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    [Rituximab for the treatment of poly- and dermatomyositis : Results from the GRAID-2 registry].
    Z Rheumatol 2017 May 23. Epub 2017 May 23.
    Charité Universitätsmedizin Berlin, Berlin, Deutschland.
    Introduction: In the treatment of poly- and dermatomyositis, only a limited number of treatment modalities are established.

    Objective: The goal of the GRAID-2 registry was to study off-label use of biologic drugs for this indication in Germany.

    Patients And Methods: Analysis of the data of the GRAID-2 registry for poly- and dermatomyositis. Read More

    Fetal and neonatal involvement in maternal rheumatologic disease.
    J Matern Fetal Neonatal Med 2017 May 22:1-22. Epub 2017 May 22.
    a Department of Pediatrics , Hamamatsu University School of Medicine , Shizuoka , Japan.
    A pregnancy complicated with rheumatologic diseases can have various influences on the fetus and/or neonate. Maternal systemic lupus erythematosus (SLE) may cause preterm and/or small for gestational age (SGA) delivery and neonatal lupus (NL). Some neonates with NL have congenital heart block (CHB) with increased morbidity and mortality, even requiring pacemakers. Read More

    Idiopathic Inflammatory Myopathies: A Review of the Classification and Impact of Pathogenesis.
    Int J Mol Sci 2017 May 18;18(5). Epub 2017 May 18.
    Department of Medicine, Division of Rheumatic Diseases, Case Western Reserve University School of Medicine and University Hospitals Cleveland Medical Center, Cleveland, OH 44106-5076, USA.
    Idiopathic inflammatory myopathies (IIMs) are a group of autoimmune muscle diseases with significant morbidity and mortality. This review details and updates the pathogenesis and emerging importance of myositis-specific antibodies in the development of IIMs. An increase in the understanding of how these myositis-specific antibodies play a role in IIMs has led to the further categorization of IIMs from the traditional polymyositis versus dermatomyositis, to additional subcategories of IIMs such as necrotizing autoimmune myositis (NAM). Read More

    ["Therapy-resistant polymyositis" - is the diagnosis correct?]
    Z Rheumatol 2017 May 18. Epub 2017 May 18.
    Klinik und Poliklinik für Neurologie, Universitätsklinikum Köln, Kerpener Str. 62, 50937, Köln, Deutschland.
    We report the case of a 32-year-old woman with severely elevated serum creatine kinase (CK; 80,000 U/l) and progressive proximal pareses. As muscular biopsy showed inflammatory infiltrates, polymyositis was suspected and immunosuppressive treatment was initiated. However, clinical improvement could not be achieved. Read More

    Malignancy as a comorbidity in rheumatic diseases: a retrospective hospital-based study.
    Clin Rheumatol 2017 May 16. Epub 2017 May 16.
    Division of Oncology, Guang An Men Hospital affiliated to China Academy of Traditional Chinese Medicine, Beijing, 100053, China.
    Patients with Rheumatic diseases (RDs) are at an increased risk of malignancies compared with the general population. The aim of this study was to examine the relative frequency of several cancers in a single homogeneous cohort of patients with different RDs. Patients diagnosed with rheumatoid arthritis (RA), Ankylosing spondylitis (AS), Sjögren's syndrome (SS), systemic lupus erythematosus (SLE), systemic sclerosis (SSc), dermatomyositis (DM), or polymyositis were included. Read More

    A possible role of low regulatory T cells in anti-acetylcholine receptor antibody positive myasthenia gravis after bone marrow transplantation.
    BMC Neurol 2017 May 15;17(1):93. Epub 2017 May 15.
    Department of Neurology, Fukushima Medical University, 1 Hikariga-oka, Fukushima, Fukushima, 960-1295, Japan.
    Background: Chronic graft-versus-host disease (GVHD) appears several months following allogenic hematopoietic stem cell transplantation (HSCT) and is clinically analogous to autoimmune disorder. Polymyositis is a common neuromuscular disorder in chronic GVHD, but myasthenia gravis (MG) is extremely rare. Hence, its pathophysiology and treatment have not been elucidated. Read More

