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    Scleroderma Renal Crisis in Mixed Connective Tissue Disease With Full Renal Recovery Within 3 Months: A Case Report With Expanding Treatment Modalities to Treat Each Clinical Sign as an Independent Entity.
    J Investig Med High Impact Case Rep 2017 Oct-Dec;5(4):2324709617734012. Epub 2017 Oct 10.
    Eastern Virginia Medical School, Norfolk, VA, USA.
    Mixed connective tissue disease (MCTD) is a rheumatologic overlap syndrome that can present with symptoms of systemic lupus erythematous, scleroderma, and polymyositis. A severe but rare complication that can occur in MCTD is scleroderma renal crisis. With multiple poor prognostic indicators, the renal outcome is usually poor. Read More

    [Lung transplantation for connective tissue disease-associated interstitial lung disease recipient].
    Zhonghua Jie He He Hu Xi Za Zhi 2017 Oct;40(10):744-748
    Transplantation Center of Wuxi People's Hospital Affiliated to Nanjing Medical University, Wuxi 214023, China.
    Objective: To investigate the timing and complications of lung transplantation for the treatment of connective tissue disease-associated interstitial lung disease (CTD-ILD). Method: The clinical data of connective tissue-associated disease lung transplantation recipients from September, 2015 to February, 2017 were collected. Results: 11 patients with CTD-ILD were evaluated by lung transplantation and were treated with lung transplantation. Read More

    Less Limb Muscle Involvement in Myositis Patients with Anti-Mitochondrial Antibodies.
    Eur Neurol 2017 Oct 19;78(5-6):290-295. Epub 2017 Oct 19.
    Recent studies have revealed the clinical, histological, and pathophysiological characteristics in a group of inflammatory myopathies with selected autoantibodies. We retrospectively compared the clinical manifestations and histological features between 8 anti-mitochondrial (anti-M2) antibody-positive and 33 antibody-negative patients. Patients with anti-M2 antibodies have been previously reported to have delayed diagnostic confirmation and frequent cardiopulmonary complications in comparison to those without the antibodies. Read More

    Lipid-lowering agent-triggered dermatomyositis and polymyositis: a case series and literature review.
    Rheumatol Int 2017 Oct 12. Epub 2017 Oct 12.
    Division of Rheumatology, Faculdade de Medicina FMUSP, Universidade de Sao Paulo, Sao Paulo, SP, Brazil.
    Lipid-lowering agent-triggered dermatomyositis (DM) or polymyositis (PM) is a rare event. Therefore, the aim of the present study was to describe a series of such cases. A retrospective cohort study of 5 DM and 4 PM cases triggered by prior exposure to lipid-lowering agents between 2001 and 2017 was carried out. Read More

    Cardiopulmonary Manifestations of Collagen Vascular Diseases.
    Curr Rheumatol Rep 2017 Oct 9;19(11):71. Epub 2017 Oct 9.
    Division of Diagnostic Radiology, Cardiothoracic Imaging Section, Mallinckrodt Institute of Radiology, Washington University in St. Louis, Campus Box 8131, 510 S Kingshighway Blvd, St. Louis, MO, USA.
    Purpose Of Review: The study aimed to illustrate the cardiopulmonary findings of the following collagen vascular diseases on cross-sectional imaging: rheumatoid arthritis, scleroderma (progressive systemic sclerosis), systemic lupus erythematosus, the inflammatory myopathies (polymyositis/dermatomyositis), and Sjögren's syndrome.

    Recent Findings: Although collagen vascular diseases can affect any part of the body, interstitial lung disease and pulmonary hypertension are the two most important cardiopulmonary complications and are responsible for the majority of morbidity and mortality in this patient population. Interstitial pneumonia with autoimmune features (IPAF) is a newly described entity that encompasses interstitial lung disease in patients with clinical, serologic, or morphologic features suggestive of but not diagnostic of collagen vascular disease; these patients are thought to have better outcomes than idiopathic interstitial pneumonias. Read More

    Abatacept in the treatment of adult dermatomyositis and polymyositis: a randomised, phase IIb treatment delayed-start trial.
    Ann Rheum Dis 2017 Oct 9. Epub 2017 Oct 9.
    Rheumatology Unit, Department of Medicine, Karolinska University Hospital Solna, Karolinska Institutet, Stockholm, Sweden.
    Objectives: To study the effects of abatacept on disease activity and on muscle biopsy features of adult patients with dermatomyositis (DM) or polymyositis (PM).

