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Inflammatory polymyositis

Angela Ceribelli, Natasa Isailovic, Maria De Santis, Elena Generali, Micaela Fredi, Ilaria Cavazzana, Franco Franceschini, Luca Cantarini, Minoru Satoh, Carlo Selmi
This study aims to characterize myositis-specific antibodies in a well-defined cohort of patients with idiopathic inflammatory myopathy and to determine their association with cancer. Sera from 40 patients with polymyositis, dermatomyositis, and controls were tested by protein and RNA immunoprecipitation to detect autoantibodies, and immunoprecipitation-Western blot was used for anti-MJ/NXP-2, anti-MDA5, and anti-TIF1γ/α identification. Medical records were re-evaluated with specific focus on cancer. Anti-MJ/NXP-2 and anti-TIF1γ/α were the most common antibodies in dermatomyositis...
October 20, 2016: Clinical Rheumatology
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
Saleh Saleh Elessawy, Eman Muhammad Abdelsalam, Eman Abdel Razek, Samar Tharwat
BACKGROUND: Conventional magnetic resonance imaging (MRI) is a highly valuable tool for full assessment of the extent of bilateral symmetrical diffuse inflammatory myopathy, owing to its high sensitivity in the detection of edema which correlates with, and sometimes precedes, clinical findings. PURPOSE: To evaluate the use of whole-body (WB)-MRI in characterization and full assessment of the extent and distribution of diffuse inflammatory myopathy. MATERIAL AND METHODS: A prospective study on 15 patients presenting with clinical evidence of inflammatory myopathy...
September 2016: Acta Radiologica Open
Andrea Váncsa
Using current recommended treatment, a majority of patients with idiopathic inflammatory myopathy develop muscle impairment and poor health. Beneficial effects of exercise have been reported on muscle performance, aerobic capacity and health in chronic polymyositis and dermatomyositis, as well as in active disease and inclusion body myositis to some extent. Importantly, randomized controlled trials indicate that improved health and decreased clinical disease activity could be mediated through increased aerobic capacity...
September 2016: Orvosi Hetilap
Matthew B Carroll, Michelle R Newkirk, Nathan S Sumner
Necrotizing autoimmune myopathy (NAM) is a recently recognized entity within the spectrum of idiopathic inflammatory myopathies. Diagnosis critically rests on histopathologic demonstration of macrophage predominant myocyte destruction, with few to no lymphocytes. We report our experience with identifying and treating this subset of inflammatory myositis, highlighting the importance of muscle biopsy in diagnosis, association with statin use and malignancy, and challenges of therapy.We present 3 cases that presented to 2 hospitals within our academic system in calendar year 2014 with acute/subacute onset of profound proximal muscle weakness and markedly elevated creatine kinase levels...
October 2016: Journal of Clinical Rheumatology: Practical Reports on Rheumatic & Musculoskeletal Diseases
Iago Pinal-Fernandez, Maria Casal-Dominguez, John A Carrino, Arash H Lahouti, Pari Basharat, Jemima Albayda, Julie J Paik, Shivani Ahlawat, Sonye K Danoff, Thomas E Lloyd, Andrew L Mammen, Lisa Christopher-Stine
OBJECTIVES: The aims of this study were to define the pattern of muscle involvement in patients with immune-mediated necrotising myopathy (IMNM) relative to those with other inflammatory myopathies and to compare patients with IMNM with different autoantibodies. METHODS: All Johns Hopkins Myositis Longitudinal Cohort subjects with a thigh MRI (tMRI) who fulfilled criteria for IMNM, dermatomyositis (DM), polymyositis (PM), inclusion body myositis (IBM) or clinically amyopathic DM (CADM) were included in the study...
September 20, 2016: Annals of the Rheumatic Diseases
Aarat Patel, Georgia Seely, Rohit Aggarwal
Idiopathic inflammatory myopathies are a group of systemic autoimmune diseases that involve inflammation of skeletal muscle. The two most common forms are dermatomyositis and polymyositis, the former of which entails a skin component. There are few approved therapeutics available for treatment of this group of diseases and the first-line therapy is usually corticosteroid treatment. Considering that a large proportion of patients do not respond to or cannot tolerate corticosteroids, additional treatments are required...
