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By Wei No
Raphael Carapito, Jacques-Eric Gottenberg, Irina Kotova, Meiggie Untrau, Sandra Michel, Lydie Naegely, Ismail Aouadi, Marius Kwemou, Nicodème Paul, Angélique Pichot, James Locke, Simon J Bowman, Bridget Griffiths, Kathy L Sivils, Jean Sibilia, Hidetoshi Inoko, Corinne Micelli-Richard, Gaétane Nocturne, Masao Ota, Wan-Fai Ng, Xavier Mariette, Seiamak Bahram
The association of primary Sjögren's syndrome (pSS) with Major Histocompatibility Complex (MHC) alleles is quintessential of MHC-disease associations. Indeed, although disease associations with classical HLA class I and II alleles/haplotypes are amply documented, further dissection is often prevented by the strong linkage disequilibrium across the entire MHC complex. Here we study the association of pSS, not with HLA genes, but with the non-conventional MHC encoded class I gene, MICA (MHC class I chain-related gene A)...
April 4, 2017: Human Molecular Genetics
Hiroto Tsuboi, Shinya Hagiwara, Hiromitsu Asashima, Hiroyuki Takahashi, Tomoya Hirota, Hisashi Noma, Hisanori Umehara, Atsushi Kawakami, Hideki Nakamura, Hajime Sano, Kazuo Tsubota, Yoko Ogawa, Etsuko Takamura, Ichiro Saito, Hiroko Inoue, Seiji Nakamura, Masafumi Moriyama, Tsutomu Takeuchi, Yoshiya Tanaka, Shintaro Hirata, Tsuneyo Mimori, Isao Matsumoto, Takayuki Sumida
OBJECTIVES: To compare the performance of the new 2016 American College of Rheumatology (ACR)-European League Against Rheumatism (EULAR) classification criteria for primary Sjögren's syndrome (SS) with 1999 revised Japanese Ministry of Health criteria for diagnosis of SS (JPN), 2002 American-European Consensus Group classification criteria for SS (AECG) and 2012 ACR classification criteria for SS (ACR) in Japanese patients. METHODS: The study subjects were 499 patients with primary SS (pSS) or suspected pSS who were followed up in June 2012 at 10 hospitals in Japan...
March 22, 2017: Annals of the Rheumatic Diseases
Fulvia Ceccarelli, Laura Massaro, Carlo Perricone, Monica Pendolino, Enrica Cipriano, Simona Truglia, Francesca Miranda, Francesca Romana Spinelli, Cristiano Alessandri, Guido Valesini, Fabrizio Conti
OBJECTIVES: Jaccoud's arthropathy (JA) is a deforming, non-erosive arthritis, occurring in 2-35% of systemic lupus erythematosus (SLE) patients. We aimed to evaluate JA patients in a wide monocentric SLE cohort in terms of clinical, serological and ultrasonographic features. METHODS: Consecutive SLE patients (ACR criteria 1997) were evaluated. The JA index was applied for patients with reducible deformities. Patients with a JA index ≥5 underwent physical examination, blood testing and ultrasound (US) assessment...
March 23, 2017: Clinical and Experimental Rheumatology
J Antonio Aviña-Zubieta, Michael Jansz, Eric C Sayre, Hyon K Choi
OBJECTIVE: To estimate the future risk and time trends of venous thromboembolism (VTE) in individuals with newly diagnosed primary Sjögren syndrome (pSS) in the general population. METHODS: Using a population database that includes all residents of British Columbia, Canada, we created a study cohort of all patients with incident SS and up to 10 controls from the general population matched for age, sex, and entry time. We compared incidence rates (IR) of pulmonary embolism (PE), deep vein thrombosis (DVT), and VTE between the 2 groups according to SS disease duration...