    Idiopathic inflammatory myopathy and the risk of venous thromboembolism: a meta-analysis.
    Rheumatol Int 2017 May 10. Epub 2017 May 10.
    Division of Rheumatology, Department of Internal Medicine, Korea University Medical Center, Korea University College of Medicine, 73, Inchon-ro, Seongbuk-gu, Seoul, 02841, Korea.
    This study aimed at analyzing published data on the association between idiopathic inflammatory myopathy and venous thromboembolism (VTE). We examined studies on VTE risk in patients with polymyositis (PM) and/or dermatomyositis (DM), in the MEDLINE, EMBASE, and Cochrane databases and via manual searches. We performed a meta-analysis on the relative risks (RRs) of VTE, pulmonary embolism (PE), and deep vein thrombosis (DVT) in these patients. Read More

    Juvenile dermatomyositis/polymyositis and lymphoma.
    J Neurol Sci 2017 Jun 27;377:19-24. Epub 2017 Mar 27.
    Department of Neurology, Weill Cornell Medicine/New York Presbyterian Hospital, 525 East 68th Street, New York, NY 10065-4885, USA. Electronic address:
    In patients with juvenile dermatomyositis/polymyositis (JDM/PM), malignancy is a rare phenomenon. An extensive workup for neoplastic disease is not routinely indicated, but should be considered if unusual physical findings are noted at JDM/PM diagnosis or during follow-up period. The objective of this literature review was to assess for any association between, and disease profile of, JDM/PM and lymphoma in childhood. Read More

    Diagnosis and Management of Immune-Mediated Myopathies.
    Mayo Clin Proc 2017 May;92(5):826-837
    Neuromuscular Medicine Division, Department of Neurology, Mayo Clinic, Rochester, MN. Electronic address:
    Immune-mediated myopathies (IMMs) are a heterogeneous group of acquired muscle disorders characterized by muscle weakness, elevated creatine kinase levels, and myopathic electromyographic findings. Most IMMs feature the presence of inflammatory infiltrates in muscle. However, the inflammatory exudate may be absent. Read More

    Positive Association between ANKRD55 Polymorphism 7731626 and Dermatomyositis/Polymyositis with Interstitial Lung Disease in Chinese Han Population.
    Biomed Res Int 2017 2;2017:2905987. Epub 2017 Apr 2.
    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.
    Single nucleotide polymorphisms (SNPs) in TNFSF4 and ANKRD55 genes have been shown to be associated with several autoimmune diseases, although whether these genes are susceptibility genes for dermatomyositis/polymyositis (DM/PM) has, to date, not been reported. This study aimed to investigate the potential associations of these SNPs with DM/PM in a Chinese Han population. Five SNPs in TNFSF4 (rs2205960, rs844644, and rs844648) and ANKRD55 (rs6859219, rs7731626) genes were genotyped using the SequenomMassArray system in 2297 Chinese individuals. Read More

    Anti-SS-A/Ro antibody positivity as a risk factor for relapse in patients with polymyositis/dermatomyositis.
    Mod Rheumatol 2017 May 2:1-6. Epub 2017 May 2.
    a Department of Nephrology, Rheumatology, Endocrinology and Metabolism , Okayama University Graduate School of Medicine, Dentistry and Pharmaceutical Sciences , Okayama , Japan.
    Objective: The objective of this study is to elucidate predictors of relapse in patients with polymyositis and dermatomyositis (PM/DM).

    Methods: Fifty PM/DM patients who achieved disease stabilization at Okayama University Hospital in 2004-2014 were enrolled retrospectively. Candidate predictors such as demographic factors, clinical symptoms, laboratory data, and treatment status were compared. Read More

    New Insights into Antisynthetase Syndrome.
    Maedica (Buchar) 2016 Jun;11(2):130-135
    "Dr. Ion Stoia" Clinical Center for Rheumatic Diseases, Bucharest, Romania.
    Antisynthetase syndrome (ASS) is a rare chronic autoimmune disorder (2-3 times more common in women than in men), associated with interstitial lung disease (the most important feature), dermatomyositis (DM), and polymyositis (PM). The cause of ASS is unknown. Recent developments in immunology have improved our knowledge and it is now possible to classify ASS according to the presence of myositis specific autoantibodies. Read More

