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https://www.readbyqxmd.com/read/28293456/acute-digital-ischemia-a-rare-presentation-of-antisynthetase-syndrome
#1
Jin Ei Chan, Sandeep Palakodeti, Matthew J Koster
Antisynthetase syndrome (ASS) is recognized as a subgroup of idiopathic inflammatory myopathies (IIMs). It is associated with autoantibodies directed against aminoacyl-transfer ribonucleic acid (tRNA) synthetase enzymes. We report the first case of anti-PL-7/anti-SSA 52kD ASS presenting as acute digital ischemia, an association not described previously. Occlusive vasculopathy is a rare but serious manifestation that can be seen at presentation in patients with ASS and may herald the onset of severe interstitial lung disease (ILD)...
March 2017: European Journal of Rheumatology
https://www.readbyqxmd.com/read/28286105/animal-models-in-idiopathic-inflammatory-myopathies-how-to-overcome-a-translational-roadblock
#2
REVIEW
Ali Afzali, Tobias Ruck, Heinz Wiendl, Sven G Meuth
Idiopathic inflammatory myopathies (IIMs) encompass a heterogenic group of rare muscle diseases with common symptoms including muscle weakness and the presence of certain histological features. Since the pathogenesis remains unclear, therapeutic approaches in general comprise unspecific immunosuppression strategies that have been met with limited success. Therefore, a deeper understanding of the underlying pathophysiological mechanisms is critically required to assist in development of targeted therapies. Animal models have proven to be tremendously helpful in mechanistic studies and allow researchers to overcome the inevitable restrictions of human research...
March 7, 2017: Autoimmunity Reviews
https://www.readbyqxmd.com/read/28251541/profile-of-pediatric-idiopathic-inflammatory-myopathies-from-a-tertiary-care-center-of-eastern-india
#3
Sumantra Sarkar, Tanushree Mondal, Arpan Saha, Rakesh Mondal, Supratim Datta
OBJECTIVES: To report data on Idiopathic inflammatory myopathies (IIM) from eastern India. METHODS: All IIM patients diagnosed over the last 5 y (2011-2016) were included through a retrospective review of records from the hospital and specialty clinic at Institute of Postgraduate Medical Education & Research (I.P.G.M.E.&R.), Kolkata. RESULTS: Out of the 11 IIM patients, 9 had Juvenile dermatomyositis (JDM) and 2 had overlap myositis (OM) [with systemic lupus erythematosus (SLE) and scleroderma]...
March 2, 2017: Indian Journal of Pediatrics
https://www.readbyqxmd.com/read/28224635/infections-and-vaccinations-as-possible-triggers-of-inflammatory-myopathies
#4
Vidya Limaye, Caroline Smith, Barbara Koszyca, Peter Blumbergs, Sophia Otto
INTRODUCTION: The role of vaccinations and infections in triggering idiopathic inflammatory myopathies (IIM) has not been confirmed. METHODS: Among patients with histologically-confirmed myositis, infections or vaccinations administered prior to myositis onset were determined. The characteristics of this group were compared with controls (myositis patients without prior infection or vaccination). RESULTS: The frequency of IIM with a prior vaccination was 20 of 206, (9...
February 22, 2017: Muscle & Nerve
https://www.readbyqxmd.com/read/28193727/inference-of-gene-flow-in-the-process-of-speciation-an-efficient-maximum-likelihood-method-for-the-isolation-with-initial-migration-model
#5
Rui J Costa, Hilde Wilkinson-Herbots
The isolation-with-migration (IM) model is commonly used to make inferences about gene flow during speciation, using polymorphism data. However, Becquet and Przeworski (2009) report that the parameter estimates obtained by fitting the IM model are very sensitive to the model's assumptions, including the assumption of constant gene flow until the present. This paper is concerned with the isolation-with-initial-migration (IIM) model, which drops precisely this assumption. In the IIM model, one ancestral population divides into two descendant subpopulations, between which there is an initial period of gene flow and a subsequent period of isolation...
February 13, 2017: Genetics
https://www.readbyqxmd.com/read/28118525/predictors-of-reduced-health-related-quality-of-life-in-adult-patients-with-idiopathic-inflammatory-myopathies
#6
Michal Feldon, Payam Noroozi Farhadi, Hermine I Brunner, Lukasz Itert, Bob Goldberg, Abdullah Faiq, Jesse Wilkerson, Kathryn M Rose, Lisa G Rider, Frederick W Miller, Edward H Giannini
OBJECTIVE: Extensive studies on health-related quality of life (HRQOL) in idiopathic inflammatory myopathies (IIM) are lacking. Our objective was to document HRQOL and to identify factors associated with a reduced HRQOL in IIM patients. METHODS: A total of 1,715 patients (median age 49.9, 70% female, 87% Caucasian) who met probable or definite Bohan and Peter or Griggs criteria for myositis were included from the MYOVISION registry. HRQOL was ascertained via the SF-12v2® Health Survey questionnaire...
