Quartier, Johannes Roth, Grant Schulert, Juliana M.F. Silva, Donna Wall, Ulrike Zeilhofer
It has been increasingly recognized that there is a subset of patients with refractory systemic JIA, who have failed all available medications and may benefit from HSCT. The increasing experience with HSCT in SJIA, suggests that despite the complicated post-HSCT course, short-term, the transplanted patients either achieved SJIA remission or reduced burden of disease. Longer follow-up, however, is needed
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The 4th NextGen therapies of SJIA and MAS, part 4: it is time for IL-18 based trials in systemic juvenile idiopathic arthritis? Pediatr Rheumatol. (IF 2.5) Pub Date : 2024-01-05 Scott W. Canna, Fabrizio De Benedetti
Since IL-18 has recently emerged as a biomarker associated with refractory disease course in SJIA, the focus of the discussion was the feasibility of the biomarker-driven drug development to SJIA. Overall, there was broad agreement on the conclusion that IL-18 is a uniquely specific biomarker for many of the subsets of SJIA most in need of new therapies, and it may define a class of diseases mediated
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A20 haploinsufficiency in a neonate caused by a large deletion on chromosome 6q Pediatr Rheumatol. (IF 2.5) Pub Date : 2024-01-05 Fan Zhang, Liang Zhang
Haploinsufficiency of A20 (HA20) is a rare monogenic disease caused by heterozygous loss-of-function mutations in the tumor necrosis factor alpha-induced protein 3 (TNFAIP3) gene located on chromosome 6q23.3. The majority of disease-causing mutations in most cases of HA20 comprise single nucleotide variations, small insertions, or deletions in TNFAIP3, which result in a premature termination codon
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Tocilizumab for treating mevalonate kinase deficiency and TNF receptor-associated periodic syndrome: a case series and literature review Pediatr Rheumatol. (IF 2.5) Pub Date : 2024-01-05 Yandie Li, Meiping Lu
Mevalonate kinase deficiency (MKD) and TNF receptor-associated periodic syndrome (TRAPS) are categorized as systemic autoinflammatory diseases (SAIDs), which are rare diseases characterized by early onset, severe conditions, and challenging diagnosis and treatment. Although different SAIDs have varying standard treatments, some SAIDs are poorly controlled after routine treatment, seriously affecting
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Mental comorbidities in adolescents and young adults with juvenile idiopathic arthritis: an analysis of German nationwide health insurance data Pediatr Rheumatol. (IF 2.5) Pub Date : 2024-01-05 Florian Milatz, Katinka Albrecht, Kirsten Minden, Ursula Marschall, Jens Klotsche, Johanna Callhoff
Studies on prevalence rates of mental comorbidities in patients with juvenile idiopathic arthritis (JIA) have reported varying results and provided limited information on related drugs. The purpose of this study was to determine the prevalence of selected mental health diagnoses and the range of associated drug prescriptions among adolescents and young adults (AYA) with JIA compared with general population
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A rare manifestation of STING-associated vasculopathy with onset in infancy: a case report Pediatr Rheumatol. (IF 2.5) Pub Date : 2024-01-04 Sophia Weidler, Sarah Koss, Christine Wolf, Nadja Lucas, Jürgen Brunner, Min Ae Lee-Kirsch
STING-associated vasculopathy with onset in infancy (SAVI) is a rare type I interferonopathy caused by heterozygous variants in the STING gene. In SAVI, STING variants confer a gain-of-function which causes overactivation of type I interferon (IFN) signaling leading to autoinflammation and various degrees of immunodeficiency and autoimmunity. We report the case of a 5 year old child and his mother
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Juvenile idiopathic recurrent parotitis (JIRP) treated with short course steroids, a case series study and one decade follow up for potential autoimmune disorder Pediatr Rheumatol. (IF 2.5) Pub Date : 2024-01-04 Farhad Salehzadeh, Rasol Molatefi, Ali Mardi, Negin Nahanmoghaddam
Juvenile idiopathic recurrent parotitis (JIRP) in children is a condition characterized with recurrent episodes of idiopathic parotid gland inflammation. Since there are no definitive guidelines for diagnosis and management of this condition, we present a consecutive case series of patients with more than one decade follow up and their dramatic response to short course treatment by prednisolone. We
