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Characterization of autoantibodies, immunophenotype and autoimmune disease in a prospective cohort of patients with idiopathic CD4 lymphocytopenia Clin. Immunol. (IF 3.368) Pub Date : 2021-01-07 Cornelia D. Cudrici; Afroditi Boulougoura; Virginia Sheikh; Alexandra Freeman; Ornella Sortino; James D. Katz; Irini Sereti; Richard M. Siegel
Objective Characterize autoantibodies and autoimmune diseases in a prospective cohort of patients with Idiopathic CD4 Lymphocytopenia (ICL) a rare immunodeficiency characterized by an absolute CD4+ T count of <300 cells/μl in the absence of HIV or HTLV infection. Methods Single-Center prospective study of 67 patients conducted over an 11-year period. Rheumatologic evaluation and measurement of autoantibodies
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Peripheral blood hsa-circRNA5333-4: A novel biomarker for myasthenia gravis Clin. Immunol. (IF 3.368) Pub Date : 2021-01-17 Jie Lv; Lu Ren; Sensen Han; Jing Zhang; Xue Zhao; Yingna Zhang; Hua Fang; Linyuan Zhang; Haonan Yang; Shumin Wang; Junhong Yang; Xinzheng Cui; Qingyong Zhang; Yunke Zhang; Feng Gao
In this study, the potential of specific Circular RNAs (circRNAs) as novel peripheral blood biomarkers for myasthenia gravis (MG) was explored. We analyzed circRNAs in the peripheral blood of three normal controls and three MG patients using RNA microarray. Candidate circRNAs were validated in three independent cohorts by Quantitative Real-time polymerase chain reaction (qPCR). Eleven differentially
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Subgroup analysis of nelipepimut-S plus GM-CSF combined with trastuzumab versus trastuzumab alone to prevent recurrences in patients with high-risk, HER2 low-expressing breast cancer Clin. Immunol. (IF 3.368) Pub Date : 2021-01-22 R. Connor Chick; G. Travis Clifton; Diane F. Hale; Timothy J. Vreeland; Annelies T. Hickerson; Phillip M. Kemp Bohan; Patrick M. McCarthy; Jennifer K. Litton; Gheath Alatrash; Rashmi K. Murthy; Na Qiao; Anne Philips; Jason Lukas; Jarrod P. Holmes; Elizabeth A. Mittendorf; George E. Peoples
HER2-targeted therapy has not benefited patients with low levels of HER2 expression; however, combination therapy may be effective. Primary analysis of a phase IIb trial investigating the HER2-derived vaccine nelipepimut-S (NPS) did not benefit the intention-to-treat population, but subset analysis showed a benefit in triple-negative breast cancer (TNBC) patients. The subset analysis of this multicenter
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Autoimmune experimental Orchitis and chronic glomerulonephritis with end stage renal disease are controlled by Cgnz1 for susceptibility to end organ damage Clin. Immunol. (IF 3.368) Pub Date : 2021-01-19 Zhenhuan Zhao; Hui Qiao; Y. Ge; C.C. Kannapel; Sun-Sang J. Sung; Felicia Gaskin; Kenneth S.K. Tung; Shu Man Fu
Cgnz1 on chromosome 1 mapped into a 1.34 Mb region of chromosome 1 in NZM2328 confers the progression of immune complex (IC) mediated glomerulonephritis (GN) from acute GN (aGN) to chronic GN (cGN) with severe proteinuria and end stage renal disease in female mice. This genetic locus mediates podocyte susceptibility to IC mediated damage. Taking advantage of the published observation that Cgnz1 is
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Immunological features of circulating monocyte subsets in patients with squamous cell carcinoma of the head and neck Clin. Immunol. (IF 3.368) Pub Date : 2021-01-19 Koichi Sakakura; Hideyuki Takahashi; Sei-ichiro Motegi; Yuki Yokobori-Kuwabara; Tetsunari Oyama; Kazuaki Chikamatsu
Background Circulating monocytes are classified into three subsets according to their CD14 and CD16 expressions. Here we investigated all three subsets in patients with squamous cell carcinoma of the head and neck (SCCHN). Methods Peripheral blood from 54 patients with SCCHN and 24 healthy donors (HDs) was tested for flowcytometry. Immunohistochemical staining of the primary tumor was performed. SCCHN
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Interrelations between COVID-19 and other disorders Clin. Immunol. (IF 3.368) Pub Date : 2020-12-14 Amin Gasmi; Massimiliano Peana; Lyudmila Pivina; Shvetha Srinath; Asma Gasmi Benahmed; Yuliya Semenova; Alain Menzel; Maryam Dadar; Geir Bjørklund
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Identification of a potent regulatory T cell epitope in factor V that modulates CD4+ and CD8+ memory T cell responses Clin. Immunol. (IF 3.368) Pub Date : 2021-01-04 Anne S. De Groot; Amy Rosenberg; S.M. Shahjahan Miah; Gail Skowron; Brian J. Roberts; Sandra Lélias; Frances E. Terry; William D. Martin
Identification of T cell epitopes that are recognized by Tregs may elucidate the relative contributions of thymic Tregs and induced Tregs to control of autoimmune diseases and allergy. One such T regulatory cell epitope or ‘Tregitope’, derived from blood Factor V, is described here. Tregs responding to Tregitope FV621 are potent suppressors of CD4+ T effector responses to Tetanus Toxoid in an in vitro
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SARS-CoV-2 infection associated with hepatitis in an infant with X-linked severe combined immunodeficiency Clin. Immunol. (IF 3.368) Pub Date : 2021-01-04 Nicolai S.C. van Oers; Natasha W. Hanners; Paul Sue; Victor Aquino; Quan-Zhen Li; John W. Schoggins; Christian A. Wysocki