2016: Case Reports in Rheumatology
Tomoko Noda, Masahiro Iijima, Seiya Noda, Shinya Maeshima, Hirotaka Nakanishi, Seigo Kimura, Haruki Koike, Shinsuke Ishigaki, Yohei Iguchi, Masahisa Katsuno, Gen Sobue
Objective An association has been reported between inflammatory myopathies (IMs), which include polymyositis (PM) and dermatomyositis (DM), and malignancy, and the concept of cancer-associated myositis (CAM) was recently proposed. We herein attempted to determine the features and etiologies of these myopathies. Methods We analyzed the gene expression levels via microarray and real-time quantitative reverse transcription polymerase chain reaction analyses to identify genes that were specifically upregulated or downregulated with suspected inflammatory involvement and verified the microarray data via an immunohistochemical (IHC) analysis in additional cases...
2016: Internal Medicine
Leah J Witt, James J Curran, Mary E Strek
Anti-synthetase syndrome is an autoimmune condition, characterized by antibodies directed against an aminoacycl transfer RNA synthetase along with clinical features that can include interstitial lung disease, myositis, Raynaud's phenomenon, and arthritis. There is a higher prevalence and increased severity of interstitial lung disease in patients with anti-synthetase syndrome, as compared to dermatomyositis and polymyositis, inflammatory myopathies with which it may overlap phenotypically. Diagnosis is made by a multidisciplinary approach, synthesizing rheumatology and pulmonary evaluations, along with serologic, radiographic, and occasionally muscle and/or lung biopsy results...
September 2016: Clinical Pulmonary Medicine
Hina N Khan, Usman Jilani, Shitij Arora
Polymyositis is a rare disease with incidence rates at about 1 per 100,000 people annually. In this case report we will review a case of proximal muscle weakness with an elevated creatine phosphokinase that was initially misdiagnosed twice as rhabdomyolysis. Therefore, emphasizing that idiopathic inflammatory myopathy is a potential cause of myasthenia that must be considered in the differential. The case will also describe the current treatment and treatment response in polymyositis.
2016: F1000Research
Shiro Koh, Hideo Koh, Yasuhiro Nakashima, Takako Katayama, Manami Sakabe, Hiroshi Okamura, Takuro Yoshimura, Satoru Nanno, Mitsutaka Nishimoto, Yoshiki Hayashi, Takahiko Nakane, Hirohisa Nakamae, Masahiko Ohsawa, Masayuki Hino
We herein describe a case of myelodysplastic syndrome with chronic graft-versus-host disease (cGVHD)-related polymyositis. On approximately day 1,570 post HLA-identical sibling bone marrow transplant, the patient presented with a fever, myalgia and liver dysfunction. A muscle biopsy revealed destruction of the muscle fibers and infiltration of CD20(+) B cells and CD4(+) and CD8(+) T cells, and a liver biopsy confirmed the findings of cGVHD. An analysis of plasma cytokine profiles indicated elevation of not only T-helper (Th)1 and Th2, but also Th17 cytokines...
2016: Internal Medicine
Liguo Yin, Yongpeng Ge, Hanbo Yang, Qinglin Peng, Xin Lu, Yamei Zhang, Guochun Wang
The objectives of this study are to assess the levels of serum Interleukin-35 (IL-35) in patients with idiopathic inflammatory myopathies (IIMs) and to evaluate the association between IL-35 levels and IIM-related features. Serum IL-35 was detected in 76 patients with dermatomyositis (DM), 28 patients with polymyositis (PM), 98 disease controls (40 rheumatoid arthritis (RA), 34 systemic lupus erythematosus (SLE), 12 systemic sclerosis (SSc), and 12 sjogren syndrome (SS)), and 43 healthy controls by ELISA. Follow-up was conducted on 34 patients...
November 2016: Clinical Rheumatology
Marta Amaral Silva, Estefania Cogollo, David A Isenberg
OBJECTIVES: Causes of death in inflammatory myopathies have rarely been studied. We have assessed a cohort of myositis patients followed in a single centre over a 37-year period, reviewing the mortality rate, causes of death and predictors of poor prognosis. METHODS: We performed a single-centre, retrospective study on patients aged ≥16 years fulfilling 3 or 4 of the Bohan and Peter criteria, noting their demographic data, clinical features, serology, treatment and outcome...
September 2016: Clinical and Experimental Rheumatology
Zhibo Chen, Zhongqian Su, Wanhui Pang, Yuanyuan Huang, Jie Lin, Zhangna Ding, Senmin Wu, Shunyao Xu, Weiwei Quan, Juzeng Zheng, Huale Chen, Zhengzheng Li, Xiang Li, Jia Li, Yiyun Weng, Xu Zhang
OBJECTIVE: Oxidative stress and variations in antioxidant status are implicated in the pathogenesis of inflammatory and autoimmune diseases. Polymyositis and dermatomyositis (PM/DM) are autoimmune diseases with inflammatory cell infiltrating into skeletal muscles, and the antioxidant status is still controversial. The aim of our study was to investigate the correlation between PM/DM and the antioxidant status of serum bilirubin (Tbil, Dbil and Ibil) and uric acid (UA). MATERIALS AND METHODS: We measured serum concentrations of bilirubin (Tbil, Dbil and Ibil) and uric acid in 384 individuals, including 110 PM/DM patients and 274 healthy controls...