January 24, 2017: Arthritis Care & Research
https://www.readbyqxmd.com/read/28101144/histopathological-findings-in-systemic-sclerosis-related-myopathy-fibrosis-and-microangiopathy-with-lack-of-cellular-inflammation
#7
Claudio Corallo, Maurizio Cutolo, Nila Volpi, Daniela Franci, Margherita Aglianò, Antonio Montella, Chiara Chirico, Stefano Gonnelli, Ranuccio Nuti, Nicola Giordano
OBJECTIVES: The objective of this study was to identify specific histopathological features of skeletal muscle involvement in systemic sclerosis (SSc) patients. METHODS: A total of 35 out of 112 SSc-patients (32%, including 81% female and 68% diffuse scleroderma) presenting clinical, biological and electromyographic (EMG) features of muscle weakness, were included. Patients underwent vastus lateralis biopsy, assessed for individual pathologic features including fibrosis [type I collagen (Coll-I), transforming growth factor β (TGF-β)], microangiopathy [cluster of differentiation 31 (CD31), pro-angiogenic vascular endothelial growth factor A (VEGF-A), anti-angiogenic VEGF-A165b], immune/ inflammatory response [CD4, CD8, CD20, human leucocyte antigens ABC (HLA-ABC)], and membranolytic attack complex (MAC)...
January 2017: Therapeutic Advances in Musculoskeletal Disease
https://www.readbyqxmd.com/read/28088340/the-role-of-magnetic-resonance-imaging-techniques-in-evaluation-and-management-of-the-idiopathic-inflammatory-myopathies
#8
REVIEW
Jessica Day, Sandy Patel, Vidya Limaye
Magnetic resonance imaging (MRI) is an important tool in the evaluation of neuromuscular disorders. MRI accurately demonstrates muscle oedema, atrophy, subcutaneous pathology and fatty infiltration and also highlights the distribution of muscle involvement. This review examines the role of MRI in evaluation of the idiopathic inflammatory myopathies (IIMs), a heterogeneous group of autoimmune conditions characterised by muscle inflammation and a variety of extra-muscular manifestations. MRI has a clear role in aiding diagnosis of these conditions, guiding muscle biopsy, differentiating subtypes of IIM using a pattern-based approach, and monitoring disease activity in a longitudinal fashion...
November 5, 2016: Seminars in Arthritis and Rheumatism
https://www.readbyqxmd.com/read/28076913/use-of-intravenous-immunoglobulin-therapy-for-myositis-an-audit-in-south-australian-patients
#9
Caroline Foreman, Paul Russo, Noelene Davies, Pravin Hissaria, Susanna Proudman, Tiffany Hughes, Vidya Limaye
In South Australia, between 2000 and 2014, 57 patients with idiopathic inflammatory myositis (IIM) were treated with intravenous immunoglobulin (IVIg). We reviewed disease characteristics to determine predictors of response to therapy and IVIg dosing and duration to identify opportunities to rationalise IVIg use. Patients with dermatomyositis/polymyositis had a response rate of 77% and were more likely than inclusion body myositis to respond to therapy. Consideration should be given to the use of the lowest possible dose of IVIg and to the undertaking of trials of cessation of IVIg in patients with stable IIM...
January 2017: Internal Medicine Journal
https://www.readbyqxmd.com/read/28032847/molecular-markers-of-systemic-autoimmune-disorders-the-expression-of-mhc-located-hsp70-genes-is-significantly-associated-with-autoimmunity-development
#10
Martina Mišunová, Tana Svitálková, Lenka Pleštilová, Olga Kryštufková, Dana Tegzová, Radka Svobodová, Marketa Hušáková, Michal Tomčík, Radim Bečvář, Jakub Závada, Herman Mann, Libor Kolesár, Antonij Slavčev, Jiri Vencovský, Peter Novota
OBJECTIVES: To analyse the expression regulation of two inducible HSP70 genes - HSPA1A and HSPA1B - located within the major histocompatibility complex (MHC) in patients with various systemic autoimmune diseases and to prove the reliability of MHC-located HSP70 genes as molecular markers reflecting the autoimmune process. METHODS: 94 adult patients with idiopathic inflammatory myopathy (IIM, n=31), systemic lupus erythematosus (SLE, n=31) or systemic sclerosis (SSc, n=32) and 37 healthy individuals were analysed...