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The 4th NextGen therapies for SJIA and MAS: part 3 clinical trials in refractory SJIA: historic controls as an alternative to a withdrawal design study Pediatr Rheumatol. (IF 2.5) Pub Date : 2024-01-03 Fabrizio de Benedetti, Alexei A. Grom, Hermine Brunner
The substantial morbidity and mortality associated with refractory systemic JIA underlies the need for new treatment approaches. However, progress in this area has been limited by the difficulty of enrolling these patients in clinical trials with traditional designs, particularly in patients presenting with the life-threatening macrophage activation syndrome. At the NextGen 2022 conference, there was
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Towards a standardized program of transitional care for adolescents with juvenile idiopathic arthritis for Turkey: a national survey study Pediatr Rheumatol. (IF 2.5) Pub Date : 2024-01-02 Betül Sözeri, Nihal Şahin, Ceyhun Açarı, Pinar Ozge Avar Aydın, Ozge Baba, Esra Bağlan, Sevcan Bakkaloğlu, Sibel Bakırcı, Yelda Bilginer, Burcu Yücel Bozkaya, Şengül Çağlayan, Mustafa Çakan, Figen Çakmak, Taner Coşkuner, Ferhat Demir, Fatma Gül Demirkan, Şeyda Doğantan, Hatice Adıgüzel Dündar, Emine Duygu Ersözlü, Sercan Gücenmez, Oğuz Gürler, Rana İşgüder, Adem Küçük, Mukaddes Kalyoncu, Levent Kılıç
Juvenile idiopathic arthritis (JIA) is a prevalent childhood chronic arthritis, often persisting into adulthood. Effective transitional care becomes crucial as these patients transition from pediatric to adult healthcare systems. Despite the concept of transitional care being recognized, its real-world implementation remains inadequately explored. This study aims to evaluate the thoughts and practices
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Methotrexate inhibits BMP4 and abrogates the hypertrophic chondrocyte phenotype of synovial fibroblasts in juvenile idiopathic arthritis Pediatr Rheumatol. (IF 2.5) Pub Date : 2024-01-02 Megan M. Simonds, Samuel T. Freer, Anne Marie C. Brescia
Juvenile Idiopathic Arthritis (JIA) induces growth disturbances in affected joints. Fibroblast-like synoviocytes (FLS) play a crucial role in JIA pathogenesis. FLS overexpress bone morphogenetic protein 4 (BMP4) and have a chondrocyte-like phenotype. FLS contribute directly to joint growth disturbances through endochondral bone formation. We investigated the ability of methotrexate to inhibit BMP4
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Prospective study to characterize adalimumab exposure in pediatric patients with rheumatic diseases Pediatr Rheumatol. (IF 2.5) Pub Date : 2024-01-02 Tatjana Welzel, Klervi Golhen, Andrew Atkinson, Verena Gotta, David Ternant, Jasmin B. Kuemmerle-Deschner, Christine Michler, Gilbert Koch, Johannes N. van den Anker, Marc Pfister, Andreas Woerner
In pediatric rheumatic diseases (PRD), adalimumab is dosed using fixed weight-based bands irrespective of methotrexate co-treatment, disease activity (DA) or other factors that might influence adalimumab pharmacokinetics (PK). In rheumatoid arthritis (RA) adalimumab exposure between 2–8 mg/L is associated with clinical response. PRD data on adalimumab is scarce. Therefore, this study aimed to analyze
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A rare case of coronary artery complication in a child with systemic juvenile idiopathic arthritis and macrophage activation syndrome: case report and literature review Pediatr Rheumatol. (IF 2.5) Pub Date : 2024-01-02 Lian Zhang, Yanwen Wei, Ningjing Zeng, Lianyu Wang, Xinying Chen, Jinghua Yang, Xiaolan Xiao
A rare case of coronary artery involvement in a child with Systemic Juvenile Idiopathic Arthritis (sJIA) complicated by Macrophage Activation Syndrome (MAS) is reported. The patient initially received an inaccurate diagnosis of Kawasaki Disease, sepsis, and mycoplasma infection and showed no improvement after Intravenous Immune Globulin (IVIG) treatment. Upon admission, symptoms included diffuse red
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Predictors of fibrogenesis in children with JIA: a single-center pilot study Pediatr Rheumatol. (IF 2.5) Pub Date : 2024-01-02 Olga Pavlova, Natalia Shevchenko, Sergey Pavlov, Tetiana Holovko, Liudmyla Bogmat
Patients with rheumatological diseases are at high risk of developing irreversible fibrotic changes, both articular and extra-articular, as a result of tissue damage caused by the chronic phase of persistent inflammation. Thus, our purpose was to study early markers of fibrosis formation in children with juvenile idiopathic arthritis (JIA). Seventy patients with juvenile idiopathic arthritis, namely
