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Current Opinion in Rheumatology

Yves Allenbach, Olivier Benveniste
PURPOSE OF REVIEW: In the past decade, immune-mediated necrotizing myopathies have emerged as a separate entity in the heterogenous group of autoimmune myopathies. This group is characterized by clinical manifestations restricted to the muscle tissue, and until recently, the definition was based on muscular pathological features. RECENT FINDINGS: It was shown that they are the most severe autoimmune myopathies in term of muscle damages. They have been associated with two myositis-specific antibodies: either anti-signal recognition particle (anti-SRP) or anti-hydroxy-3-methylglutaryl-CoA reductase (anti-HMGCR) antibodies...
September 19, 2018: Current Opinion in Rheumatology
Zsuzsanna H McMahan, Laura K Hummers
PURPOSE OF REVIEW: This review provides important updates in systemic sclerosis (SSc)-related gastrointestinal disease, with a particular focus on the diagnosis and management of dysmotility. RECENT FINDINGS: In the past 2 years, several studies were published that present interesting diagnostic insights into SSc and gastrointestinal dysmotility. Studies focusing on new therapies and the novel application of existing therapies, both in SSc and non-SSc-associated gastrointestinal dysmotility syndromes, demonstrate progress in the management of these challenging complications...
September 17, 2018: Current Opinion in Rheumatology
Flavia V Castelino, Paul F Dellaripa
PURPOSE OF REVIEW: Systemic sclerosis is a systemic autoimmune disorder wherein interstitial lung disease (ILD) is the major source or morbidity and mortality. Research into mechanisms of fibrosis and its intersection with autoimmunity, specifically lung fibrosis, has accelerated and been applied to autoimmune disorders such as scleroderma. This review highlights important emerging insights and treatment trials. RECENT FINDINGS: The important elements of this review focus on the challenges faced in identifying patients not only who develop lung disease but who are at a higher risk for progression given the heterogeneous natural history of ILD in scleroderma...
September 17, 2018: Current Opinion in Rheumatology
Patrizia Fuschiotti
PURPOSE OF REVIEW: Dysregulation of both the innate and the adaptive immune systems has been identified in systemic sclerosis (SSc). However, the mechanisms underlying aberrant immune cell function remain poorly understood. T cells represent a predominant cell type in the affected tissues of patients, particularly in the early inflammatory stage of the disease. Antigen specificity of infiltrating T cells has not been identified; however, recent studies implicate specific T-cell subsets and the cytokines they produce in SSc pathogenesis by modulating the development of autoimmunity, inflammation and fibrosis...
September 17, 2018: Current Opinion in Rheumatology
Anna Ghirardello, Andrea Doria
PURPOSE OF REVIEW: The aim of this study was to provide the most recent evidence on clinical utility of myositis-specific autoantibodies (MSAs) in the management of patients with myositis. RECENT FINDINGS: In the last few years, several evidences have emerged on the clinical and pathogenetic role of established and novel MSA. Antisynthetase antibodies represent a reliable biomarker for pulmonary involvement also in patients with connective tissue diseases other than myositis...
September 17, 2018: Current Opinion in Rheumatology
Max Brown, Steven O'Reilly
PURPOSE OF REVIEW: Systemic sclerosis (SSc) is an autoimmune connective tissue disease in which inflammation and cytokine dysregulation leads to skin fibrosis. Toll-like receptors (TLRs) are conserved pattern recognition receptors, recognizing pathogens danger-associated molecular patterns (DAMPs) that elicit a cascade of proinflammatory signaling. Recently, TLRs have been found to be critically important in SSc pathogenesis, with increased levels of the TLRs and their ligands present in the disease...
September 17, 2018: Current Opinion in Rheumatology
Laure Gallay, Catherine Gayed, Baptiste Hervier
PURPOSE OF REVIEW: Antisynthetase syndrome (ASyS) is an acquired myopathy characterized by the presence of myositis-specific autoantibodies directed against tRNA-synthetases. ASyS is potentially life threatening due to lung involvement and treatment remains a challenge to date. With symptoms not limited to muscles but also involving lung, skin and joints, ASyS appears specific and has a particular pathogenesis, different from the other inflammatory myopathies. This review is intended to discuss the current understanding of ASyS pathogenesis, pointing its current knowledge and also the crucial prospects that may lead to critical improvement of ASyS care...