X-linked severe combined immunodeficiency (X-SCID) is a disorder of adaptive immunity caused by mutations in the IL-2 receptor common gamma chain gene resulting in deficiencies of T and natural killer cells, coupled with severe dysfunction in B cells. X-SCID is lethal without allogeneic stem cell transplant or gene therapy due to opportunistic infections. An infant with X-SCID became infected with
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Recent insights into the impact of immune dysfunction on reproduction in autoimmune thyroiditis Clin. Immunol. (IF 3.368) Pub Date : 2021-01-02 Qian Zhu; Qian-Han Xu; Ting Xie; Li-Ling Wang; Hong Liu; Kahindo P. Muyayalo; Xiao-Bo Huang; Si-Jia Zhao; Ai-Hua Liao
Autoimmune thyroiditis (AIT) is a common organ-specific autoimmune disease with a high incidence among women of childbearing age. Recent studies have reported that women with AIT are more susceptible to infertility, miscarriage and preterm birth. It has been investigated that abnormal changes in maternal immune system and maternal–fetal interface can dampen the immune tolerance between mother and fetus
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Leukocyte glucocorticoid receptor expression and related transcriptomic gene signatures during early sepsis Clin. Immunol. (IF 3.368) Pub Date : 2020-12-25 Jiabao Li; Miaorong Xie; Yanan Yu; Ziren Tang; Chenchen Hang; Chunsheng Li
Purpose The study aimed to understand the molecular mechanisms that might lead to differences in the glucocorticoid response during sepsis. Methods Patients diagnosed with sepsis (n = 198) and 40 healthy controls were enrolled. Glucocorticoid receptor (GR) expression in circulating leukocytes and plasma levels of adrenocorticotropic hormone and cortisol on days 1 and 7 were measured in all participants
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BTK inhibition modulates multiple immune cell populations involved in the pathogenesis of immune mediated nephritis Clin. Immunol. (IF 3.368) Pub Date : 2020-12-06 Samantha A. Chalmers; Sayra J. Garcia; Deborah Webb; Leal Herlitz; Jay Fine; Elliott Klein; Meera Ramanujam; Chaim Putterman
Lupus nephritis (LN) is a serious end organ complication of systemic lupus erythematosus. Nephrotoxic serum nephritis (NTN) is an inducible model of LN, which utilizes passive transfer of pre-formed nephrotoxic antibodies to initiate disease. In previous studies, we demonstrated that the Bruton's tyrosine kinase inhibitor, BI-BTK-1, prevents the development of nephritis in NTN when treatment was started
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HBV upregulated triggering receptor expressed on myeloid cells-1 (TREM-1) expression on monocytes participated in disease progression through NF-Kb pathway Clin. Immunol. (IF 3.368) Pub Date : 2020-12-13 Xiaojuan Wu; Bei Cai; Wang Lu; Yang Fu; Bin Wei; Qian Niu; Zhenzhen Su; Yamei Li; Lanlan Wang
The triggering receptor expressed on myeloid cells-1 (TREM-1) signal is related to the continuous amplification of inflammatory pathway. However, it is not clear whether and how HBV can regulated the expression of TREM-1 on monocyte participated in the progression of liver disease. Here, we showed that the expression of TREM-1 on monocyte subsets were increased significantly in HBV related liver cirrhosis
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Influenza infection, SARS, MERS and COVID-19: Cytokine storm – The common denominator and the lessons to be learned Clin. Immunol. (IF 3.368) Pub Date : 2020-12-14 Varvara A. Ryabkova; Leonid P. Churilov; Yehuda Shoenfeld
The outbreak of COVID-19 reminds us that the emerging and reemerging respiratory virus infections pose a continuing threat to human life. Cytokine storm syndromes of viral origin seem to have a common pathogenesis of the imbalanced immune response with the exaggerated inflammatory reaction combined with the reduction and functional exhaustion of T cells. Immunomodulatory therapy is gaining interest
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Expression of APOBEC family members as regulators of endogenous retroelements and malignant transformation in systemic autoimmunity Clin. Immunol. (IF 3.368) Pub Date : 2020-12-14 Clio P. Mavragani; Kyriakos A. Kirou; Adrianos Nezos; Surya Seshan; Teresa Wild; Sharon M. Wahl; Haralampos M. Moutsopoulos; Mary K. Crow
Objective To explore whether APOBEC family members are involved in the response to inappropriate expression of L1 retroelements in primary Sjögren's syndrome (SS) and systemic lupus erythematosus (SLE), as well as in SS related lymphomagenesis. Methods Minor salivary glands (MSG) and kidney biopsy (KB) specimens were obtained from 41 SS patients (10 with lymphoma) and 23 patients with SLE, respectively
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Endoplasmic reticulum stress exacerbates inflammation in chronic rhinosinusitis with nasal polyps via the transcription factor XBP1 Clin. Immunol. (IF 3.368) Pub Date : 2020-12-25 Min Li; Yadong Xie; Keqing Zhao; Kun Chen; Yujie Cao; Jia Zhang; Miaomiao Han; Li Hu; Rui He; Dehui Wang; Huabin Li
Endoplasmic reticulum (ER) stress results in the activation of the unfolded protein response (UPR), a process that is involved in the pathogenesis of many inflammatory diseases. However, the role of ER stress in chronic rhinosinusitis with nasal polyps (CRSwNP) has yet to be elucidated. In this study, we found that the protein expression levels of a range of ER stress regulators, including p-PERK,