    The long-term outcome of interstitial lung disease with anti-aminoacyl-tRNA synthetase antibodies.
    Respir Med 2017 Jun 15;127:57-64. Epub 2017 Apr 15.
    Department of Respiratory Medicine, Graduate School of Medicine, Kyoto University, Kyoto, Japan.
    Rationale: Anti-aminoacyl transfer RNA synthetase antibodies (anti-ARS) are a group of myositis-specific autoantibodies that are detected in the sera of patients with polymyositis and dermatomyositis (PM/DM) and also in those of patients with idiopathic interstitial pneumonias without any connective tissue disease (CTD), including PM/DM. Although we reported the clinical characteristics of interstitial lung disease with anti-ARS antibodies (ARS-ILD) with and without PM/DM, the long-term prognosis of ARS-ILD remains undetermined. As our previous studies revealed that ARS-ILD without PM/DM was similar to CTD-associated ILD, and that ARS-ILD with PM/DM was radiologically suggestive of a nonspecific interstitial pneumonia (NSIP) pathological pattern, we hypothesized that the prognosis of ARS-ILD might be distinct from that of idiopathic pulmonary fibrosis (IPF) without anti-ARS. Read More

    Anti-MDA5 antibody as a potential diagnostic and prognostic biomarker in patients with dermatomyositis.
    Oncotarget 2017 Apr;8(16):26552-26564
    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.
    The presence of anti-MDA5 antibodies in serum represents an important biomarker in the diagnosis and prediction of prognosis for patients with idiopathic inflammatory myopathies (IIMs). Due to conflicting results that have been reported regarding the detection of anti-MDA5 antibodies, the goal of this study was to assess a potential association between the presence of anti-MDA5 antibodies and dermatomyositis/polymyositis (DM/PM), as well as the diagnostic and prognostic values of anti-MDA5 antibodies for DM/PM. For this, a review of literature published prior to October 15, 2016 was conducted. Read More

    A case of chronic sarcoid myopathy with Basedow's disease and Sjogren's syndrome: A case series of sarcoid myopathy.
    Rinsho Shinkeigaku 2017 Apr 27. Epub 2017 Apr 27.
    Department of Neurology, National Center Hospital, National Center of Neurology and Psychiatry, Tokyo, Japan.
    We report a 62-year-old woman with a history of Basedow's disease and Sjogren's syndrome who presented with slowly progressive limb muscle weakness over the course of ten years. On physical examination, she had dry eye and mouth, but was otherwise normal. Neurological examination revealed symmetrical proximal dominant muscle weakness. Read More

    Toll-Like Receptor 4-Myeloid Differentiation Primary Response Gene 88 Pathway Is Involved in the Inflammatory Development of Polymyositis by Mediating Interferon-γ and Interleukin-17A in Humans and Experimental Autoimmune Myositis Mouse Model.
    Front Neurol 2017 12;8:132. Epub 2017 Apr 12.
    Department of Neurology, Xijing Hospital, The Fourth Military Medical University, Xi'an, China.
    Objective: Toll-like receptor 4 (TLR4) is one of the key players in the development of many autoimmune diseases. To determine the possible role of TLR4 in polymyositis (PM) development, we collected muscle samples from PM patients and mice subjected to an experimental autoimmune myositis (EAM) model.

    Methods: We measured TLR4-MyD88 pathway-related factors, interferon-γ (IFN-γ), and interleukin-17A (IL-17A) in EAM mice and PM patients. Read More

    A Rare Case of Necrotizing Myopathy and Fibrinous and Organizing Pneumonia with Anti-EJ Antisynthetase Syndrome and SSA Antibodies.
    Am J Case Rep 2017 Apr 25;18:448-453. Epub 2017 Apr 25.
    Division of Pulmonary and Critical Care Medicine, Bronx Lebanon Hospital Center, Affiliated with Icahn School of Medicine at Mount Sinai, Bronx, NY, USA.
    BACKGROUND Idiopathic inflammatory myopathies are autoimmune disorders that can involve the skin, joints, muscles, and lungs. The most common of these disorders are dermatomyositis, polymyositis, overlap syndrome, and inclusion body myositis. Necrotizing autoimmune myopathy is an idiopathic inflammatory myopathy that is rarely associated with Sjögren's syndrome. Read More

    Inflammatory myopathy associated with myasthenia gravis with and without thymic pathology: Report of four cases and literature review.
    Autoimmun Rev 2017 Jun 13;16(6):644-649. Epub 2017 Apr 13.
    Nuffield Department of Clinical Neurosciences, Oxford University Hospitals and University of Oxford, Oxford, United Kingdom. Electronic address:
    Introduction: The association of myasthenia gravis (MG) and inflammatory myopathy is rare and often only one of the diseases is diagnosed. Thymus pathology may be in the origin of such disease association.