March 15, 2017: Journal of Rheumatology
Michael Croft, Richard M Siegel
TNF blockers are highly efficacious at dampening inflammation and reducing symptoms in rheumatic diseases such as rheumatoid arthritis, psoriatic arthritis and ankylosing spondylitis, and also in nonrheumatic syndromes such as inflammatory bowel disease. As TNF belongs to a superfamily of 19 structurally related proteins that have both proinflammatory and anti-inflammatory activity, reagents that disrupt the interaction between proinflammatory TNF family cytokines and their receptors, or agonize the anti-inflammatory receptors, are being considered for the treatment of rheumatic diseases...
April 2017: Nature Reviews. Rheumatology
Julia Yu-Yun Lee, Chao-Kai Hsu, Ming-Fei Liu, Sheau-Chiou Chao
OBJECTIVE: Persistent pruritic eruptions (PPE) are common among our patients with adult-onset Still disease (AOSD). We aimed to characterize the clinicopathologic features of the AOSD-associated evanescent and persistent rashes. METHODS: We reviewed the clinicopathologic features of the skin lesions from all AOSD cases diagnosed in our hospital during 1988 to 2009. The diagnoses were based on Yamaguchi criteria for AOSD. RESULTS: Altogether, there were 36 patients (6 men and 30 women) with age of onset ranging from 17 to 67 years (average 35...
December 2012: Seminars in Arthritis and Rheumatism
Nobuyuki Kikuchi, Masataka Satoh, Mikio Ohtsuka, Toshiyuki Yamamoto
Typical skin rash, which appears and disappears along with respective rise and fall of fever, is well-known, and included as one of the major criteria of adult-onset Still's disease (AOSD) (Yamaguchi's criteria). In addition, various skin lesions are occasionally observed in association with AOSD. Persistent pruritic eruptions present with some clinical features, such as urticarial erythema, flagellate erythema, erythematous, slightly scaly or crusted papules, and/or plaques on the trunk and extremities. These lesions show unique histological features such as dyskeratosis with a peculiar, distinctive distribution in the upper epidermis and cornified layers with focal hyperkeratosis...
May 2014: Journal of Dermatology
Toshiyuki Yamamoto
Adult-onset Still's disease (AOSD) is a systemic inflammatory condition, characterized by a high spiking fever, leukocytosis with neutrophilia, arthralgia, and skin rash. Typical skin rash is an evanescent, salmon-pink erythema predominantly involving extremities, which is included as one of the diagnostic criteria; however, recent findings show that not only typical evanescent rash but also various skin lesions are associated with AOSD. The representative characteristic skin lesion among the non-classical skin rash is called persistent pruritic papules and plaques, which presents erythematous, slightly scaly papules with linear configuration on the trunk...
August 2012: Rheumatology International
Janet Dua, Anupama Nandagudi, Nurhan Sutcliffe
Mycoplasma pneumoniae is well known to be a frequent cause of atypical pneumonia worldwide. However, it may also present with a wide variety of clinical features, including cutaneous symptoms, which are not widely recognised. Urticarial vasculitis occurring with M. pneumoniae has been described to occur in only one other case report. This amalgamation of non-specific clinical symptoms and signs can lead to a diagnostic dilemma. We describe a case of M. pneumoniae infection presenting with extrapulmonary manifestations and urticarial vasculitis, which was misdiagnosed as adult-onset Still's disease (AOSD)...
December 2012: Rheumatology International
Alessandra Cozzi, Anastasia Papagrigoraki, Domenico Biasi, Chiara Colato, Giampiero Girolomoni
Adult onset Still's disease (AOSD) is a systemic inflammatory disorder of unknown etiology and pathogenesis characterized by high spiking fever, arthralgia or arthritis, sore throat, lymphadenopathy, hepatosplenomegaly, serositis, and transient cutaneous manifestations. Although more common in children, cases are seen also in adults. Cutaneous involvement is common and may be suggestive for the diagnosis. A case of AOSD in a 35-year-old man is reported here, presenting with urticarial maculopapular rash of trunk, high spiking fever, acute respiratory distress syndrome, and myopericarditis...
