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Aisling Kelly, Graham Dinsdale, Joanne Manning, Ariane L Herrick
No abstract text is available yet for this article.
February 19, 2019: Rheumatology
Matthew Wells, Emma Davies, Harsha Gunawardena
No abstract text is available yet for this article.
February 19, 2019: Rheumatology
Massimiliano Copetti, Silvia Morlino, Marina Colombi, Paola Grammatico, Andrea Fontana, Marco Castori
OBJECTIVES: This study is aimed at identifying discrete severity classes among adults with hypermobile Ehlers-Danlos syndrome (hEDS)/hypermobility spectrum disorders (HSD). METHODS: Subjects were selected according to the old and new nomenclatures and all completed a set of questionnaires exploring pain, fatigue, dysautonomic symptoms, coordination and attention/concentration deficits and quality of life in general. Data were investigated by hierarchical clustering on principal components...
February 19, 2019: Rheumatology
Juliana Imgenberg-Kreuz, Astrid Rasmussen, Kathy Sivils, Gunnel Nordmark
Primary Sjögren's syndrome (pSS) is considered to be a multifactorial disease, where underlying genetic predisposition, epigenetic mechanisms and environmental factors contribute to disease development. In the last 5 years, the first genome-wide association studies in pSS have been completed. The strongest signal of association lies within the HLA genes, whereas the non-HLA genes IRF5 and STAT4 show consistent associations in multiple ethnicities but with a smaller effect size. The majority of the genetic risk variants are found at intergenic regions and their functional impact has in most cases not been elucidated...
February 15, 2019: Rheumatology
Gwenny M Verstappen, Frans G M Kroese, Hendrika Bootsma
A histologic hallmark of primary SS (pSS) is lymphocytic infiltration of the salivary and lacrimal glands, in particular by CD4+ T and B cells. In the early stages of the disease, infiltrates are dominated by CD4+ T cells, while B cell accumulation occurs at later stages. Activated T cells contribute to pathogenesis by producing pro-inflammatory cytokines and by inducing B cell activation, which results in the establishment of a positive feedback loop. In the inflamed glandular tissues, many different CD4+ effector subsets are present, including IFN-γ-producing Th1 cells, IL-17-producing Th17 cells and IL-21-producing T follicular helper cells...
February 15, 2019: Rheumatology
Mariano Andrés, José-Antonio Bernal, Maria-Dolores Arenas, Eliseo Pascual
No abstract text is available yet for this article.
February 15, 2019: Rheumatology
Samira T Miyamoto, Valéria Valim, Benjamin A Fisher
Health-related quality of life (HRQoL) has an increasing role in medical decision-making. This review of the literature aims to provide an overview on HRQoL, costs, and work disability in SS, a disease characterized by focal lymphocytic infiltration of exocrine glands with no therapeutics of proven immunomodulatory potential. HRQoL is markedly reduced in SS in multiple studies across many countries when compared with HRQoL in healthy controls. The reduction in HRQoL is similar to that observed in other chronic diseases such as RA, SLE, FM and, interestingly, non-SS sicca syndrome...
February 15, 2019: Rheumatology
Elizabeth J Price, Alan N Baer
SS is a chronic, autoimmune disease of unknown aetiology for which there is no known curative treatment. Although dryness of the eyes and mouth are the classically described features, patients often experience drying of other mucosal surfaces and systemic manifestations, including fatigue and arthralgia. There is an association with other autoimmune diseases, especially thyroid disease, coeliac disease and primary biliary cholangitis. Systemic features may affect up to 70% and include inflammatory arthritis, skin involvement, haematological abnormalities, neuropathies, interstitial lung disease and a 5-10% lifetime risk of B cell lymphoma...
February 15, 2019: Rheumatology
Julie Mielle, Alice Tison, Divi Cornec, Laëtitia Le Pottier, Claire Daien, Jacques-Olivier Pers
Biological abnormalities associated with B lymphocytes are a hallmark of patients with primary Sjögren's syndrome. Those patients present abnormal distribution of B lymphocytes in peripheral blood and B cells in exocrine glands. B cells produce auto-antibodies, cytokines and present antigens but can also suppressive functions. In this review, we will summarize current knowledge on B cells in primary Sjögren's syndrome patients, demonstrate their critical role in the immunopathology of the disease and describe the past and current trials targeting B cells...
February 15, 2019: Rheumatology
Zahava Vadasz, Alexandra Balbir Gurman, Pierluigi Meroni, Dominique Farge, Yair Levi, Francesca Ingegnoli, Yolada Braun-Moscovici, Itzhak Rosner, Gleb Slobodin, Michael Rozenbaum, Nizar Jiries, Lisa Kaly, Nina Boulman, Karina Zilber, Shira Ginsberg, Abid Awisat, Yair Goldberg, Michael Lurie, Maria-Rosa Ghigna, Christophe Guignabert, Marc Humbert, Doron Rimar
OBJECTIVE: Lysyl oxidase (LOX) is an extracellular enzyme that cross-links collagen fibrils. LOX was found to be increased in serum of SSc patients and was suggested to be related to skin fibrosis, yet a vascular source of LOX has been demonstrated in idiopathic pulmonary arterial hypertension (iPAH). We aimed to validate elevated LOX serum levels in SSc and to study its correlation with clinical characteristics and investigate its main source at the tissue level. METHODS: A total of 86 established SSc patients were compared with 86 patients with very early diagnosis of systemic sclerosis (VEDOSS), 110 patients with primary RP (PRP) and 80 healthy controls...