    Methods: Twenty patients with DM (n=9) or PM (n=11) with refractory disease were enrolled in a randomised treatment delayed-start trial to receive either immediate active treatment with intravenous abatacept or a 3 month delayed-start. The primary endpoint was number of responders, defined by the International Myositis Assessment and Clinical Studies Group definition of improvement (DOI), after 6 months of treatment. Read More

    2016 ACR-EULAR adult dermatomyositis and polymyositis and juvenile dermatomyositis response criteria-methodological aspects.
    Rheumatology (Oxford) 2017 Aug 2. Epub 2017 Aug 2.
    Department of Medicine, Division of Rheumatology and Clinical Immunology, University of Pittsburgh, Pittsburgh, PA, USA.
    Objective: The objective was to describe the methodology used to develop new response criteria for adult DM/PM and JDM.

    Methods: Patient profiles from prospective natural history data and clinical trials were rated by myositis specialists to develop consensus gold-standard ratings of minimal, moderate and major improvement. Experts completed a survey regarding clinically meaningful improvement in the core set measures (CSM) and a conjoint-analysis survey (using 1000Minds software) to derive relative weights of CSM and candidate definitions. Read More

    Risk factors for the recurrence of interstitial lung disease in patients with polymyositis and dermatomyositis: a retrospective cohort study.
    Clin Rheumatol 2017 Oct 3. Epub 2017 Oct 3.
    Division of Rheumatology, Department of Internal Medicine, Keio University School of Medicine, 35 Shinanomachi, Shinjuku-ku, Tokyo, Japan.
    To identify risk factors for the recurrence of interstitial lung disease (ILD) in patients with polymyositis (PM)/dermatomyositis (DM). Forty-four PM/DM-ILD patients who had been treated with glucocorticoid and/or immunosuppressive agents as a remission induction therapy were enrolled. The patients were first classified into two groups: the early recurrence group that recurred within 52 weeks, and the non-early recurrence group, which was further classified into the late recurrence group that recurred after 52 weeks, and the non-recurrence group. Read More

    Comment on the article titled "Increased Incidence of Amyotrophic Lateral Sclerosis in Polymyositis: A Nationwide Cohort Study".
    Arthritis Care Res (Hoboken) 2017 Oct 3. Epub 2017 Oct 3.
    Department of Internal Medicine, Division of Rheumatology, Maricopa Medical Center and University of Arizona, College of Medicine, Phoenix.
    With interest, I read the recent article in Arthritis Care and Research titled "Increased Incidence of Amyotrophic Lateral Sclerosis in Polymyositis: A Nationwide Cohort Study" (1). Tseng at al (1) conducted a retrospective cohort study in Taiwan, exploring a link between amyotrophic lateral sclerosis (ALS) and polymyositis (PM). This article is protected by copyright. Read More

    Reply.
    Arthritis Care Res (Hoboken) 2017 Oct 3. Epub 2017 Oct 3.
    Division of Rheumatology, Department of Internal Medicine, Kaohsiung Medical University Hospital, Kaohsiung, Taiwan.
    We appreciate the comments from Dr Parperis. The question raised by Parperis was the possibility of amyotrophic lateral sclerosis (ALS) misdiagnosed as polymyositis (PM) initially. Dr Parperis cited the study of Harrington et al to support his claims (1). Read More

    Successful treatment using rituximab in a patient with refractory polymyositis complicated by scleroderma renal crisis.
    BMJ Case Rep 2017 Sep 23;2017. Epub 2017 Sep 23.
    Department of Rheumatology, Kawasaki Medical School, Kurashiki, Japan.
    Corticosteroids are the first-line treatment for patients with inflammatory myopathies. Myositis can be a clinical feature of scleroderma (polymyositis-scleroderma overlap syndrome), and treatment of this syndrome is a challenge for clinicians because moderate to high doses of corticosteroids are considered a risk factor for development of acute kidney injury in affected patients. We report here the case of a 56-year-old woman with scleroderma who developed polymyositis and was successfully treated with rituximab. Read More

    Paradoxical development of polymyositis-like autoimmunity through augmented expression of autoimmune regulator (AIRE).
    J Autoimmun 2017 Sep 17. Epub 2017 Sep 17.
    Division of Molecular Immunology, Institute for Enzyme Research, Tokushima University, Tokushima 770-8503, Japan; AMED-CREST, Japan Agency for Medical Research and Development, Tokyo 100-0004, Japan. Electronic address:
    Autoimmunity is prevented by the function of the autoimmune regulator [AIRE (Aire in mice)], which promotes the expression of a wide variety of tissue-restricted antigens (TRAs) from medullary thymic epithelial cells (mTECs) and from a subset of peripheral antigen-presenting cells (APCs). We examined the effect of additive expression of human AIRE (huAIRE) in a model of autoimmune diabetes in NOD mice. Unexpectedly, we observed that mice expressing augmented AIRE/Aire developed muscle-specific autoimmunity associated with incomplete maturation of mTECs together with impaired expression of Aire-dependent TRAs. Read More