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A set of clinical and laboratory markers differentiates hyper-IgE syndrome from severe atopic dermatitis Clin. Immunol. (IF 3.368) Pub Date : 2020-12-07 Nurhan Kasap; Velat Celik; Sakine Isik; Pakize Cennetoglu; Ayca Kiykim; Sevgi Bilgic Eltan; Ercan Nain; Ismail Ogulur; Dilek Baser; Emre Akkelle; Mehmet Halil Celiksoy; Burcu Kocamis; Funda Erol Cipe; Ayse Deniz Yucelten; Elif Karakoc-Aydiner; Ahmet Ozen; Safa Baris
Hyper-IgE syndrome (HIES) patients may share many features observed in severe atopic dermatitis (SAD), making a diagnostic dilemma for physicians. Determining clinical and laboratory markers that distinguish both disorders could provide early diagnosis and treatment. We analyzed patients (DOCK8 deficiency:14, STAT3-HIES:10, SAD:10) with early-onset SAD. Recurrent upper respiratory tract infection and
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Immune modulating effects of receptor interacting protein 2 (RIP2) in autoinflammation and immunity Clin. Immunol. (IF 3.368) Pub Date : 2020-12-11 Sigrun Ruth Hofmann; Leonie Girschick; Robert Stein; Felix Schulze
Receptor-interacting protein 2 (RIP2) is a kinase that is involved in downstream signaling of nuclear oligomerization domain (NOD)-like receptors NOD1 and 2 sensing bacterial peptidoglycans. RIP2-deficiency or targeting of RIP2 by pharmaceutical inhibitors partially ameliorates inflammatory diseases by reducing pro-inflammatory signaling in response to peptidoglycans. However, RIP2 is widely expressed
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Effectiveness and safety of intravenous tocilizumab to treat COVID-19-associated hyperinflammatory syndrome: Covizumab-6 observational cohort Clin. Immunol. (IF 3.368) Pub Date : 2020-11-12 Hèctor Corominas; Ivan Castellví; Virginia Pomar; Rosa Antonijoan; Isabel Mur; Laia Matas; Ignasi Gich; Natividad de Benito; Ana Laiz; Diego Castillo; Laura Villamarin; David Filella; Ana Milena Millán; María Ángeles Quijada; Mireia Puig; Jordi Casademont; Pere Domingo
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Kawasaki or Kawasaki-like disease? A debate on COVID-19 infection in children Clin. Immunol. (IF 3.368) Pub Date : 2020-12-07 Mohammad-Salar Hosseini
Kawasaki disease (KD) is an inflammatory syndrome which is generally observed among children. Considering the significant number of COVID-19-positive children presenting with the manifestations of typical/atypical KD, it has been mentioned as a possible complication of COVID-19 infection among the children. However, many of the reported cases do not completely fill the clinical diagnostic criteria
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COVID-19: A review of therapeutic strategies and vaccine candidates Clin. Immunol. (IF 3.368) Pub Date : 2020-11-17 Vladislav Izda; Matlock A. Jeffries; Amr H. Sawalha
The world is engulfed by one of the most widespread and significant public health crises in decades as COVID-19 has become among the leading causes of death internationally. The novel SARS-CoV-2 coronavirus which causes COVID-19 has unified the scientific community in search of therapeutic and preventative solutions. The top priorities at the moment are twofold: first, to repurpose already-approved
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Class Switch Recombination Defects: impact on B cell maturation and antibody responses Clin. Immunol. (IF 3.368) Pub Date : 2020-12-01 Ellen D. Renner; Carolin E. Krätz; Jordan S. Orange; Beate Hagl; Stacey Rylaarsdam; Gundula Notheis; Anne Durandy; Troy R. Torgerson; Hans D. Ochs
To assess how B cell phenotype analysis correlates with antigen responses in patients with class switch recombination defects (CSRD) we quantified memory B cells by flow-cytometry and immunized CSRD patients with the neoantigen bacteriophage phiX174 (phage). CSRD patients showed uniformly absent or markedly reduced switched memory B cells (IgM−IgD−CD27+). CD40L patients had reduced CD27+ memory B cells
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Tim-3 is a potential regulator that inhibits monocyte inflammation in response to intermittent hypoxia in children with obstructive sleep apnea syndrome Clin. Immunol. (IF 3.368) Pub Date : 2020-11-30 Wei Wang; Zhifei Xu; Jie Zhang; Shengcai Wang; Wentong Ge; Xiaodan Li; Wenjun Mou; Xiaolin Wang; Wenjia Chai; Jing Zhao; Guixiang Wang; Yue Xi; Yue Qiu; Tingting Ji; Jingang Gui; Jun Tai; Xin Ni
The mechanism of the characteristic intermittent hypoxia (IH) of obstructive sleep apnea syndrome (OSAS) on monocyte remain unclear. Our study found that OSAS children had a significantly upregulated expression in circulating proinflammatory cytokines IL-6 and IL-12, and endothelial injury markers VEGF and ICAM1. Association analysis revealed that the plasma TNFα, IL-1β, IL-6, IL-10 and IL-12 concentration
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Successful hematopoietic stem cell transplantation in a 4-1BB deficient patient with EBV-induced lymphoproliferation Clin. Immunol. (IF 3.368) Pub Date : 2020-11-28 Christine Wildermann; Mohammed Alosaimi; Sophie Liebenehm; Eva-Maria Jacobsen; Thomas F.E. Barth; Peter Möller; Klaus-Michael Debatin; Ansgar Schulz; Mehtap Sirin; Ibraheem F. Abosoudah; Fowzan S. Alkuraya; Raif S. Geha; Manfred Hönig
Complete remission from recurrent EBV-positive lymphoma is not mandatory before HSCT to achieve long-term cure in a patient suffering from a recently described immunodeficiency affecting the T-cell coactivation molecule 4-1BB.