February 15, 2019: Rheumatology
José M Pego-Reigosa, Juliana Restrepo Vélez, Chiara Baldini, Íñigo Rúa-Figueroa Fernández de Larrinoa
The information about comorbidities (excluding lymphoma) in primary Sjögren's syndrome (pSS) is relatively scarce. Cardiovascular disease, infections, musculoskeletal conditions or malignancy are likely the most relevant comorbid conditions in pSS. Different infections (particularly oral candidal infections) and fibromyalgia are extremely frequent in the daily clinical practice. On the other hand, the incidence of cardiovascular events and cancer in pSS is low, so information about them comes from large epidemiological studies or meta-analysis...
February 15, 2019: Rheumatology
Iris L A Bodewes, Albin Björk, Marjan A Versnel, Marie Wahren-Herlenius
Primary SS (pSS) is a rheumatic disease characterized by an immune-mediated exocrinopathy, resulting in severe dryness of eyes and mouth. Systemic symptoms include fatigue and joint pain and a subset of patients develop more severe disease with multi-organ involvement. Accumulating evidence points to involvement of innate immunity and aberrant activity of the type I IFN system in both the initiation and propagation of this disease. Analysis of the activity of IFN-inducible genes has evidenced that more than half of pSS patients present with a so-called 'type I IFN signature'...
February 15, 2019: Rheumatology
(no author information available yet)
No abstract text is available yet for this article.
February 11, 2019: Rheumatology
Kundan Iqbal, Dennis W Lendrem, Ben Hargreaves, John D Isaacs, Ben Thompson, Arthur G Pratt
Objective: The diagnostic value added by musculoskeletal ultrasound (MSUS) over standard clinical and laboratory parameters has proved difficult to quantify. The additive contribution to diagnostic classification of a pragmatic, 15 min MSUS protocol was appraised in a large, unselected cohort of early arthritis clinic attendees. Methods: Detailed baseline characteristics were recorded. Semi-quantitative MSUS scoring of the most symptomatic wrist, second/third MCPs and PIPs and second/fifth MTPs was recorded, along with the sonographer's scan impression (definitely inflammatory, possibly inflammatory or non-inflammatory)...
February 8, 2019: Rheumatology
Arielle Mendel, Sasha Bernatsky, Christian A Pineau, Yvan St-Pierre, John G Hanly, Murray B Urowitz, Ann E Clarke, Juanita Romero-Diaz, Caroline Gordon, Sang-Cheol Bae, Daniel J Wallace, Joan T Merrill, Jill Buyon, David A Isenberg, Anisur Rahman, Ellen M Ginzler, Michelle Petri, Mary Anne Dooley, Paul Fortin, Dafna D Gladman, Kristján Steinsson, Rosalind Ramsey-Goldman, Munther A Khamashta, Cynthia Aranow, Meggan Mackay, Graciela Alarcón, Susan Manzi, Ola Nived, Andreas Jönsen, Asad A Zoma, Ronald F van Vollenhoven, Manuel Ramos-Casals, Giuillermo Ruiz-Irastorza, Sam Lim, Kenneth C Kalunian, Murat Inanc, Diane L Kamen, Christine A Peschken, Søren Jacobsen, Anca Askanase, Jorge Sanchez-Guerrero, Ian N Bruce, Nathalie Costedoat-Chalumeau, Evelyne Vinet
Objectives: To assess the prevalence of combined hormonal contraceptives (CHCs) in reproductive-age women with SLE with and without possible contraindications and to determine factors associated with their use in the presence of possible contraindications. Methods: This observational cohort study included premenopausal women ages 18-45 years enrolled in the SLICC Registry ⩽15 months after SLE onset, with annual assessments spanning 2000-2017. World Health Organization Category 3 or 4 contraindications to CHCs (e...
February 8, 2019: Rheumatology
Amy E Anderson, Nicola J Maney, Nisha Nair, Dennis W Lendrem, Andrew J Skelton, Julie Diboll, Philip M Brown, Graham R Smith, Ruaidhrí J Carmody, Anne Barton, John D Isaacs, Arthur G Pratt
Objectives: Dysregulated signal transduction and activator of transcription-3 (STAT3) signalling in CD4+ T cells has been proposed as an early pathophysiological event in RA. We sought further evidence for this observation, and to determine its clinical relevance. Methods: Microarray technology was used to measure gene expression in purified peripheral blood CD4+ T cells from treatment-naïve RA patients and disease controls newly recruited from an early arthritis clinic...
February 8, 2019: Rheumatology
Femke C C van Rhijn-Brouwer, Julia Spierings, Anna van Rhenen, Jürgen Kuball, Jacob M van Laar
No abstract text is available yet for this article.
February 8, 2019: Rheumatology
Adam Rischin, Thilinie De Silva, Kim Le Marshall
No abstract text is available yet for this article.
February 7, 2019: Rheumatology
Sabine Löhr, Arif B Ekici, Steffen Uebe, Christian Büttner, Michaela Köhm, Frank Behrens, Beate Böhm, Michael Sticherling, Georg Schett, David Simon, Rotraut Mössner, Ali Nimeh, Vinzenz Oji, Gunter Assmann, Jürgen Rech, Rikard Holmdahl, Harald Burkhardt, André Reis, Ulrike Hüffmeier
No abstract text is available yet for this article.
February 7, 2019: Rheumatology
Robin M Ten Brinck, Leendert A Trouw, Annette H M van der Helm-van Mil
No abstract text is available yet for this article.
February 6, 2019: Rheumatology
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