    Plasmapheresis in neurological disorders: six years experience from University Clinical center Tuzla.
    F1000Res 2017 26;6:1234. Epub 2017 Jul 26.
    Division of Nephrology and Dialysis, Department of Internal Diseases, University Clinical Centre Tuzla, University of Tuzla, Tuzla, Bosnia and Herzegovina.
    Background: Therapeutic plasma exchange (TPE) is an extracorporeal blood purification technique that is designed to remove substances with a large molecular weight. The TPE procedure includes removal of antibodies, alloantibodies, immune complexes, monoclonal protein, toxins or cytokines, and involves the replenishment of a specific plasma factor. The aim of the study was to describe the clinical response to TPE in various neurological patients, and to assess the clinical response to this therapy. Read More

    Extensive Dystrophic Calcinosis Cutis: A Severe Complication of Polymyositis/Systemic Sclerosis Overlap Syndrome.
    J Clin Rheumatol 2017 Sep 19. Epub 2017 Sep 19.
    From the *Department of Medicine, Cairns Hospital; and †College of Medicine and Dentistry, James Cook University Cairns Campus, Cairns; ‡The Kirby Institute, University of New South Wales, Sydney; and §Menzies School of Health Research, Darwin, Australia.

    Inflammatory myopathy associated with antimitochondrial antibodies: A distinct phenotype with cardiac involvement.
    Semin Arthritis Rheum 2017 Jun 13. Epub 2017 Jun 13.
    Department of Medicine, Division of Rheumatology, Johns Hopkins University, School of Medicine, Baltimore, MD. Electronic address:
    Objective: In the context of clinical evaluations performed on our prospective myositis cohort, we noted a striking association of severe cardiac disease in myositis patients with antimitochondrial antibodies. We sought to review all cases of antimitochondrial antibody (AMA) associated myositis in our cohort to describe the clinical features of this disease subset.

    Methods: We identified 7 patients with confirmed antimitochondrial antibodies who presented as an inflammatory myopathy. Read More

    Pulmonary arterial hypertension, a novelty in idiopathic inflammatory myopathies: insights and first experiences with vasoactive therapy.
    RMD Open 2017 9;3(1):e000331. Epub 2017 Jun 9.
    Department of Rheumatology, Radboud University Medical Center, Nijmegen, The Netherlands.
    To characterise the different types of pulmonary hypertension (PH) among idiopathic inflammatory myopathy (IIM). A retrospective case series with assessment of PH by right heart catheterisation, extent of interstitial lung disease (ILD) and outcome of vasoactive therapy.The group of patients with IIM with PH (n=9) showed a median age at PH diagnosis of 62 years (IQR 48-71 years; eight women), seven diagnosed with polymyositis and two with dermatomyositis; median disease duration of 5. Read More

    [Manifestations of the connective tissue associated interstitial lung disease under high resolution computed tomography].
    Zhong Nan Da Xue Xue Bao Yi Xue Ban 2017 Aug;42(8):934-939
    Department of Radiology, Xiangya Hospital, Central South University, Changsha 410008, China.
    Objective: To analyze the features of the connective tissue associated interstitial lung disease (CTD-ILD) by high resolution computed tomography (HRCT).
 Methods: A total of 127 patients with CTD-ILD, who were diagnosed by clinic laboratory examination and pathology in Xiangya Hospital of Central South University form September 2013 to September 2015, were enrolled for this study. Their lung features of HRCT imaging were retrospectively analyzed. Read More

    Altered chemokine receptor expression in the peripheral blood lymphocytes in polymyositis and dermatomyositis.
    Cytokine 2017 Aug 28. Epub 2017 Aug 28.
    Department of Rheumatology and Immunology, First Affiliated Hospital, China Medical University, Shenyang 110001, People's Republic of China. Electronic address:
    Objective: To examine the expression of chemokine receptors in different peripheral blood T-cell subsets in patients with polymyositis (PM) and dermatomyositis (DM).

    Methods: We used flow cytometry to measure the frequencies of chemokinereceptors CXCR3 and CCR4 expression in the CD4(+) or CD8(+) lymphocytes. Enzyme linked immunosorbent assays were also used to measure the concentrations of C-X-C motif chemokine 10 (CXCL10), thymus and activation regulated chemokine (TARC) and macrophage derived chemokine (MDC). Read More

    Mortality and prognostic factors in idiopathic inflammatory myositis: a retrospective analysis of a large multicenter cohort of Spain.
    Rheumatol Int 2017 Nov 2;37(11):1853-1861. Epub 2017 Sep 2.
    Servicio de Reumatología, Hospital General Universitario Gregorio Marañón, Madrid, Spain.
    The present study was undertaken to assess mortality, causes of death, and associated prognostic factors in a large cohort of patients diagnosed with idiopathic inflammatory myositis (IIM) from Spain. A retrospective longitudinal study was carried out in 467 consecutive patients with IIM, identified from 12 medical centers. Patients were classified as primary polymyositis, primary dermatomyositis (DM), overlap myositis, cancer-associated myositis (CAM), and juvenile idiopathic inflammatory myopathies. Read More