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Peripheral immunological features of COVID-19 patients in Taizhou, China: A retrospective study Clin. Immunol. (IF 3.368) Pub Date : 2020-11-28 Xiao-Hong Jin; Hui-Li Zhou; Ling-Ling Chen; Guan-Fu Wang; Qiu-Yue Han; Jian-Gang Zhang; Xia Zhang; Qiong-Yuan Chen; Yao-Han Ye; Aifen Lin; Wei-Hua Yan
Background Abnormal peripheral immunological features are associated with the progression of coronavirus disease 2019 (COVID-19). Methods Clinical and laboratory data were retrieved in a cohort of 146 laboratory-confirmed COVID-19 patients. Potential risk factors for the development of severe COVID-19 were evaluated. Results On admission, lymphocytes, CD3+, CD4+ and CD8+ T cells, eosinophils, and albumin
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miR-126-3p and miR-16-5p as novel serum biomarkers for disease activity and treatment response in symptomatic dermographism Clin. Immunol. (IF 3.368) Pub Date : 2020-11-29 Qi Gao; Liming Zhang; Ruiqun Qi; Li Qiu; Xinghua Gao; Ting Xiao; Hongduo Chen
Symptomatic dermographism (SD) is the most common form of physical urticaria. So far no promising serum biomarkers for SD have been reported. Recently, microRNAs (miRNAs) have been reported to be serum biomarkers for chronic spontaneous urticaria. However, association of miRNAs with SD remains unclear. We enrolled 55 SD patients and 52 healthy controls in this study. We found that serum expressions
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A study of multinucleated giant cells in esophageal cancer Clin. Immunol. (IF 3.368) Pub Date : 2020-11-13 Hui Wang; Junjie Zhou; Jun Li; Yiqun Geng; Pei Meng; Changchun Ma; Ziqi Zhu; Weifeng Zhang; Liangli Hong; Yan Quan; Jiacong Wei; Qiongyi Huang; You Zhou; Zuoqing Su; Xiaoqing Zhu; Chuangzhen Chen; Shaobin Chen; Jiang Gu
Objectives To evaluate the occurrence, abundance, distribution, nature and clinical significance of multinucleated giant cell (MGC) in esophageal cancer. Materials and methods MGCs were examined with conventional pathology, immunohistochemistry and immunofluorescence in 107 esophageal cancer tissues. The findings were correlated to pathological diagnosis and clinical behavior of the cancers. Results
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Association of cardiometabolic risk status with clinical activity and damage in systemic lupus erythematosus patients: A cross-sectional study Clin. Immunol. (IF 3.368) Pub Date : 2020-11-21 Bertha Campos-López; Mónica R. Meza-Meza; Isela Parra-Rojas; Adolfo I. Ruiz-Ballesteros; Barbara Vizmanos-Lamotte; José Francisco Muñoz-Valle; Margarita Montoya-Buelna; Sergio Cerpa-Cruz; Luis E. Bernal-Hernández; Ulises De la Cruz-Mosso
Cardiometabolic status is a key factor in mortality by cardiovascular disease (CVD) in systemic lupus erythematosus (SLE). This study evaluated the association of cardiometabolic risk status with clinical activity and damage in SLE patients. A cross-sectional study was conducted in 158 SLE patients and 123 healthy subjects (HS). Anthropometry, glucose, hs-CRP, lipid profile, oxLDL, sCD36, anti-oxLDL
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Mechanistic consideration of the effect of perioperative volatile anesthetics on phagocytes Clin. Immunol. (IF 3.368) Pub Date : 2020-11-17 Koichi Yuki; Lifei Hou; Miho Shibamura-Fujiogi; Sophia Koutsogiannaki; Sulpicio G. Soriano
A growing literature has shown that volatile anesthetics are promiscuous molecules targeting multiple molecules, some of which are critical for immunological functions. We focused on studies that delineated target molecules of volatile anesthetics on immune cells and summarized the effects of volatile anesthetics on immune functions. We also presented the perspectives of studying volatile anesthetics-mediated
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T cell receptor repertoire as a potential diagnostic marker for celiac disease Clin. Immunol. (IF 3.368) Pub Date : 2020-11-13 Ying Yao; Asima Zia; Ralf Stefan Neumann; Milena Pavlovic; Gabriel Balaban; Knut E.A. Lundin; Geir Kjetil Sandve; Shuo-Wang Qiao
An individual's T cell repertoire is skewed towards some specificities as a result of past antigen exposure and subsequent clonal expansion. Identifying T cell receptor signatures associated with a disease is challenging due to the overall complexity of antigens and polymorphic HLA allotypes. In celiac disease, the antigen epitopes are well characterised and the specific HLA-DQ2-restricted T-cell repertoire
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Major reduction of NKT cells in patients with severe COVID-19 pneumonia Clin. Immunol. (IF 3.368) Pub Date : 2020-11-13 Maria Antonella Zingaropoli; Valentina Perri; Patrizia Pasculli; Francesco Cogliati Dezza; Parni Nijhawan; Giulia Savelloni; Giuseppe La Torre; Claudia D'Agostino; Fabio Mengoni; Miriam Lichtner; Maria Rosa Ciardi; Claudio Maria Mastroianni
