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Connective tissue disease

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14 papers 0 to 25 followers
Tim Both, Virgil A S H Dalm, P Martin van Hagen, Paul L A van Daele
Primary Sjögren's syndrome (pSS) is a systemic autoimmune disease, characterized by lymphocytic infiltration of the secretory glands. This process leads to sicca syndrome, which is the combination of dryness of the eyes, oral cavity, pharynx, larynx and/or vagina. Extraglandular manifestations may also be prevalent in patients with pSS, including cutaneous, musculoskeletal, pulmonary, renal, hematological and neurological involvement. The pathogenesis of pSS is currently not well understood, but increased activation of B cells followed by immune complex formation and autoantibody production are thought to play important roles...
2017: International Journal of Medical Sciences
Alessia Alunno, Onelia Bistoni, Francesco Carubbi, Valentina Valentini, Giacomo Cafaro, Elena Bartoloni Bocci, Roberto Giacomelli, Roberto Gerli
OBJECTIVES: Saccharomyces cerevisiae is a common yeast used in the food industry. IgG and IgA antibodies against the phosphopeptidomannan of the S. cerevisiae cell wall (ASCA) are a well known marker of disease severity in Crohn's disease. Moreover, a number of studies assessed ASCA in several systemic and organ-specific autoimmune diseases postulating molecular mimicry as a possible link between ASCA and autoimmunity. However, since they have never been tested in primary Sjögren's syndrome (pSS), the purpose of this study was to investigate these antibodies in a large cohort of pSS patients, compared to healthy donors (HD), and their significance as potentially helpful biomarker in a clinical setting...
June 29, 2017: Clinical and Experimental Rheumatology
Salem Almaani, Alexa Meara, Brad H Rovin
SLE is a chronic inflammatory disease that affects the kidneys in about 50% of patients. Lupus nephritis is a major risk factor for overall morbidity and mortality in SLE, and despite potent anti-inflammatory and immunosuppressive therapies still ends in CKD or ESRD for too many patients. This review highlights recent updates in our understanding of disease epidemiology, genetics, pathogenesis, and treatment in an effort to establish a framework for lupus nephritis management that is patient-specific and oriented toward maintaining long-term kidney function in patients with lupus...
May 8, 2017: Clinical Journal of the American Society of Nephrology: CJASN
Z Betteridge, N McHugh
The idiopathic inflammatory myopathies are characterized by muscle weakness, skin disease and internal organ involvement. Autoimmunity is known to have a role in myositis pathogenesis, and myositis-specific autoantibodies, targeting important intracellular proteins, are regarded as key biomarkers aiding in the diagnosis of patients. In recent years, a number of novel myositis autoantibodies including anti-TIF1, anti-NXP2, anti-MDA5, anti-SAE, anti-HMGCR and anti-cN1A have been identified in both adult and juvenile patients...
July 2016: Journal of Internal Medicine
Caroline H Shiboski, Stephen C Shiboski, Raphaèle Seror, Lindsey A Criswell, Marc Labetoulle, Thomas M Lietman, Astrid Rasmussen, Hal Scofield, Claudio Vitali, Simon J Bowman, Xavier Mariette
OBJECTIVES: To develop and validate an international set of classification criteria for primary Sjögren's syndrome (SS) using guidelines from the American College of Rheumatology (ACR) and the European League Against Rheumatism (EULAR). These criteria were developed for use in individuals with signs and/or symptoms suggestive of SS. METHODS: We assigned preliminary importance weights to a consensus list of candidate criteria items, using multi-criteria decision analysis...
January 2017: Annals of the Rheumatic Diseases
Ming-Han Chen, Chun-Ku Chen, Hsiao-Ping Chou, Ming-Huang Chen, Chang-Youh Tsai, Deh-Ming Chang
OBJECTIVES: Interstitial lung disease (ILD) is one of the major systemic manifestations of primary Sjögren's syndrome (pSS). The aim of this study was to evaluate the therapeutic effect of rituximab on pSS patients with ILD. METHODS: Pulmonary function test results, including diffusing capacity for carbon monoxide (DLCO) and DLCO/alveolar volume (Va) ratio, and high-resolution computed tomography (HRCT) findings/scores in ten pSS patients with ILD treated with rituximab were retrospectively investigated...
November 2016: Clinical and Experimental Rheumatology
Marek M Chojnowski, Anna Felis-Giemza, Marzena Olesińska
Capillaroscopy is a non-invasive, easy and safe diagnostic technique designed to evaluate small vessels of the microcirculation in the nailfold. It can reveal both the general architecture of capillary rows and fine details of particular vessels. The most important indications for performing capillaroscopy include differential diagnosis of primary and secondary Raynaud's phenomenon, as well as assessment of scleroderma spectrum disorders. In systemic sclerosis capillary abnormalities appear and evolve in a clearly defined sequence called the scleroderma pattern, which correlates with internal organ involvement...
2016: Reumatologia
Zhe Cai, Chun Kwok Wong, Jie Dong, Delong Jiao, Man Chu, Ping Chung Leung, Clara Bik San Lau, Ching Po Lau, Lai Shan Tam, Christopher Wai Kei Lam
BACKGROUND: Ganoderma lucidum (Lingzhi; LZ) and San-Miao-San (SMS) are Chinese medicines (CMs) used to treat inflammatory ailments and numbing syndrome/arthralgia syndrome (Bi Zheng), respectively. Given that the main symptoms of systemic lupus erythematosus (SLE) include inflammation of the joints, joint pain, edema and palpitations of the heart because of problems associated with Bi Zheng, it was envisaged that LZ and SMS could be used as potential treatments for this autoimmune disease...