August 3, 2016: International Journal of Neuroscience
Simon Rothwell, Janine A Lamb, Hector Chinoy
PURPOSE OF REVIEW: This article reviews the advances that have been made in our understanding of the genetics of the idiopathic inflammatory myopathies (IIM) in the past 2 years, with a particular focus on polymyositis, dermatomyositis and inclusion body myositis. RECENT FINDINGS: Two large human leukocyte antigen (HLA) imputation studies have confirmed a strong association with the 8.1 ancestral haplotype in clinical subgroups of myositis and suggest multiple independent associations on this haplotype...
November 2016: Current Opinion in Rheumatology
Jung Min Bae, Ji Yoon Choo, Ki-Jo Kim, Kyung-Su Park
OBJECTIVES: Tantalizing connections between autoimmune rheumatic diseases (ARDs) and inflammatory bowel disease (IBD) have become evident with regard to their genetic and immunologic background. However, the association between these two disease entities remains unclear. The aim of this study is to investigate the association between each ARD and IBD. METHODS: A nationwide population-based cross-sectional study was performed using the Korean National Health Insurance Claims database...
July 26, 2016: Modern Rheumatology
Andrea Váncsa, Katalin Dankó
Myositis, which means inflammation of the muscles, is a general term used for inflammatory myopathies. Myositis is a rare idiopathic autoimmune disease. It is believed that environmental factors such as virus, bacteria, parasites, direct injuries, drugs side effect can trigger the immune system of genetically susceptible individuals to act against muscle tissues. There are several types of myositis with the same systemic symptoms such as muscle weakness, fatigue, muscle pain and inflammation. These include dermatomyositis, juvenile dermatomyositis, inclusion-body myositis, polymyositis, orbital myositis and myositis ossificans...
July 2016: Orvosi Hetilap
Kira V Blankenbach, Stephanie Schwalm, Josef Pfeilschifter, Dagmar Meyer Zu Heringdorf
The sphingosine-1-phosphate (S1P) signaling system with its specific G-protein-coupled S1P receptors, the enzymes of S1P metabolism and the S1P transporters, offers a multitude of promising targets for drug development. Until today, drug development in this area has nearly exclusively focused on (functional) antagonists at the S1P1 receptor, which cause a unique phenotype of immunomodulation. Accordingly, the first-in class S1P1 receptor modulator, fingolimod, has been approved for the treatment of relapsing-remitting multiple sclerosis, and novel S1P1 receptor (functional) antagonists are being developed for autoimmune and inflammatory diseases such as psoriasis, inflammatory bowel disease, lupus erythematodes, or polymyositis...
2016: Frontiers in Pharmacology
Gurveen Malhotra, Nitya Ramreddy, Serafin Chua, Mira Iliescu, Tanjeev Kaur
Antisynthetase (AS) syndrome is a major subgroup of inflammatory myopathies seen in a minority of patients with dermatomyositis and polymyositis. Although it is usually associated with elevated creatine phosphokinase level, some patients may have amyopathic dermatomyositis (ADM) like presentation with predominant skin involvement. Interstitial lung disease (ILD) is the main pulmonary manifestation and may be severe thereby determining the prognosis. It may rarely present with a very aggressive course resulting in acute respiratory distress syndrome (ARDS)...
2016: Case Reports in Critical Care
Seo-Hyun Kim, I-Nae Park
Antisynthetase syndrome has been recognized as an important cause of autoimmune inflammatory myopathy in a subset of patients with polymyositis and dermatomyositis. It is associated with serum antibody to aminoacyl-transfer RNA synthetases and is characterized by a constellation of manifestations, including fever, myositis, interstitial lung disease, mechanic's hand-like cutaneous involvement, Raynaud phenomenon, and polyarthritis. Lung disease is the presenting feature in 50% of the cases. We report a case of a 60-year-old female with acute respiratory distress syndrome (ARDS), which later proved to be an unexpected and initial manifestation of anti-Jo-1 antibody-positive antisynthetase syndrome...
July 2016: Tuberculosis and Respiratory Diseases
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