January 2017: Clinical and Experimental Rheumatology
https://www.readbyqxmd.com/read/27991411/one-year-in-review-2016-idiopathic-inflammatory-myopathies
#11
REVIEW
Martina Orlandi, Simone Barsotti, Elisa Cioffi, Sara Tenti, Carmela Toscano, Chiara Baldini, Rossella Neri
Idiopathic inflammatory myopathies (IIM) are a group of rare, acquired, clinically heterogeneous autoimmune inflammatory muscle disorders characterised by muscle weakness and multisystem involvement. Recently, new concepts about pathogenesis, diagnosis and treatment of these complex diseases have been provided. The purpose of this manuscript is to summarise the most relevant literature contributions published over the last year.
November 2016: Clinical and Experimental Rheumatology
https://www.readbyqxmd.com/read/27974098/use-of-biologic-agents-in-idiopathic-inflammatory-myopathies-in-sweden-a-descriptive-study-of-real-life-treatment
#12
John Svensson, Marie Holmqvist, Anna Tjärnlund, Maryam Dastmalchi, Balsam Hanna, Sara Magnusson Bucher, Ingrid E Lundberg
OBJECTIVES: Biologic treatment has revolutionised treatment in rheumatology in the last decades. Patients with idiopathic inflammatory myopathies (IIM) have so far only been treated with biologics off-label, with little published follow-up on those who are treated and how they are treated. We therefore set out to characterise the Swedish IIM patients who have been treated with biologics. METHODS: By linking Swedish registers we identified 95 patients with IIM who were treated with biologics between 2000 and 2011...
December 9, 2016: Clinical and Experimental Rheumatology
https://www.readbyqxmd.com/read/27936488/ro52-trim21-deficient-expression-and-function-in-different-subsets-of-peripheral-blood-mononuclear-cells-is-associated-with-a-proinflammatory-cytokine-response-in-patients-with-idiopathic-inflammatory-myopathies
#13
D Gómez-Martín, A S Galindo-Feria, A Barrera-Vargas, J Merayo-Chalico, G Juárez-Vega, J Torres-Ruiz, J Alcocer-Varela
The presence of anti-Ro52/tripartite motif 21 (Trim21) autoantibodies has been associated with a distinctive clinical profile and has gained value as a prognostic marker in idiopathic inflammatory myopathies (IIM). The aim of the present work was to analyse Ro52/Trim21 expression in different subsets of peripheral blood mononuclear cells (PBMCs) of patients with IIM, as well as the ubiquitination profile and its association with proinflammatory cytokine production. We included 18 patients with recent-onset IIM and 18 age- and gender-matched healthy donors...
April 2017: Clinical and Experimental Immunology
https://www.readbyqxmd.com/read/27908534/critical-review-of-the-role-of-intravenous-immunoglobulins-in-idiopathic-inflammatory-myopathies
#14
REVIEW
Sabrina Anh-Tu Hoa, Marie Hudson
OBJECTIVE: The aim of this review was to summarize key findings from the literature concerning the therapeutic role of intravenous immunoglobulins (IVIg) in idiopathic inflammatory myopathies (IIM), dissecting the evidence according to disease subtype and treatment indication, and to review the evidence relating to the mechanism of action of IVIg in IIM to ascertain rationale for continued research. METHODS: Medline (Ovid) and Pubmed databases were searched from inception to July 2016 using relevant keywords...
February 2017: Seminars in Arthritis and Rheumatism
https://www.readbyqxmd.com/read/27907058/choice-of-high-dose-intravenous-iron-preparation-determines-hypophosphatemia-risk
#15
Benedikt Schaefer, Philipp Würtinger, Armin Finkenstedt, Vickie Braithwaite, André Viveiros, Maria Effenberger, Irene Sulzbacher, Alexander Moschen, Andrea Griesmacher, Herbert Tilg, Wolfgang Vogel, Heinz Zoller
BACKGROUND: Ferric carboxymaltose (FCM) and iron isomaltoside 1000 (IIM) are increasingly used because they allow correction of severe iron deficiency in a single infusion. A transient decrease in serum phosphate concentrations is a frequent side effect of FCM. AIM: To characterize this adverse event and search for its predictors in a gastroenterology clinic patient cohort. METHODS: Electronic medical records of patients attending the University Hospital of Innsbruck were searched for the keywords ferric carboxymaltose or iron isomaltoside...