May 2016: Clinical Rheumatology
Natalie Z Sun, Elizabeth A Brezinski, Jacqueline Berliner, Anna Haemel, M Kari Connolly, Lianne Gensler, Timothy H McCalmont, Kanade Shinkai
Adult-onset Still disease (AOSD) is a systemic inflammatory disorder that is clinically characterized by a heterogeneous constellation of symptoms and signs. Though an evanescent eruption is the classic cutaneous finding, recent literature has highlighted atypical rashes associated with Still disease. A second emerging concept in presentations of AOSD is its association with malignancy. This review focuses on these concepts: the clinical spectrum of atypical skin manifestations and AOSD as a paraneoplastic phenomenon...
August 2015: Journal of the American Academy of Dermatology
Tove Ragna Reksten, Christopher J Lessard, Kathy L Sivils
The genes associated with Sjögren syndrome (SS) can be assigned to the NF-kB pathway, the IFN signaling pathway, lymphocyte signaling, and antigen presentation. The frequencies of risk variants show they are common with modest genetic effects. The strongest genetic association outside the human leukocyte antigen region is in IRF5, a gene relevant in the IFN signaling pathway and for B cell differentiation. Although no association has been found with the NF-kB gene itself, associations in TNFAIP3 and TNIP1 (both genome-wide significant), VCAM1 and IRAK1BP (both suggestive), point to genetic explanations for dysregulation of the NF-kB pathway in SS...
August 2016: Rheumatic Diseases Clinics of North America
Mary Beth Yu, William H R Langridge
Rheumatoid arthritis (RA) is a systemic autoimmune disease that causes joint pain, inflammation, and loss of function. Disease pathogenesis involves activation and proliferation of autoreactive pro-inflammatory effector T cells. While the details of RA onset and progression remain controversial, dendritic cell (DC) numbers dramatically increase in the synovial joint tissues of RA patients. Based on their key functions as antigen-presenting cells and inducers of T cell differentiation, DCs may play an important role in the initiation of joint inflammation...
February 24, 2017: Rheumatology International
G J Kahaly, J Zimmermann, M P Hansen, F Gundling, F Popp, M Welcker
Polyglandular autoimmune syndromes encompass several endocrine and nonendocrine autoimmune disorders with variable onset and phenotype. Rheumatoid and gastroenterological symptoms in patients with autoimmune polyglandular syndromes are suggestive of additional rheumatoid gastrointestinal and hepatological autoimmune diseases. Autoimmune gastritis, celiac disease, autoimmune hepatitis, rheumatoid arthritis, Sjögren syndrome, and systemic Lupus erythematodus are of particular clinical relevance. In addition, unspecific rheumatoid and gastrointestinal attendant symptoms of the existing autoimmune endocrinopathy must be considered...
February 23, 2017: Der Internist
Vicenç Torrente-Segarra, Hèctor Corominas, Carlos Sánchez-Piedra, Mónica Fernández-Castro, José Luis Andreu, Víctor Manuel Martínez-Taboada, Alejandro Olivé, José Rosas, Fernando Sánchez-Alonso
OBJECTIVES: To assess fibromyalgia (FM) prevalence in a large cohort of primary Sjögren's syndrome patients (pSS) from a National Database. METHODS: Data included in the national retrospective register of pSS patients of the Spanish Society of Rheumatology (SJOGRENSER) were analysed. RESULTS: 437 pSS patients were included and a 14.6% of FM prevalence was found. FM-pSS patients significantly showed more constitutional, fatigue and arthralgia symptoms, splenomegaly, genital, skin and ear involvement and dyslipidaemia (p<0...