2016: Chinese Medicine
Samuel K Shinjo, Eloísa Bonfá, Daniel Wojdyla, Eduardo F Borba, Luis A Ramirez, Hugo R Scherbarth, João C Tavares Brenol, Rosa Chacón-Diaz, Oscar J Neira, Guillermo A Berbotto, Ignacio Garcia De La Torre, Eduardo M Acevedo-Vázquez, Loreto Massardo, Leonor A Barile-Fabris, Francisco Caeiro, Luis H Silveira, Emilia I Sato, Sandra Buliubasich, Graciela S Alarcón, Bernardo A Pons-Estel
OBJECTIVE: To evaluate the beneficial effect of antimalarial treatment on lupus survival in a large, multiethnic, international longitudinal inception cohort. METHODS: Socioeconomic and demographic characteristics, clinical manifestations, classification criteria, laboratory findings, and treatment variables were examined in patients with systemic lupus erythematosus (SLE) from the Grupo Latino Americano de Estudio del Lupus Eritematoso (GLADEL) cohort. The diagnosis of SLE, according to the American College of Rheumatology criteria, was assessed within 2 years of cohort entry...
March 2010: Arthritis and Rheumatism
Katrine Brække Norheim, Juliana Imgenberg-Kreuz, Kristin Jonsdottir, Emiel A M Janssen, Ann-Christine Syvänen, Johanna K Sandling, Gunnel Nordmark, Roald Omdal
OBJECTIVE: Chronic fatigue is a common, disabling and poorly understood phenomenon. Recent studies indicate that epigenetic mechanisms may be involved in the expression of fatigue, a prominent feature of primary SS (pSS). The aim of this study was to investigate whether DNA methylation profiles of whole blood are associated with fatigue in patients with pSS. METHODS: Forty-eight pSS patients with high (n = 24) or low (n = 24) fatigue as measured by a visual analogue scale were included...
June 2016: Rheumatology
Salvatore De Vita, Luca Quartuccio, Raphaèle Seror, Sara Salvin, Philippe Ravaud, Martina Fabris, Gaétane Nocturne, Saviana Gandolfo, Miriam Isola, Xavier Mariette
OBJECTIVE: To report the efficacy and safety of long-term treatment of SS with belimumab, targeting the B-cell-activating factor. METHODS: Patients with primary SS were included in the BELISS open-label phase II study, a 1-year open-label trial, if they were positive for anti-SSA or anti-SSB antibodies and had systemic complications or persistent salivary gland enlargement or early disease or biomarkers of B-cell activation. They received belimumab, 10 mg/kg i.v...
December 2015: Rheumatology
Francesco Benvenuti, Mariele Gatto, Maddalena Larosa, Luca Iaccarino, Leonardo Punzi, Andrea Doria
INTRODUCTION: Risk of developing cardiovascular disease (CVD) is increased in systemic lupus erythematosus (SLE) compared with the general population. Traditional risk factors cannot account for the totality of CV events and adequate prevention may be challenging. AREAS COVERED: This review summarizes traditional and emerging risk factors of CVD in SLE patients and goes over potential pathogenic mechanisms involved in CVD development. Role of commonly used drugs and preventive strategies exploitable in everyday clinical practice are also discussed...
2015: Expert Opinion on Drug Safety
Eric Hachulla, Pierre-Yves Hatron, Patrick Carpentier, Christian Agard, Emmanuel Chatelus, Patrick Jego, Luc Mouthon, Viviane Queyrel, Anne-Laure Fauchais, Ulrique Michon-Pasturel, Roland Jaussaud, Alexis Mathian, Brigitte Granel, Elisabeth Diot, Dominique Farge-Bancel, Arsène Mekinian, Jérôme Avouac, Hélène Desmurs-Clavel, Pierre Clerson
OBJECTIVE: To assess the effect of sildenafil, a phosphodiesterase type 5 inhibitor, on digital ulcer (DU) healing in systemic sclerosis (SSc). METHODS: Randomised, placebo-controlled study in patients with SSc to assess the effect of sildenafil 20 mg or placebo, three times daily for 12 weeks, on ischaemic DU healing. The primary end point was the time to healing for each DU. Time to healing was compared between groups using Cox models for clustered data (two-sided tests, p=0...
June 2016: Annals of the Rheumatic Diseases
Alsya J Affandi, Timothy R D J Radstake, Wioleta Marut
Systemic sclerosis (SSc) is a complex autoimmune disease in which immune activation, vasculopathy, and extensive fibrosis of the skin and internal organs are among the principal features. SSc is a heterogeneous disease with varying manifestations and clinical outcomes. Currently, patients' clinical evaluation often relies on subjective measures, non-quantitative methods, or requires invasive procedures as markers able to predict disease trajectory or response to therapy are lacking. Therefore, current research is focusing on the discovery of useful biomarkers reflecting ongoing inflammatory or fibrotic activity in the skin and internal organs, as well as being predictive of future disease course...
September 2015: Seminars in Immunopathology
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