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DNA methylation signatures of autoimmune diseases in human B lymphocytes Clin. Immunol. (IF 3.368) Pub Date : 2020-11-12 Moncef Zouali
B lymphocytes play key roles in adaptive and innate immunity. In autoimmune diseases, their participation in disease instigation and/or progression has been demonstrated in both experimental models and clinical trials. Recent epigenetic investigations of human B lymphocyte subsets revealed the importance of DNA methylation in exquisitely regulating the cellular activation and differentiation programs
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Proinflammatory cytokines are associated with prolonged viral RNA shedding in COVID-19 patients Clin. Immunol. (IF 3.368) Pub Date : 2020-10-14 Chun Gao; Li Zhu; Cheng Cheng Jin; Yi Xin Tong; Ai Tang Xiao; Sheng Zhang
Since December 2019, Coronavirus Disease 2019 (COVID-19) has emerged as a global pandemic. We aimed to investigate the clinical characteristics and analyzed the risk factors for prolonged viral RNA shedding. We retrospectively collected data from 112 hospitalized COVID-19 patients in a single center in Wuhan, China. Factors associated with prolonged viral RNA shedding (≥28 days) were investigated.
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Treg-dependent immunosuppression triggers effector T cell dysfunction via the STING/ILC2 axis Clin. Immunol. (IF 3.368) Pub Date : 2020-11-08 Kalliopi Domvri; Savvas Petanidis; Paul Zarogoulidis; Doxakis Anestakis; Drosos Tsavlis; Chong Bai; Haidong Huang; Lutz Freitag; Wolfgang Hohenforst-Schmidt; Konstantinos Porpodis; Theodora Katopodi
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Treatment of antiphospholipid syndrome. Clin. Immunol. (IF 3.368) Pub Date : 2020-09-19 Radin Massimo,Cecchi Irene,Rubini Elena,Foddai Silvia Grazietta,Barinotti Alice,Menegatti Elisa,Roccatello Dario,Savino Sciascia
Antiphospholipid syndrome (APS) is the most common acquired thrombophilia. The clinical manifestations of APS are mainly vascular thrombosis (venous and/or arterial) and/or recurrent pregnancy morbidity with the concomitant persistent presence of antiphospholipid antibodies (aPL). Therefore, the goals of the treatment of patients with APS are reducing the pregnancy morbidity and/or the prevention of
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Clinical markers combined with HMGB1 polymorphisms to predict efficacy of conventional DMARDs in rheumatoid arthritis patients. Clin. Immunol. (IF 3.368) Pub Date : 2020-09-11 Ke Xu,Xiaoyu Ren,Bomiao Ju,Yirixiati Aihaiti,Yongsong Cai,Yingang Zhang,Lan He,Jing Wang
The efficacy of conventional synthetic disease-modifying antirheumatic drugs (csDMARDs) for rheumatoid arthritis (RA) patients was limited. However, there were no predictive markers for poor csDMARDs outcome. Clinical information of RA patients was collected and the high-mobility group box 1 (HMGB1) polymorphisms (rs4145277, rs2249825, rs1412125 and rs1045411) were examined. Among the 252 patients
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Ex vivo assays to detect complement activation in complementopathies Clin. Immunol. (IF 3.368) Pub Date : 2020-10-24 Xuan Yuan; Jia Yu; Gloria Gerber; Shruti Chaturvedi; Michael Cole; Hang Chen; Ara Metjian; C. John Sperati; Evan M. Braunstein; Robert A. Brodsky
In complement-driven thrombotic microangiopathies, failure to regulate complement activation leads to end-organ damage. The modified Ham (mHam) test measures complement-mediated killing of a nucleated cell in vitro but lacks a confirmatory assay and reliable positive controls. We demonstrate that C5b-9 accumulation on the surface of TF1 PIGAnull cells correlates with cell killing in the mHam. We also
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Laboratory biomarkers associated with COVID-19 severity and management Clin. Immunol. (IF 3.368) Pub Date : 2020-10-22 S. Keddie; O. Ziff; M.K.L. Chou; R.L. Taylor; A. Heslegrave; E. Garr; N. Lakdawala; A. Church; D. Ludwig; J. Manson; M. Scully; E. Nastouli; M.D. Chapman; M. Hart; M.P. Lunn
The heterogeneous disease course of COVID-19 is unpredictable, ranging from mild self-limiting symptoms to cytokine storms, acute respiratory distress syndrome (ARDS), multi-organ failure and death. Identification of high-risk cases will enable appropriate intervention and escalation. This study investigates the routine laboratory tests and cytokines implicated in COVID-19 for their potential application
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A simple and highly efficient method of IFI44L methylation detection for the diagnosis of systemic lupus erythematosus Clin. Immunol. (IF 3.368) Pub Date : 2020-10-16 Bo Zhang; Limin Liu; Tian Zhou; Xiaoli Shi; Haijing Wu; Zhongyuan Xiang; Ming Zhao; Qianjin Lu
Systemic lupus erythematosus (SLE) is a complex heterogenous autoimmune disease that can be challenging to diagnose. We previously identified the IFN-induced protein 44-like (IFI44L) methylation marker for SLE diagnosis, which can be detected by pyrosequencing. Although the previous technique has high sensitivity and specificity, it requires special equipment and high cost for detection. Here, we established