    Myositis-associated Interstitial Lung Disease: Predictors of Failure of Conventional Treatment and Response to Tacrolimus in a US Cohort.
    J Rheumatol 2017 Sep 1. Epub 2017 Sep 1.
    From the Department of Clinical Immunology and Rheumatology, University of Alabama at Birmingham, Birmingham, Alabama; Department of Internal Medicine, and Section of Pulmonary and Critical Care Medicine, and Section of Rheumatology, University of Chicago, Chicago, Illinois, USA. Dr. Dua serves on the advisory board for Genentech and Janssen. Dr. Strek has served as the PI for her institution for studies of therapy for idiopathic pulmonary fibrosis with Gilead, InterMune, and MedImmune. She has served as a member of the Data Safety Committee for Boehringer- Ingelheim. Dr. Vij has received grant funding from Genentech for the study of interstitial lung diseases. N. Sharma, MD, Assistant Professor of Medicine, Department of Clinical Immunology and Rheumatology, University of Alabama at Birmingham; M.S. Putman, MD, Internal Medicine Resident, Department of Internal Medicine, University of Chicago; R. Vij, MD, Clinical Associate of Medicine, Section of Pulmonary and Critical Care Medicine, University of Chicago; M.E. Strek, MD, Professor of Medicine, Section of Pulmonary and Critical Care Medicine, University of Chicago; A. Dua, MD, MPH, Assistant Professor of Medicine, Rheumatology Fellowship Program Director, Section of Rheumatology, University of Chicago. Address correspondence to Dr. N. Sharma, Department of Clinical Immunology and Rheumatology, University of Alabama at Birmingham, 1201 11th Ave. S, Suite 510, Birmingham, Alabama 35205-3410, USA. E-mail: Accepted for publication June 15, 2017.
    Objective: Patients with myositis-associated interstitial lung disease (MA-ILD) are often refractory to conventional treatment, and predicting their response to therapy is challenging. Recent case reports and small series suggest that tacrolimus may be useful in refractory cases.

    Methods: A retrospective cohort study of patients with MA-ILD comparing clinical characteristics between those who responded to or failed conventional treatment. Read More

    Facts and controversies in mixed connective tissue disease.
    Med Clin (Barc) 2017 Aug 29. Epub 2017 Aug 29.
    Servicio de Reumatología, Hospital General Universitario Gregorio Marañón, Madrid, España; Instituto de Investigación Biomédica Hospital Gregorio Marañón, Madrid, España; Universidad Complutense de Madrid, Madrid, España.
    Mixed connective tissue disease (MCTD) is a systemic autoimmune rheumatic disease (SARD) characterised by the combination of clinical manifestations of systemic lupus erythematosus (SLE), cutaneous systemic sclerosis (SSc) and polymyositis-dermatomyositis, in the presence of elevated titers of anti-U1-RNP antibodies. Main symptoms of the disease are polyarthritis, hand oedema, Raynaud's phenomenon, sclerodactyly, myositis and oesophageal hypomobility. Although widely discussed, most authors today accept MCTD as an independent entity. Read More

    Infections and respiratory tract disease as risk factors for idiopathic inflammatory myopathies: a population-based case-control study.
    Ann Rheum Dis 2017 Nov 30;76(11):1803-1808. Epub 2017 Aug 30.
    Clinical Epidemiology Unit, Department of Medicine Solna, Karolinska Institutet, Stockholm, Sweden.
    Objectives: To investigate the association between infection or respiratory tract disease and future risk of developing idiopathic inflammatory myopathy (IIM).

    Methods: A case-control study was performed using Swedish nationwide registers. Adults with newly diagnosed IIM were identified (2002-2011) from the National Patient Register (NPR) and the Swedish Rheumatology Register (n=957). Read More

    Automated diagnosis of myositis from muscle ultrasound: Exploring the use of machine learning and deep learning methods.
    PLoS One 2017 30;12(8):e0184059. Epub 2017 Aug 30.
    Division of Rheumatology, Johns Hopkins School of Medicine, Baltimore, Maryland, United States of America.
    Objective: To evaluate the use of ultrasound coupled with machine learning (ML) and deep learning (DL) techniques for automated or semi-automated classification of myositis.