2016: PloS One
https://www.readbyqxmd.com/read/27878344/comparison-of-soluble-urokinase-plasminogen-activator-receptor-soluble-triggering-receptor-expressed-on-myeloid-cells-1-procalcitonin-and-c-reactive-protein-in-distinguishing-concurrent-bacterial-infection-from-idiopathic-inflammatory-myopathy
#16
Yizhi Xiao, Hui Luo, Bin Zhou, Xiaodan Dai, Jing Huang, Liping Duan, Yunhui You, Weiru Zhang, Hongjun Zhao, Yanli Xie, Yaou Zhou, Wangbin Ning, Tong Li, Sijia Liu, Honglin Zhu, Xiaoyun Xie, Ying Jiang, Shiyao Wu, Weijia He, Yisha Li
The aim of the study was to measure the diagnostic values of biomarkers of bacterial infection in idiopathic inflammatory myopathy (IIM) patients. The serum and clinical data of 82 IIM patients with/without bacterial infection were collected. Concentrations of soluble urokinase plasminogen activator receptor (suPAR), soluble triggering receptor expressed on myeloid cells 1 (sTREM-1), procalcitonin (PCT) and C-reactive protein (CRP) were measured in IIM patients and healthy controls. There were no significant differences in serum suPAR and sTREM-1 levels between healthy controls and non-infection IIM patients...
November 22, 2016: Rheumatology International
https://www.readbyqxmd.com/read/27752355/cardiac-involvement-in-adult-and-juvenile-idiopathic-inflammatory-myopathies
#17
REVIEW
Thomas Schwartz, Louise Pyndt Diederichsen, Ingrid E Lundberg, Ivar Sjaastad, Helga Sanner
Idiopathic inflammatory myopathies (IIM) include the main subgroups polymyositis (PM), dermatomyositis (DM), inclusion body myositis (IBM) and juvenile DM (JDM). The mentioned subgroups are characterised by inflammation of skeletal muscles leading to muscle weakness and other organs can also be affected as well. Even though clinically significant heart involvement is uncommon, heart disease is one of the major causes of death in IIM. Recent studies show an increased prevalence of traditional cardiovascular risk factors in JDM and DM/PM, which need attention...
2016: RMD Open
https://www.readbyqxmd.com/read/27684828/pulmonary-arterial-hypertension-in-idiopathic-inflammatory-myopathies-data-from-the-french-pulmonary-hypertension-registry-and-review-of-the-literature
#18
REVIEW
Sébastien Sanges, Cécile M Yelnik, Olivier Sitbon, Olivier Benveniste, Kuberaka Mariampillai, Mathilde Phillips-Houlbracq, Christophe Pison, Christophe Deligny, Jocelyn Inamo, Vincent Cottin, Luc Mouthon, David Launay, Marc Lambert, Pierre-Yves Hatron, Laurence Rottat, Marc Humbert, Eric Hachulla
Occurrence of pulmonary arterial hypertension (PAH) in idiopathic inflammatory myopathies (IIMs) without extensive interstitial lung disease (ILD) has rarely been described in the medical literature. This study aimed to report all cases with association of PAH and IIM in the French Pulmonary Hypertension (PH) Registry, to identify IIM features associated with the presence of PAH, and to describe treatment modalities of these patients.All cases of IIM-PAH were retrieved from the French PH Registry, which gathers PH patients prospectively enrolled by 27 referral hospital centers across France...
September 2016: Medicine (Baltimore)
https://www.readbyqxmd.com/read/27666811/dysregulated-innate-immune-function-in-the-aetiopathogenesis-of-idiopathic-inflammatory-myopathies
#19
REVIEW
Jessica Day, Sophia Otto, Susanna Proudman, John D Hayball, Vidya Limaye
The idiopathic inflammatory myopathies (IIMs) are a heterogeneous group of systemic muscle conditions that are believed to be autoimmune in nature. They have distinct pathological features, but the aetiopathogenesis of each subtype remains largely unknown. Recently, there has been increased interest in the complex role the innate immune system plays in initiating and perpetuating these conditions, and how this may differ between subtypes. This article summarises the traditional paradigms of IIM pathogenesis and reviews the accumulating evidence for disturbances in innate immune processes in these rare, but debilitating chronic conditions...
January 2017: Autoimmunity Reviews
https://www.readbyqxmd.com/read/27629259/curvilinear-bodies-are-associated-with-adverse-effects-on-muscle-function-but-not-with-hydroxychloroquine-dosing
#20
Thomas Khoo, Sophia Otto, Caroline Smith, Barbara Koszyca, Sue Lester, Peter Blumbergs, Vidya Limaye
The clinical significance of curvilinear bodies (CB) seen in association with hydroxychloroquine (HCQ) therapy is uncertain. Patients with CB on muscle biopsy performed between 2006 and the present were identified, and their clinical features including body mass index and cumulative HCQ dose were recorded. A control group of 16 patients with idiopathic inflammatory myositis (IIM) on HCQ at time of biopsy but without evidence of CB was identified. Nineteen patients with CB were identified; details were available for 18...
March 2017: Clinical Rheumatology
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