February 24, 2017: Clinical and Experimental Rheumatology
Camille Roubille, Nathalie Rincheval, Maxime Dougados, René-Marc Flipo, Jean-Pierre Daurès, Bernard Combe
OBJECTIVE: To explore the 7-year tolerability profile of glucocorticoids (GC) for early rheumatoid arthritis (RA). METHODS: We examined data for 602 patients with RA from the early arthritis Etude et Suivi des POlyarthrites Indifférenciées Récentes (ESPOIR) cohort (<6 months disease duration) stratified into two groups: with or without GC treatment at least once during follow-up (median 7 years (IQR 0.038-7.65)). The main outcome was a composite of death, cardiovascular disease (including myocardial ischaemia, cerebrovascular accident and heart failure), severe infection and fracture...
February 17, 2017: Annals of the Rheumatic Diseases
Debashish Danda, Rohan Sharma, Dat Truong, Kristi A Koelsch, Biji T Kurien, Harini Bagavant, Umesh Deshmukh, C Erick Kaufman, David M Lewis, Donald U Stone, Lida Radfar, Astrid Rasmussen, Kathy L Sivils, Robert H Scofield
OBJECTIVES: To characterise the serological and clinical findings in primary Sjögren's syndrome in which anti-La was found without anti-Ro. We hypothesised that a significant portion of these are falsely negative for anti-Ro60. METHODS: Twenty-nine sera from primary Sjögren's syndrome patients were tested for antibodies directed against La and Ro. Anti-La was detected using bovine La treated with or without DNAase and RNAase to identify potential false positivity...
February 20, 2017: Clinical and Experimental Rheumatology
M Vadacca, D Margiotta, D Sambataro, F Buzzulini, M Lo Vullo, A Rigon, A Afeltra
OBJECTIVES: BAFF and APRIL belong to the tumor necrosis factor (TNF) superfamily and are crucial for the survival, maturation, and differentiation of B cells. Aim of the study is to evaluate BAFF and APRIL in patients affected by Sjögren syndrome (SS) and systemic lupus erythematosus (SLE). METHODS: Sixty patients, (40 SLE, 20 SS) and 20 healthy subjects were enrolled in this study. All subjects were evaluated for laboratory data (ESR, CRP, immunoglobulin G, A and M, complement fragments C3 and C4, LDH, beta2microglobulin, serum levels of rheumatoid factor), autoantibodies (ANA; ENA-SSA, -SSB, -Sm) and lymphocytes subpopulations...
October 2010: Reumatismo
Fengyuan Deng, Juan Chen, Junfeng Zheng, Yan Chen, Renliang Huang, Junping Yin, Xing Gao, Qingyan Lin, Chaoyang Huang, Yushan Gao, Xinhua Yu, Zuguo Liu
AIM: Primary Sjögren's syndrome (pSS) is an autoimmune disease affecting exocrine glands. Both autoreactive T cells and B cells are involved in the development of pSS, but their exact contribution to the pathogenesis is not clear. Here, we aimed to investigate the association of B-cell activating factor (BAFF) and interleukin (IL)-17A with subphenotypes of pSS. METHODS: Peripheral blood samples were collected from 31 pSS patients and 28 healthy controls. The serum levels of BAFF and IL-17A were quantified by sandwich ELISA...
July 2016: International Journal of Rheumatic Diseases
Arjun Sharma, Jeremy Kiripolsky, Ekaterina Klimatcheva, Alan Howell, Farzad Fereidouni, Richard Levenson, Thomas L Rothstein, Jill M Kramer
Sjögren's syndrome (SS) is a debilitating autoimmune disease. Patients with SS may develop xerostomia. This process is progressive, and there are no therapeutics that target disease etiology. We hypothesized BAFF receptor (BAFFR) blockade would mitigate SS disease development, and neutralization of CXCL13 and BAFF signaling would be more efficacious than BAFFR blockade alone. We treated NOD/ShiLtJ SS mice with soluble BAFF receptor (BAFFR-Fc) or anti-CXCL13/BAFFR-Fc in combination, prior to the development of clinical disease...
March 2016: Clinical Immunology: the Official Journal of the Clinical Immunology Society
2017-02-23 08:26:38
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