    Methods: Eighty subjects comprised of 19 with inclusion body myositis (IBM), 14 with polymyositis (PM), 14 with dermatomyositis (DM), and 33 normal (N) subjects were included in this study, where 3214 muscle ultrasound images of 7 muscles (observed bilaterally) were acquired. We considered three problems of classification including (A) normal vs. Read More

    Muscle ultrasound: a potential tool for detecting of fasciitis among dermatomyositis and polymyositis: Comment on article by Yoshida et al.
    Arthritis Rheumatol 2017 Aug 29. Epub 2017 Aug 29.
    Department of Rheumatology, Radboud University Medical Center, Nijmegen.
    We read with interest the article by Yoshida and colleagues on the ability of muscle ultrasound for detecting of fasciitis among patients with dermatomyositis (DM) in the early phase of their disease (1). The authors used power Doppler ultrasonography to assess inflammation and vascularity of the fascia of different muscles. This article is protected by copyright. Read More

    GLIS3 and TYK2 Single Nucleotide Polymorphisms Are Not Associated with Dermatomyositis/Polymyositis in Chinese Han Population.
    Genet Test Mol Biomarkers 2017 Sep 28;21(9):565-570. Epub 2017 Aug 28.
    1 Department of Rheumatology and Clinical Immunology, Peking Union Medical College Hospital , Chinese Academy of Medical Sciences & Peking Union Medical College, Key Laboratory of Rheumatology and Clinical Immunology, Ministry of Education, Beijing, China .
    Aim: Racial differences and genetic overlap have been shown to be responsible for the difference in susceptibility to dermatomyositis (DM)/polymyositis (PM) in a variety of populations. Single nucleotide polymorphisms (SNPs) in the GLI-similar 3 (GLIS3) and tyrosine kinase 2 (TYK2) genes have been associated with various autoimmune diseases. The aim of this study was to investigate whether SNPs in GLIS3 (rs7020673, rs10758593, and rs10814916) and TYK2 (rs280519, rs2304256, rs17000730, and rs280501) were associated with an increase in susceptibility to DM/PM in a Chinese Han population. Read More

    Neutrophil extracellular traps may contribute to interstitial lung disease associated with anti-MDA5 autoantibody positive dermatomyositis.
    Clin Rheumatol 2017 Aug 25. Epub 2017 Aug 25.
    Department of Rheumatology, West China Hospital, Sichuan University, No.37 Guoxue Lane, Chengdu, 610041, China.
    In dermatomyositis (DM), anti-melanoma differentiation-associated gene 5 (MDA5) autoantibody (autoAb) marks a subtype with low grade or absent muscle inflammation but frequent and rapidly progressive interstitial lung disease (ILD). The pathogenesis of ILD remains poorly unknown. The aim of the study is to explore whether neutrophil extracellular traps (NETs) are involved in the development of ILD in DM patients with anti-MDA5 autoAb. Read More

    Cardiovascular involvement in myositis.
    Curr Opin Rheumatol 2017 Nov;29(6):598-603
    Department of Rheumatology, Odense University Hospital, Odense, Denmark.
    Purpose Of Review: The purpose of this review is to provide an update on cardiovascular involvement in idiopathic inflammatory myopathy (IIM). Studies from the past 18 months are identified and reviewed. Finally, the clinical impact of these findings is discussed. Read More

    Upregulation of Interleukin 21 and Interleukin 21 Receptor in Patients with Dermatomyositis and Polymyositis.
    Chin Med J (Engl) 2017 Sep;130(17):2101-2106
    Research Institute of Neuromuscular and Neurodegenerative Diseases, Department of Neurology, Qilu Hospital, Shandong University; Brain Science Research Institute, Shandong University, Jinan, Shandong 250012, China.
    Background: The immunopathologic mechanism underlying dermatomyositis (DM) and polymyositis (PM) remains poorly understood. Many cytokines play a pathogenic role in DM and PM. Interleukin 21 (IL-21) has a pleiotropic effect on inflammation regulation. Read More

    Which nonautoimmune myopathies are most frequently misdiagnosed as myositis?
    Curr Opin Rheumatol 2017 Nov;29(6):618-622
    Muscle Disease Unit, Laboratory of Muscle Stem Cells and Gene Expression, National Institute of Arthritis and Musculoskeletal and Skin Diseases, National Institutes of Health, Bethesda, Maryland, USA.
    Purpose Of Review: To discuss the spectrum of nonautoimmune myopathies that may be misdiagnosed as autoimmune myopathy.