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Local injection of infliximab into calcinosis lesions in patients with juvenile dermatomyositis (JDM): a clinical trial Pediatr Rheumatol. (IF 2.5) Pub Date : 2024-01-02 Reza Shiari, Mitra Khalili, Vahide Zeinali, Niloufar Shashaani, Mohammad Samami, Foroughossadat Hosseini Moghaddamemami
Juvenile Dermatomyositis (JDM) is a rare autoimmune disorder that primarily affects muscles and skin. One of the severe complications associated with JDM is calcinosis, and treating this condition presents significant challenges. This study aimed to evaluate the efficacy and safety of local injection of infliximab into calcinosis lesions in patients with JDM. In this clinical trial, five patients diagnosed
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Pediatrics severe low back pain by disc herniation: an uncommon entity Pediatr Rheumatol. (IF 2.5) Pub Date : 2024-01-02 Wendlassida Joelle Stéphanie Tiendrebeogo/Zabsonre, Denlewende Sylvain Zabsonre, Fulgence Kabore, Abdoulaye Sanou, Yakouba Haro, Inoussa Zoungrana, Dieu-Donné Ouedraogo
Common severe back pain due to disc herniation is rare in the paediatric population which involves children under eighteen years of age. Paediatric lumbar disc herniation (LDH) cannot be considered the same disease as in adults, as it has potentially different natural and clinical backgrounds. The treatment of pediatric LDH is the other particularity of this condition. Indeed, in children, delaying
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Transition readiness among finnish adolescents with juvenile idiopathic arthritis Pediatr Rheumatol. (IF 2.5) Pub Date : 2023-12-21 Mikola Katriina, Rebane Katariina, Kautiainen Hannu, Aalto Kristiina
With chronic diseases, the responsibility for care transfers to adult clinics at some point. Juvenile idiopathic arthritis (JIA) is the most common persistent rheumatic condition in children. A successful transition requires sufficient self-management skills to manage one´s chronic condition and all the tasks involved. In this study, we evaluated transition readiness in Finnish patients with JIA. We
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Defining a therapeutic range for adalimumab serum concentrations in the management of pediatric noninfectious uveitis, a step towards personalized treatment Pediatr Rheumatol. (IF 2.5) Pub Date : 2023-12-20 Jo L. Dehoorne, Helena Groth, Emma Carlé, Ilse De Schrijver, Celine Sys, Patricia Delbeke, Elke O. Kreps, Thomas Renson, Carolien Bonroy
Adalimumab is currently considered the most efficacious anti-TNFα agent for childhood noninfectious uveitis (NIU). The objective of this study was to define a therapeutic range for adalimumab trough levels in the treatment of childhood NIU. A retrospective, observational, pilot study of 36 children with NIU aged < 18 years, treated with adalimumab. Serum adalimumab through levels and adalimumab anti-drug
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Lung infection or inflammation-a puzzling case of MDA-5 associated juvenile dermatomyositis Pediatr Rheumatol. (IF 2.5) Pub Date : 2023-12-20 Anusha Vuppala, Manush Sondhi, Sarwat Umer
Juvenile dermatomyositis (JDM) is an uncommon inflammatory myopathy predominantly affecting children under 18 years of age. Diagnosis relies on identifying specific clinical features, such as muscle weakness, skin rash, elevated muscle enzymes, and MRI and muscle biopsy findings. Autoantibodies associated with inflammatory myopathy offer valuable prognostic insights and can indicate the risk of internal
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Role of ultrasound and inflammatory factors in the management of pediatric hip joint effusion Pediatr Rheumatol. (IF 2.5) Pub Date : 2023-12-19 Seyed Ali Alamdaran, Mohadeseh Taheri-nezhad, Ahmad Nouri, Farzaneh Khoroushi, Mohammad Hasan Aalami, Abdoreza Malek, Arezou Mohtasham, Mohamadreza Alizadeh
Septic arthritis is an important differential diagnosis of hip joint pain. Joint aspiration analysis is a necessary diagnostic measure for septic arthritis. In order to reduce the need for joint aspiration, we compared the combination of ultrasound findings and laboratory findings to separate septic arthritis from reactive arthritis. Children aged < 14 years who were referred to Akbar pediatric hospital
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Towards telehealth delivery in pediatric rheumatology practice Pediatr Rheumatol. (IF 2.5) Pub Date : 2023-12-17 Raju Khubchandani, Tadej Avčin, Angelo Ravelli