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An enlightening role for cytokine storm in coronavirus infection Clin. Immunol. (IF 3.368) Pub Date : 2020-10-23 Zhongyi Zhao; Yinhao Wei; Chuanmin Tao
The severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) outbreak in Wuhan, China has dispersed rapidly worldwide. Although most patients present with mild fever, cough with varying pulmonary shadows, a significant portion still develops severe respiratory dysfunction. And these severe cases are often associated with manifestations outside the respiratory tract. Currently, it is not difficult
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Incidental diagnosis of leukocyte adhesion deficiency type II following ABO typing Clin. Immunol. (IF 3.368) Pub Date : 2020-09-28 Nina Cooper; Yu-Tung Li; Anette Möller; Nelly Schulz-Weidner; Ulrich J. Sachs; Franz Wagner; Holger Hackstein; Sandra Wienzek-Lischka; Marianne Grüneberg; Martin K. Wild; Gregor Bein; Thorsten Marquardt
Individuals with the Bombay phenotype (Oh) in the ABO blood group system do not express the H, A, and B antigens but have no clinical symptoms. Bombay phenotype with clinical symptoms has been described in leukocyte adhesion deficiency type II (LAD II), a fucosylation disorder caused by mutations in SLC35C1. Only few LAD II patients have been described so far. Here we describe an additional patient
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Airways therapy of obstructive sleep apnea dramatically improves aberrant levels of soluble cytokines involved in autoimmune disease Clin. Immunol. (IF 3.368) Pub Date : 2020-10-02 Bradley G. Phillips; Ye Wang; Suresh Ambati; Ping Ma; Richard B. Meagher
Obstructive Sleep Apnea (OSA) damages the health of 35% of adult Americans. Disordered sleep results in increased risk of several autoimmune disorders, but the molecular links to autoimmunity are poorly understood. Herein, we identified four cytokines associated with autoimmune disease, whose median serum levels were significantly different for OSA patients receiving airways therapy, from the levels
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Immunophenotyping reveals distinct subgroups of lupus patients based on their activated T cell subsets Clin. Immunol. (IF 3.368) Pub Date : 2020-09-29 Daniel J. Perry; Anton A. Titov; Eric S. Sobel; Todd M. Brusko; Laurence Morel
Objective This study performed an integrated analysis of the cellular and transcriptional differences in peripheral immune cells between patients with Systemic Lupus Erythematosus (SLE) and healthy controls (HC). Methods Peripheral blood was analyzed using standardized flow cytometry panels. Transcriptional analysis of CD4+ T cells was performed by microarrays and Nanostring assays. Results SLE CD4+
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Infection-induced myeloperoxidase specific antineutrophil cytoplasmic antibody (MPO-ANCA) associated vasculitis: A systematic review. Clin. Immunol. (IF 3.368) Pub Date : 2020-09-19 Loukas Kakoullis,Konstantinos Parperis,Eleni Papachristodoulou,George Panos
Background We conducted a systematic review to identify cases of infection-induced anti-myeloperoxidase (MPO) antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV). Methods PubMed/Medline databases were searched from inception to July of 2020, according to PRISMA guidelines. Results Among the 618 abstracts identified, 18 articles describing 23 patients (60.9% female, mean age 50.5 years)
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Transcriptome of airway neutrophils reveals an interferon response in life-threatening respiratory syncytial virus infection. Clin. Immunol. (IF 3.368) Pub Date : 2020-09-11 Sjanna B Besteman,Amie Callaghan,Annefleur C Langedijk,Marije P Hennus,Linde Meyaard,Michal Mokry,Louis J Bont,Jorg J A Calis
Background Neutrophils are the most abundant cell type infiltrating the airways during severe respiratory syncytial virus (RSV) infection. Their exact role in disease pathophysiology remains enigmatic. Therefore, we determined genome-wide RNA expression profiles of local and systemic neutrophils in RSV bronchiolitis to provide further insight into local neutrophil biology. Methods We performed a single-center
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Bronchoalveolar lavage differential cell count on prognostic assessment of patients with stable or acute interstitial lung disease: A retrospective real-life study. Clin. Immunol. (IF 3.368) Pub Date : 2020-09-12 Johanna Salonen,Henri Lampela,Eerika Keskitalo,Hannu Vähänikkilä,Minna Purokivi,Riitta Kaarteenaho
BAL cell differential counts of 133 therapy naive ILD patients and 43 patients during acute exacerbation of ILD (AE-ILD) were retrospectively evaluated. In the 20 patients who underwent BAL both at baseline and during an AE-ILD, there was an increase in neutrophils but a decrease in macrophages and eosinophils in the BAL obtained during AE-ILD. A detectable number of basophils at the baseline was a