    Recent Findings: Inherited myopathies, such as dysferlinopathy, calpainopathy, and facioscapulohumeral dystrophy may be misdiagnosed as autoimmune myopathy, especially when they have inflammatory muscle biopsies. Inclusion body myositis is frequently misdiagnosed as polymyositis when rimmed vacuoles are absent on muscle biopsy, and a careful neuromuscular evaluation is not performed. Read More

    Multiple values of (18)F-FDG PET/CT in idiopathic inflammatory myopathy.
    Clin Rheumatol 2017 Aug 22. Epub 2017 Aug 22.
    Department of Nuclear Medicine, Peking University People's Hospital, 11th Xizhimen South St., Beijing, 100044, China.
    This study aimed to investigate the multiple values of (18)F-FDG PET/CT in detecting malignant tumors, evaluating myopathy, and determining interstitial lung disease in patients with idiopathic inflammatory myopathy (IIM). We retrospectively analyzed the data of 38 patients who were examined by (18)F-FDG PET/CT and eventually diagnosed as IIM. We also collected the data of another 22 cases with negative PET/CT as the control. Read More

    Effect of endurance exercise on microRNAs in myositis skeletal muscle-A randomized controlled study.
    PLoS One 2017 22;12(8):e0183292. Epub 2017 Aug 22.
    Research Center for Genetic Medicine, Children's National Health System, Washington, D.C., United States of America.
    Objective: To identify changes in skeletal muscle microRNA expression after endurance exercise and associate the identified microRNAs with mRNA and protein expression to disease-specific pathways in polymyositis (PM) and dermatomyositis (DM) patients.

    Methods: Following a parallel clinical trial design, patients with probable PM or DM, exercising less than once a week, and on stable medication for at least one month were randomized into two groups at Karolinska University Hospital: a 12-week endurance exercise group (n = 12) or a non-exercised control group (n = 11). Using an Affymetrix microarray, microRNA expression was determined in paired muscle biopsies taken before and after the exercise intervention from 3 patients in each group. Read More

    A review of inflammatory idiopathic myopathy focusing on polymyositis.
    Eur J Neurol 2017 Aug 17. Epub 2017 Aug 17.
    Department of Rheumatology, University College London Hospitals, London, UK.
    Inflammatory idiopathic myopathies are a group of autoimmune diseases affecting predominantly the proximal skeletal muscles, with raised muscle enzymes, with or without skin involvement and extramuscular organ involvement. Autoantibodies help to characterize patients into different clinical phenotypes. Successful treatment necessitates controlling inflammation early with corticosteroids and invariably requires additional immunosuppressive therapy. Read More

    Mortality in idiopathic inflammatory myopathy: results from a Swedish nationwide population-based cohort study.
    Ann Rheum Dis 2017 Aug 16. Epub 2017 Aug 16.
    Rheumatology Unit, Department of Medicine, Karolinska Institutet, Karolinska University Hospital, Stockholm, Sweden.
    Patients with idiopathic inflammatory myopathies (IIMs) suffer an increased burden of comorbidities, but data on mortality in recently diagnosed IIM are conflicting. Also, little is known when, if ever, in relation to IIM diagnosis, mortality is increased.

    Methods: A population-based IIM cohort of patients diagnosed between 2002 and 2011 and general population comparators were identified using healthcare registers. Read More

    Clinicopathologic features of myositis patients with CD8-MHC-1 complex pathology.
    Neurology 2017 Sep 9;89(10):1060-1068. Epub 2017 Aug 9.
    From the Department of Neurology (C.I., A.K., K.T., N.U., M.H.M., Y.N., H.I., S.T., J.S.), Graduate School of Medicine, the University of Tokyo; Division of Neurology (M.K., K.K.), Department of Internal Medicine, National Defense Medical College, Saitama; Department of Neurology (A.H.), National Center for Global Health and Medicine; Department of Neurology (M.H.M.), Federation of National Public Service Personnel Mutual Aid Associations Toranomon Hospital; Okinaka Memorial Institute For Medical Research (M.H.M.); and Department of Neurology (J.G.), International University of Health and Welfare, Mita Hospital, Tokyo, Japan.
    Objective: To determine the clinical features of myositis patients with the histopathologic finding of CD8-positive T cells invading non-necrotic muscle fibers expressing major histocompatibility complex class 1 (CD8-MHC-1 complex), which is shared by polymyositis (PM) and inclusion body myositis (IBM), in relation to the p62 immunostaining pattern of muscle fibers.

    Methods: All 93 myositis patients with CD8-MHC-1 complex who were referred to our hospital from 1993 to 2015 were classified on the basis of the European Neuromuscular Center (ENMC) diagnostic criteria for IBM (Rose, 2013) or PM (Hoogendijk, 2004) and analyzed.

    Results: The 93 patients included were 17 patients with PM, 70 patients with IBM, and 6 patients who neither met the criteria for PM nor IBM in terms of muscle weakness distribution (unclassifiable group). Read More

    Relevance of ultrasonography in assessing disease activity in patients with idiopathic inflammatory myopathies.
    Int J Rheum Dis 2017 Aug 7. Epub 2017 Aug 7.
    Department of Rheumatology, Hospital Conde de Bertiandos, ULSAM, Ponte de Lima, Portugal.
    Aim: Idiopathic inflammatory myopathies (IIM) comprise a group of rare and heterogeneous diseases difficult to diagnose and follow up. Precise measures for assessing disease activity are not available at the moment. Our objective was to evaluate the usefulness of ultrasonography (US) as a monitoring tool in IIM. Read More

    Statin-associated immune mediated necrotising myopathy: a New Zealand case series showing possible over-representation in Pacific Islanders.
    Intern Med J 2017 Aug 7. Epub 2017 Aug 7.
    Department of Medicine, Dunedin school of Medicine, University of Otago.
    Aims: To review incident cases of statin-associated immune mediated necrotising myopathy (IMNM) associated with anti-HMGCR antibodies in a single New Zealand centre, over a two year period.