Much has been written and spoken about telemedicine since about two decades including an article in this journal at the start of the pandemic. It took a global catastrophe to enforce its usage across the world in various medical specialties. Telemedicine however remains unstructured, unregulated and lacks uniformity. This article highlights the practical learnings and opinions of the authors who provided
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Bridging gaps: a qualitative inquiry on improving paediatric rheumatology care among healthcare workers in Kenya Pediatr Rheumatol. (IF 2.5) Pub Date : 2023-12-13 Angela Migowa, Sasha Bernatsky, Anthony K. Ngugi, Helen E. Foster, Peterrock Muriuki, Roselyter M. Riang’a, Stanley Luchters
Due to the paucity of paediatric rheumatologists in Kenya, it is paramount that we explore strategies to bridge clinical care gaps for paediatric rheumatology patients in order to promote early diagnosis, prompt referral, and optimal management. To identify proposed interventions which can improve the ability of non-specialist healthcare workers to care for paediatric rheumatology patients across Kenya
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Identification of key biomarkers in RF-negative polyarticular and oligoarticular juvenile idiopathic arthritis by bioinformatic analysis Pediatr Rheumatol. (IF 2.5) Pub Date : 2023-11-24 Yun Liu, Xuemei Tang
Juvenile idiopathic arthritis (JIA) is a broad term used to describe arthritis of unknown origin. JIA commonly persists into adulthood, often causing substantial morbidity such as restricted joint function, which can lead to challenges in employment and independence. This study aims to identify diagnostic biomarkers and investigate the role of immune cells in the pathogenesis of rheumatoid factor-negative
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Chronic limping in childhood, what else other than juvenile idiopathic arthritis: a case series Pediatr Rheumatol. (IF 2.5) Pub Date : 2023-11-24 Cristina Tumminelli, Serena Pastore, Andrea Taddio
Limping is a common clinical symptom in childhood; different clinical conditions may lead to limping and the diagnosis of the underlying cause may often be a challenge for the pediatrician. We describe the clinical manifestations, radiological pictures and disease course of other causes of limping in childhood, through a case series of seven cases and a brief discussion of each disease. although trauma
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Impact of anakinra use on clinical outcomes in children with moderate or severe multisystem inflammatory syndrome in children: a propensity score matched retrospective cohort study Pediatr Rheumatol. (IF 2.5) Pub Date : 2023-11-23 Esra B. Akkoyun, Zachary Most, Harita Katragadda, Andrew Yu, Lorien Nassi, Nicole Oakman, Sarah Ginsburg, Mia Maamari
The treatment of children with multisystem inflammatory syndrome in children (MIS-C) related to SARS-CoV-2 infection involves immunomodulatory therapies such as IVIG and steroids. Anakinra, an interleukin-1 receptor inhibitor, has also been used, but its effectiveness is not established yet. As optimal regimens for MIS-C remain unknown, we aimed to assess the effect of anakinra in reducing hospital
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Is it possible to extend the dose interval of canakinumab treatment in children with familial Mediterranean fever? PeRA group experience Pediatr Rheumatol. (IF 2.5) Pub Date : 2023-11-23 Gülşah Kavrul Kayaalp, Şengül Çağlayan, Fatma Gül Demirkan, Vafa Guliyeva, Gülçin Otar Yener, Kübra Öztürk, Ferhat Demir, Semanur Özdel, Mustafa Çakan, Hafize Emine Sönmez, Betül Sözeri, Nuray Aktay Ayaz
There is no clear data on the optimal duration of treatment with anti-interleukin-1 drugs in colchicine-resistant familial Mediterranean fever patients, as well as on the dose interval. This study aimed to assess patients whose canakinumab dose interval was adjusted according to a specific protocol, with the objective of evaluating the effectiveness of implementing this protocol for the patient care
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“Online survey of COVID-19 immunization and infection in patients with systemic juvenile idiopathic arthritis and adult-onset still’s disease.” Pediatr Rheumatol. (IF 2.5) Pub Date : 2023-11-21 Mariana Correia Marques, Subrata Paul, Carol Lake, Ly-Lan Bergeron, Rashmi Sinha, Luciana Peixoto, Marinka Twilt, Michael J. Ombrello