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Complement C3 vs C5 inhibition in severe COVID-19: Early clinical findings reveal differential biological efficacy. Clin. Immunol. (IF 3.368) Pub Date : 2020-09-19 Dimitrios C Mastellos,Bruno G P Pires da Silva,Benedito A L Fonseca,Natasha P Fonseca,Maria A Martins,Sara Mastaglio,Annalisa Ruggeri,Marina Sironi,Peter Radermacher,Akrivi Chrysanthopoulou,Panagiotis Skendros,Konstantinos Ritis,Ilenia Manfra,Simona Iacobelli,Markus Huber-Lang,Bo Nilsson,Despina Yancopoulou,E Sander Connolly,Cecilia Garlanda,Fabio Ciceri,Antonio M Risitano,Rodrigo T Calado,John D Lambris
Growing clinical evidence has implicated complement as a pivotal driver of COVID-19 immunopathology. Deregulated complement activation may fuel cytokine-driven hyper-inflammation, thrombotic microangiopathy and NET-driven immunothrombosis, thereby leading to multi-organ failure. Complement therapeutics have gained traction as candidate drugs for countering the detrimental consequences of SARS-CoV-2
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Eicosanoid production varies by sex in mesenteric ischemia reperfusion injury. Clin. Immunol. (IF 3.368) Pub Date : 2020-09-19 Miaomiao Wu,Jennifer M Rowe,Sherry D Fleming
Intestinal ischemia/reperfusion (I/R)-induced injury is an inflammatory response with significant morbidity and mortality. The early inflammatory response includes neutrophil infiltration. However, the majority of rodent studies utilize male mice despite a sexual dimorphism in intestinal I/R-related diseases. We hypothesized that sex may alter inflammation by changing neutrophil infiltration and eicosanoid
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Lymphoproliferative disorder with polyautoimmunity and hypogammaglobulinemia: An unusual presentation of 22q11.2 deletion syndrome. Clin. Immunol. (IF 3.368) Pub Date : 2020-09-11 Diogo C Soares,Anelisa G Dantas,Marina C Matta,Antonio C Pastorino,Maria Isabel Melaragno,Leslie Kulikowski,Marilia Montenegro,Chong A Kim,Magda Carneiro-Sampaio,Leuridan C Torres
22q11.2 deletion syndrome (22q11.2DS) has a heterogeneous presentation that includes multiple congenital anomalies and immunodeficiency, one of the most striking features. Usually, it is characterized by T cell lymphopenia, B cell dysfunction and autoimmunity. Here, we describe an unusual case of 22q11.2DS in a patient with lymphoproliferative disorder, polyautoimmunity and hypogammaglobulinemia.
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On the genetics and immunopathogenesis of COVID-19. Clin. Immunol. (IF 3.368) Pub Date : 2020-09-10 Chaim Oscar Jacob
Most severe cases with COVID-19, especially those with pulmonary failure, are not a consequence of viral burden and/or failure of the ‘adaptive’ immune response to subdue the pathogen by utilizing an adequate ‘adaptive’ immune defense. Rather it is a consequence of immunopathology, resulting from imbalanced innate immune response, which may not be linked to pathogen burden at all. In fact, it might
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SARS-CoV-2 infections in children and young people. Clin. Immunol. (IF 3.368) Pub Date : 2020-09-06 Susanna Felsenstein,Christian M Hedrich
Though recent reports link SARS-CoV-2 infections with hyper-inflammatory states in children, most children experience no/mild symptoms, and hospitalization and mortality rates are low in the age group. As symptoms are usually mild and seroconversion occurs at low frequencies, it remains unclear whether children significantly contribute to community transmission. Several hypotheses try to explain age-related
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Up-regulated DERL3 in fibroblast-like synoviocytes exacerbates inflammation of rheumatoid arthritis. Clin. Immunol. (IF 3.368) Pub Date : 2020-08-29 Manman Geng,Ke Xu,Liesu Meng,Jing Xu,Congshan Jiang,Yuanxu Guo,Xiaoyu Ren,Xiaowei Li,Yizhao Peng,Si Wang,Fumeng Huang,Jing Zhang,Xipeng Wang,Wenhua Zhu,Shemin Lu
Endoplasmic reticulum (ER) stress associated proteins contribute to the pathogenesis of rheumatoid arthritis (RA) through affecting synoviocyte proliferation and proinflammatory cytokine production. The role of DERL3, an ER-associated degradation component, in joint inflammation of RA was explored. Synovial tissues from RA and osteoarthritis (OA) patients were collected, and in RA synovial tissue,
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Successful hematopoietic stem cell transplantation for complete CTLA-4 haploinsufficiency due to a de novo monoallelic 2q33.2-2q33.3 deletion. Clin. Immunol. (IF 3.368) Pub Date : 2020-09-11 Vassilios Lougaris,Michele Malagola,Manuela Baronio,Enrico Morello,Luisa Gazzurelli,Alessio Benvenuto,Laura Palumbo,Daniele Moratto,Maria Federica Girelli,Marcho Chiarini,Antonella Meini,Alessandro Turra,Simona Bernardi,Nicola Polverelli,Domenico Russo,Alessandro Plebani