    Methods: Four incident cases of statin-associated IMNM were seen between 2014 and 2016. Their presentation, investigation, treatment and current response to treatment are summarised. Read More

    Treatment of Fulminant Giant Cell Myocarditis Associated with Polymyositis Using a Left Ventricular Assist Device and Subsequent Corticosteroid and Immunosuppressive Therapy Leading to Remission.
    Intern Med 2017 Aug 1;56(16):2155-2158. Epub 2017 Aug 1.
    Division of Cardiovascular Medicine, Kobe University Graduate School of Medicine, Japan.
    A 58-year-old man with a recent history of generalized myalgia and muscle weakness was transferred to our hospital because of acute progressive dyspnea. The patient underwent left ventricular (LV) assist device (LVAD) implantation due to cardiogenic shock with a LV ejection fraction (LVEF) of 6%. The histological findings obtained from LV apex showed the infiltration of multinucleated giant cells and severe myocardial contusion. Read More

    Using multi-omics methods to understand dermatomyositis/polymyositis.
    Autoimmun Rev 2017 Oct 1;16(10):1044-1048. Epub 2017 Aug 1.
    Department of Rheumatology, Xiangya Hospital, Central South University, 87 Xiangya Road, Changsha, Hunan 410008, People's Republic of China. Electronic address:
    Idiopathic inflammatory myopathies (IIM) are a group of rare and heterogeneous autoimmune diseases, and the most common subtypes are dermatomyositis (DM) and polymyositis (PM). Despite extensive efforts, the underlying mechanism of IIM remains unclear. Recent efforts to understand the pathogenesis of IIM have included genomics, epigenetics, transcriptomics, proteomics and autoantibody studies. Read More

    Acute heart failure and rhabdomyolysis: a clue for the diagnosis of polymyositis with cardiac involvement.
    Reumatismo 2017 Aug 3;69(2):78-83. Epub 2017 Aug 3.
    Department of Internal Medicine, Centro Hospitalar São João, Porto.
    Polymyositis is an idiopathic inflammatory myopathy, characterized by proximal muscle weakness and sometimes extramuscular manifestations. We report the case of a 51-year-old male, with history of complete heart block, which required pacemaker implantation, and subsequently heart failure, presenting to the emergency department with worsening of dyspnea and peripheral edema. He was admitted to the Internal Medicine ward with acute heart failure and started on diuretic therapy. Read More

    Ultrasound in Arthritis.
    Radiol Clin North Am 2017 Sep 12;55(5):985-996. Epub 2017 Jun 12.
    Nuffield Orthopaedic Centre, Oxford University Hospitals NHS Trust, Windmill Road, Oxford, OX3 7LD, UK.
    Ultrasound is currently performed in everyday rheumatologic practice. It is used for early diagnosis, to monitor treatment results, and to diagnose remission. The spectrum of pathologies seen in arthritis with ultrasound includes early inflammatory features and associated complications. Read More

    Case of Mycobacterium haemophilum misdiagnosed as Mycobacterium intracellulare due to one base insertion in the bacterial genome.
    J Dermatol 2017 Aug 3. Epub 2017 Aug 3.
    Dermatology, Department of Internal Related, Faculty of Medicine, Kobe University Graduate School of Medicine, Kobe, Japan.
    Mycobacterium haemophilum is a slow-growing, non-tuberculous mycobacteria that causes cutaneous infection. We describe a case of cutaneous infection in a 68-year-old Japanese man with polymyositis. This was caused by M. Read More

    The interaction between anti-Ro/SSA and anti-La/SSB autoantibodies and anti-infectious antibodies in a wide spectrum of auto-immune diseases: another angle of the autoimmune mosaic.
    Clin Exp Rheumatol 2017 Jul 6. Epub 2017 Jul 6.
    Zabludowicz Center for Autoimmune Diseases, Chaim Sheba Medical Center; Sackler Faculty of Medicine, Tel-Aviv University; and Incumbent of the Laura Schwarz-KipChair for Research of Autoimmune Diseases, Tel-Aviv University, Israel.
    Objectives: The presence of anti-Ro/SSA and anti-La/SSB antibodies has been linked with autoimmunity in general and with several autoimmune diseases (AID) in particular. In the current study we evaluated these antibodies in a wide spectrum of AID as well as the links between them and anti-infectious antibodies.