September 2, 2018: Current Opinion in Rheumatology
Iago Pinal-Fernandez, Andrew L Mammen
PURPOSE OF REVIEW: The purpose of this article is to review the etiopathogenesis of dermatomyositis, including the predisposing factors, triggers, inflammatory cells, pathways and target antigens associated with dermatomyositis. RECENT FINDINGS: During the last few years, we have made considerable progress in unveiling the etiopathogenesis of dermatomyositis. In the first place, we have defined genes within the major histocompatibility complex locus as the greatest genetic risk factor for the disease...
August 24, 2018: Current Opinion in Rheumatology
Dilli R Poudel, Chris T Derk
PURPOSE OF REVIEW: Systemic sclerosis is a debilitating rheumatic disease with high morbidity and mortality. This review attempts to provide the most recent update on mortality and survival and their determinants in systemic sclerosis (SSc). RECENT FINDINGS: SSc remains an uncommon rheumatic disease with high mortality. There have been attempts to devise more comprehensive but simpler scoring systems to prognosticate survival in SSc, which will influence triaging of patients and guide the utilization of aggressive treatment strategies...
August 24, 2018: Current Opinion in Rheumatology
Julie J Paik
PURPOSE OF REVIEW: This review discusses the most updated literature of muscle disease in scleroderma in the past year. RECENT FINDINGS: In the past year, two studies have highlighted that fibrosis is a prevalent histopathologic feature in muscle biopsies of patients with scleroderma muscle disease. In addition, microangiopathy was a common co-feature on muscle biopsies. A fibrosing myopathy, or fibrosis predominance on muscle histopathology, is associated with a unique clinical phenotype in patients with scleroderma...
August 24, 2018: Current Opinion in Rheumatology
Christopher A Mecoli, Livia Casciola-Rosen
PURPOSE OF REVIEW: New research continues to provide important insights into the utility of antibody specificities. This review provides an update of recent findings, and the important insights they provide into disease mechanism. RECENT FINDINGS: A growing number of autoantibodies have been discovered in scleroderma patients with unique clinical associations. A subgroup of these antibodies may have functional consequences and contribute to disease pathogenesis, driving the vascular and fibrotic phenotype...
August 24, 2018: Current Opinion in Rheumatology
Alexander Oldroyd, Hector Chinoy
PURPOSE OF REVIEW: The aim of this review was to summarize key developments in classification and diagnosis of the idiopathic inflammatory myopathies (IIMs). RECENT FINDINGS: The recently published European League Against Rheumatism/American College of Rheumatology (EULAR/ACR) classification criteria for the IIMs provide a comprehensive, accurate and data-driven approach to identification of IIM cases appropriate for inclusion in research studies. Further, recent studies have advanced understanding of clinical manifestations of the IIMs and delineated the role of imaging, particularly magnetic resonance...
August 22, 2018: Current Opinion in Rheumatology
Antonia Valenzuela, Paula Song, Lorinda Chung
PURPOSE OF REVIEW: To provide an update on the available literature regarding the epidemiology, pathophysiology, diagnosis, and treatment of calcinosis cutis in patients with systemic sclerosis (SSc). RECENT FINDINGS: We identified observational studies that describe the frequency of calcinosis in SSc and associated clinical features; molecular studies exploring potential pathogenic mechanisms; and case reports and case series describing new diagnostic approaches and treatments...
August 16, 2018: Current Opinion in Rheumatology
Giulia C Varnier, Clarissa A Pilkington, Lucy R Wedderburn
PURPOSE OF REVIEW: The aim of this article is to provide a summary of the recent therapeutic advances and the latest research on outcome measures for juvenile dermatomyositis (JDM). RECENT FINDINGS: Several new international studies have developed consensus-based guidelines on diagnosis, outcome measures and treatment of JDM to standardize and improve patient care. Myositis-specific antibodies together with muscle biopsy histopathology may help the clinician to predict disease outcome...