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Capsaicin alleviates acetaminophen-induced acute liver injury in mice. Clin. Immunol. (IF 3.368) Pub Date : 2020-08-28 Xiang Zhan,Jianqiang Zhang,Hui Chen,Liyuan Liu,Yiming Zhou,Ting Zheng,Suxiao Li,Yanxiang Zhang,Bing Zheng,Quan Gong
Overdose of N-acetyl-para-aminophenol (APAP) can induce acute liver injury (ALI). We evaluated the potential protective effect of 8-methyl-N-geranyl-6-nonamide (capsaicin (CAP)) in APAP-induced ALI in mice. ALI was induced by APAP (150 mg/kg, i.p.) administration; CAP pretreatment (1 mg/kg) was undertaken before APAP injection for 3 consecutive days. We found that CAP pretreatment attenuated ALI significantly;
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Interferon gamma, TGF-β1 and RANTES expression in upper airway samples from SARS-CoV-2 infected patients. Clin. Immunol. (IF 3.368) Pub Date : 2020-08-29 María Caridad Montalvo Villalba,Odalys Valdés Ramírez,Mayra Muné Jiménez,Amely Arencibia Garcia,Javier Martinez Alfonso,Guelsy González Baéz,Rosmery Roque Arrieta,Dianelvys Rosell Simón,Delmis Alvárez Gainza,Beatriz Sierra Vázquez,Sonia Resik Aguirre,Maria Guadalupe Guzmán Tirado
Upper respiratory tract is the primary site of SARS-CoV-2 replication. Releasing of pro and anti-inflammatory mediators plays an important role in the immunopathogenesis of Coronavirus Disease 2019 (COVID-19). The aim of this study was to evaluate the early inflammatory response in upper airway by measuring of IFN-γ, TGF-β1 and RANTES at mRNA level. Forty five SARS-CoV-2 infected patients were enrolled
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Micronutrients as immunomodulatory tools for COVID-19 management. Clin. Immunol. (IF 3.368) Pub Date : 2020-07-22 Amin Gasmi,Torsak Tippairote,Pavan Kumar Mujawdiya,Massimiliano Peana,Alain Menzel,Maryam Dadar,Asma Gasmi Benahmed,Geir Bjørklund
COVID-19 rapidly turned to a global pandemic posing lethal threats to overwhelming health care capabilities, despite its relatively low mortality rate. The clinical respiratory symptoms include dry cough, fever, anosmia, breathing difficulties, and subsequent respiratory failure. No known cure is available for COVID-19. Apart from the anti-viral strategy, the supports of immune effectors and modulation
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Possible role of HLA class-I genotype in SARS-CoV-2 infection and progression: A pilot study in a cohort of Covid-19 Spanish patients. Clin. Immunol. (IF 3.368) Pub Date : 2020-08-15 Ignacio Iturrieta-Zuazo,Claudia Geraldine Rita,Ana García-Soidán,Ana de Malet Pintos-Fonseca,Nieves Alonso-Alarcón,Roberto Pariente-Rodríguez,Amalia Tejeda-Velarde,Sergio Serrano-Villar,José Luis Castañer-Alabau,Israel Nieto-Gañán
Human Leukocyte Antigen (HLA) includes a large set of genes with important actions in immune response against viral infection. Numerous studies have revealed the existence of significant associations between certain HLA alleles and the susceptibility and prognosis of different infectious diseases. In this pilot study we analyse the binding affinity between 66 class I HLA alleles and SARS-CoV-2 viral
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Reduction in the percentage of circulating variable delta 2 T cells in systemic lupus erythematosus. Clin. Immunol. (IF 3.368) Pub Date : 2020-08-26 Tomohiro Koga,Yushiro Endo,Masataka Umeda,Tomohito Sato,Yuko Mizunoo,Kaori Furukawa,Toshimasa Shimizu,Remi Sumiyoshi,Shin-Ya Kawashiri,Naoki Iwamoto,Takashi Igawa,Kunihiro Ichinose,Mami Tamai,Hideki Nakamura,Tomoki Origuchi,Atsushi Kawakami
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Wiskott-Aldrich Syndrome in four male siblings from a consanguineous family from Lebanon. Clin. Immunol. (IF 3.368) Pub Date : 2020-08-16 Rana Mansour,Youmna El-Orfali,Antoine Saber,Dolly Noun,Nour Youssef,Yolla Youssef,Rima Hanna-Wakim,Ghassan Dbaibo,Miguel Abboud,Michel J Massaad
Background Wiskott-Aldrich syndrome (WAS) is a rare X-linked primary immunodeficiency disorder (PID) characterized by microthrombocytopenia, bloody diarrhea, eczema, recurrent infections, and a high incidence of autoimmunity and malignancy. Objective To investigate the mechanism of thrombocytopenia and infections in four boys of consanguineous parents from Lebanon. Methods Patient gDNA was studied
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Effects of dietary restriction on gut microbiota and CNS autoimmunity. Clin. Immunol. (IF 3.368) Pub Date : 2020-08-18 Claudia Cantoni,Yair Dorsett,Luigi Fontana,Yanjiao Zhou,Laura Piccio
Multiple sclerosis (MS) is the most common central nervous system (CNS) autoimmune disease. It is due to the interplay of genetic and environmental factors. Current opinion is that diet could play a pathogenic role in disease onset and development. Dietary restriction (DR) without malnutrition markedly improves health and increases lifespan in multiple model organisms. DR regimens that utilize continuous