    Methods: We examined 2082 sera from patients with 16 different AID compared to 524 sera from geographically-matched healthy controls, for the presence and titres of anti-Ro/SSA and anti-La/SSB. Read More

    Comparisons of neutrophil-, monocyte-, eosinophil-, and basophil- lymphocyte ratios among various systemic autoimmune rheumatic diseases.
    APMIS 2017 Oct 2;125(10):863-871. Epub 2017 Aug 2.
    Department of Laboratory Diagnostics, Changzheng Hospital, Second Military Medical University, Shanghai, China.
    This study was aimed to evaluate levels of neutrophil- (NLR), monocyte- (MLR), eosinophil- (ELR), and basophil-lymphocyte ratio (BLR) and their association with inflammatory markers in systemic autoimmune rheumatic diseases (SARDs). A total of 1139 SARD patients and 170 healthy individuals were enrolled. Clinical and laboratory data were extracted. Read More

    Idiopathic inflammatory myopathies - a guide to subtypes, diagnostic approach and treatment.
    Clin Med (Lond) 2017 Jul;17(4):322-328
    NIHR Manchester Biomedical Research Centre, Central Manchester NHS Foundation Trust, Manchester Academic Health Science Centre, Manchester, UK and Salford Royal NHS Foundation Trust, Manchester Academic Health Science Centre, Salford, UK.
    The idiopathic inflammatory myopathies are a group of conditions characterised by inflammation of muscles (myositis) and other body systems. The diagnosis can be challenging because of the many potential clinical features and extra-muscular manifestations, which may be seemingly unrelated. An accurate diagnosis requires up-to-date understanding of the clinical manifestations, different clinical subtypes and appropriate interpretation of investigations, including newly described serological subtypes. Read More

    Clinical characteristics of autoimmune rheumatic disease-related organizing pneumonia.
    Clin Rheumatol 2017 Jul 26. Epub 2017 Jul 26.
    Department of Rheumatology, Beijing Chao-yang Hospital, Capital Medical University, #8 Gong-Ti South Road, Chaoyang District, Beijing, 100020, China.
    To study the clinical characteristics of autoimmune rheumatic disease-related organizing pneumonia (AIRD-OP), the clinical presentation, radiological findings, treatment, and outcome of AIRD-OP patients were analyzed, in comparison with patients with cryptogenic organizing pneumonia (COP). A total of 131 OP patients were identified, including 57 cases of AIRD-OP, 35 cases of COP, and 39 cases of other disease-related OPs. Among AIRD-OP patients, 36 (63%) presented the symptoms of OP at onset. Read More

    Hypercapnic respiratory failure during pregnancy due to polymyositis-related respiratory muscle weakness: a case report.
    J Med Case Rep 2017 Jul 26;11(1):203. Epub 2017 Jul 26.
    Department of Medical ICU, Hamad General Hospital, P.O. Box 3050, Doha, Qatar.
    Background: Polymyositis is a rare medical disorder complicating pregnancy. Ventilatory muscle weakness leading to respiratory failure is an uncommon manifestation of this autoimmune disease. We report a case of life-threatening hypercapnic respiratory failure due to polymyositis-related respiratory muscle weakness in a pregnant woman. Read More

    Malignancy screening in autoimmune myositis amongst Australian Rheumatologists.
    Intern Med J 2017 Jul 25. Epub 2017 Jul 25.
    Director of Rheumatology, 100 Angus Smith Drive, Townsville Hospital, Douglas, Queensland, Australia.
    Background: The international literature advocates for cancer screening in newly diagnosed patients with autoimmune myositis; however there is no widely-accepted consensus or guideline to outline the optimal cancer screening strategy and the evidence is currently insufficient to support any recommendation.

    Aims: Our study aimed to establish the current trends in practice in malignancy screening in autoimmune myositis amongst Australian Rheumatologists.

    Methods: All Rheumatologists who were full members of the Australian Rheumatology Association in 2016 (386) were invited to complete an online questionnaire. Read More

    RIG-I expression in perifascicular myofibers is a reliable biomarker of dermatomyositis.
    Arthritis Res Ther 2017 Jul 24;19(1):174. Epub 2017 Jul 24.
    Neuromuscular Diseases Unit, Neurology Department, Hospital de la Santa Creu i Sant Pau and Institut de Recerca Sant Pau, Universitat Autònoma de Barcelona, Sant Antoni Maria Claret 167, 08025, Barcelona, Spain.
    Background: Dermatomyositis (DM) is inflammatory myopathy or myositis characterized by muscle weakness and skin manifestations. In the differential diagnosis of DM the evaluation of the muscle biopsy is of importance among other parameters. Perifascicular atrophy in the muscle biopsy is considered a hallmark of DM. Read More

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