Patients with systemic juvenile idiopathic arthritis (sJIA) and adult-onset Still’s disease (AOSD) have been under-represented in studies about safety of the COVID-19 immunization. We aimed to inquire about the safety and tolerability of COVID-19 immunization in this population. An anonymous online survey on closed Facebook groups for patients and parents with self-reported sJIA/AOSD was posted from
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Ovarian dysfunction in adolescent girls with autoimmune rheumatic diseases Pediatr Rheumatol. (IF 2.5) Pub Date : 2023-11-14 Mohamed Taman, El-Hadidy NM, Hamed Yousef, Doaa Mosad Mosa
To explore the different menstrual and pubertal abnormalities in adolescent females with systemic autoimmune rheumatic diseases (ARD). The study included adolescent girls aged 13–18 years with juvenile idiopathic arthritis (JIA), juvenile systemic lupus erythematosus (JSLE), and juvenile dermatomyositis (JDM) classified according to their international classification criteria. Data were collected from
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Juvenile dermatomyositis: association between nail fold capillary end row loop– area under the curve– and disease damage indicators Pediatr Rheumatol. (IF 2.5) Pub Date : 2023-11-13 Amer Khojah, Gabrielle Morgan, Marisa S. Klein-Gitelman, Lauren M. Pachman
Juvenile Dermatomyositis (JDM) is a rare autoimmune disease characterized by skin and muscle inflammation. The loss of nail fold capillary end row loops (ERL) is evidence of small vessel involvement in JDM. This study aimed to examine the specific association of ERL over the disease course with evidence of JDM disease damage. We analyzed data from 68 initially treatment-naïve JDM children who had been
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Tuberculosis among children and adolescents with rheumatic diseases - case series Pediatr Rheumatol. (IF 2.5) Pub Date : 2023-11-10 Lenita de Melo Lima, Adriana Rodrigues Fonseca, Clemax Couto Sant’Anna, Ana Alice Amaral Ibiapina Parente, Rafaela Baroni Aurilio, Maria de Fátima Bazhuni Pombo Sant’Anna
Background: Rheumatic patients have a higher frequency of tuberculosis(TB) than the general population. This study aimed to describe children and adolescents with TB and rheumatic diseases(RD) who were being treated in a reference center. Methods: A series of TB cases were investigated in a reference center for childhood TB in Rio de Janeiro, Brazil, from 1995 to 2022. Results: Fifteen patients with
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Reverse complete heart block using transcutaneous pacing and repeated plasmapheresis in a neonate with lupus: a case report Pediatr Rheumatol. (IF 2.5) Pub Date : 2023-11-09 Yanfei Liu, Wanwei Li, Kun Zhou, Zhangxue Hu
It has been reported that the complete heart block (CHB) in neonatal lupus (NL) cannot be reversed. This study reported a case of NL-CHB that was reversed by transcutaneous pacing and repeated plasmapheresis. A 35+ 6-week male preterm baby was transferred to the neonatal intensive care unit of the Army Medical Center in May 2020 for slight cyanosis around the lips and nose. Two days after birth, a
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Reporting of determinants of health inequities and participant characteristics in randomized controlled trials of juvenile idiopathic arthritis in Canada: a scoping review Pediatr Rheumatol. (IF 2.5) Pub Date : 2023-11-06 Niloofar Gheshlaghi, Megan Thomas, Natasha Trehan, Mark Harrison, Mary A. De Vera
Juvenile Idiopathic Arthritis (JIA) is the most common form of childhood inflammatory arthritis. The disease burden of JIA is substantial as patients require specialized medical practitioners for diagnosis and chronic treatments that are both costly and time intensive. Discrepancies in access to care due to health inequities such as socioeconomic status or geographic location may lead to vastly different
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Reliability of the tunisian pediatric gait, arms, legs, and spine: toward a valid screening tool for tunisian children with musculoskeletal conditions Pediatr Rheumatol. (IF 2.5) Pub Date : 2023-11-02 Lassoued Ferjani Hanene, Narjess Amdouni, Rabhi Emna, Sahli Hela, Ben Abdelgheni Kaouther, Ben Nessib Dorra, Kaffel Dhia, Maatallah Kaouther, Hamdi Wafa
Pediatric musculoskeletal disorders account for 10% of first-line consultations in Tunisia. Referral delay and deficiencies in musculoskeletal screening raise a challenge to the early diagnosis and management of rheumatic conditions in children. The pGALS (Pediatric Gait Arms Legs Spine) was developed and translated into many languages to overcome these deficiencies. Our study aimed to adapt and validate