August 16, 2018: Current Opinion in Rheumatology
Dafni A Gyftaki-Venieri, David J Abraham, sMarkella Ponticos
PURPOSE OF REVIEW: The persistence of myofibroblasts is a key feature of fibrosis and in fibrotic diseases including scleroderma. This review evaluates the emerging concepts of the origins and cell populations that contribute to myofibroblasts and the molecular mechanisms that govern phenotypic conversion and that highlight opportunities for new interventional treatments in scleroderma. RECENT FINDINGS: Studies have defined heterogeneity in fibroblast-like cells that can develop into myofibroblast in normal wound healing, scarring and fibrosis...
July 31, 2018: Current Opinion in Rheumatology
James B Lilleker
PURPOSE OF REVIEW: To describe recent advancements in diagnostic and therapeutic approaches to inclusion body myositis (IBM). RECENT FINDINGS: Our understanding of the implications of anti-cytosolic 5'-nucleotidase 1A autoantibody status in IBM and other diseases is increasing. Muscle imaging using magnetic resonance techniques and ultrasound is increasingly being performed and characteristic patterns of muscle involvement may help with diagnosis. Longitudinal imaging studies are likely to help with monitoring and as an outcome measure in clinical trials...
July 31, 2018: Current Opinion in Rheumatology
Micaela Fredi, Ilaria Cavazzana, Franco Franceschini
PURPOSE OF REVIEW: To provide the most recent evidence on the overlap myositis. RECENT FINDINGS: Several new evidences on the overlap myositides have recently emerged. Regarding the classical myositis associated antibodies, several contributions focused on a better definition of the clinical associations and the disease course associated with these autoantibodies. Moreover, in the last years, new autoantibodies in idiopathic inflammatory myositis or other connective tissue diseases have been identified [namely anti-RuvBL1/2, poly-U-binding factor 60 kDa protein (PUF-60) and cytosolic 5'-nucleotidase 1A (NT5C1A)], and an increasing number of publications allow now to consider them as new myositis-associated antibodies with probably their own peculiar clinical profile...
July 31, 2018: Current Opinion in Rheumatology
Julia Spierings, Femke C C van Rhijn-Brouwer, Jacob M van Laar
PURPOSE OF REVIEW: To provide an overview of recently published work on autologous hematopoietic stem-cell transplantation (HSCT) in patients with systemic sclerosis (SSc). RECENT FINDINGS: Superiority of HSCT vs. intravenous cyclophosphamide pulses was demonstrated in the randomized controlled American Scleroderma: Cyclophosphamide or Transplantation (SCOT) Trial (n = 75), supporting the results from earlier studies. In the SCOT Trial, total body irradiation was used instead of the nonmyeloablative regimens used in other trials, and considered well tolerated during a follow-up time of 4...
July 30, 2018: Current Opinion in Rheumatology
Albert Selva-O'Callaghan, Xavier Martinez-Gómez, Ernesto Trallero-Araguás, Iago Pinal-Fernández
PURPOSE OF REVIEW: Despite the well-recognized association between malignancy and myositis, definite data indicating the best strategy for diagnosing cancer in myositis patients is lacking. In this article, we review the data on cancer screening in patients with myositis, and propose an algorithm for this purpose based on recently published data. RECENT FINDINGS: Evidence has recently emerged supporting blind screening in patients with certain myositis phenotypes...
June 29, 2018: Current Opinion in Rheumatology
Marco Lopez Velazquez, Kristin B Highland
PURPOSE OF REVIEW: Systemic lupus erythematosus (SLE) and Sjögren syndrome are chronic autoimmune inflammatory disorders that can present with multiorgan involvement including the lungs. This review will focus on recent literature pertaining to the epidemiology, pathogenesis, clinical presentation and diagnosis and management of SLE and Sjögren syndrome-associated pulmonary conditions. RECENT FINDINGS: Pulmonary manifestations of both disease entities have been well characterized and lung involvement can be observed during the course of the disease in most cases...
September 2018: Current Opinion in Rheumatology
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