    Methods: We described four patients with both MG and inflammatory myopathy. Read More

    Subcutaneous Emphysema, Pneumomediastinum and Pneumothorax in a Patient with Dermatomyositis.
    Iran J Otorhinolaryngol 2017 Mar;29(91):113-116
    Department of Otorhinolaryngology-Head & Neck Surgery, Mashhad University of Medical Sciences, Mashhad, Iran.
    Introduction: Spontaneous pneumomediastinum, pneumothorax, and subcutaneous emphysema are rare, but serious complications of inflammatory myopathies and occur more commonly in DM than PM. complications of dermatomyositis (DM) and polymyositis (PM), both of which can be fatal.

    Case Report: A 20-year-old woman was admitted with neck pain, dyspnea, cough, and fever. Read More

    A case of anti-aminoacyl tRNA synthetase (ARS) antibody-positive polymyositis (PM)/dermatomyositis (DM)-associated interstitial pneumonia (IP) successfully controlled with bosentan therapy.
    Respir Med Case Rep 2017 29;21:62-65. Epub 2017 Mar 29.
    Department of Pulmonary Medicine and Oncology, Graduate School of Medicine, Nippon Medical School, Tokyo, Japan.
    A 72-year-old woman was admitted to our hospital and was diagnosed with interstitial pneumonia (IP) associated with amyopathic dermatomyositis (ADM). The patient experienced three acute IP exacerbations in the 7 years that followed, which were each treated and resolved with steroid pulse therapy. The patient was closely examined for respiratory failure with right heart catheterization (RHC), which demonstrated that she had a mean pulmonary artery pressure (mPAP) of 34 mmHg. Read More

    Pregnancy in polymyositis or dermatomyositis: retrospective results from a tertiary centre in China.
    Rheumatology (Oxford) 2017 Apr 5. Epub 2017 Apr 5.
    Department of Rheumatology, Peking Union Medical College Hospital, Peking Union Medical College & Chinese Academy of Medical Science, Key Laboratory of Rheumatology and Clinical Immunology, Ministry of Education, Beijing.
    Objective: . To examine if patients with PM/DM are at higher risk of complicated pregnancies.

    Methods: . Read More

    2016 American College of Rheumatology/European League Against Rheumatism criteria for minimal, moderate, and major clinical response in adult dermatomyositis and polymyositis: An International Myositis Assessment and Clinical Studies Group/Paediatric Rheumatology International Trials Organisation Collaborative Initiative.
    Ann Rheum Dis 2017 May;76(5):792-801
    Charles University, Prague, Czech Republic.
    To develop response criteria for adult dermatomyositis (DM) and polymyositis (PM). Expert surveys, logistic regression, and conjoint analysis were used to develop 287 definitions using core set measures. Myositis experts rated greater improvement among multiple pairwise scenarios in conjoint analysis surveys, where different levels of improvement in 2 core set measures were presented. Read More

    2016 American College of Rheumatology/European League Against Rheumatism Criteria for Minimal, Moderate, and Major Clinical Response in Juvenile Dermatomyositis: An International Myositis Assessment and Clinical Studies Group/Paediatric Rheumatology International Trials Organisation Collaborative Initiative.
    Ann Rheum Dis 2017 May;76(5):782-791
    Istituto Giannina Gaslini, Pediatria II - Reumatologia, PRINTO, Genoa, Italy.
    To develop response criteria for juvenile dermatomyositis (DM). We analysed the performance of 312 definitions that used core set measures from either the International Myositis Assessment and Clinical Studies Group (IMACS) or the Paediatric Rheumatology International Trials Organisation (PRINTO) and were derived from natural history data and a conjoint analysis survey. They were further validated using data from the PRINTO trial of prednisone alone compared to prednisone with methotrexate or cyclosporine and the Rituximab in Myositis (RIM) trial. Read More

    Inflammatory Myopathies with Cutaneous Involvement: from Diagnosis to Therapy.
    Folia Med (Plovdiv) 2017 Mar;59(1):7-13
    Department of Dermatology and Venereology, Medical University of Sofia, 1 St Georgi Sofiiski St., 1431 Sofia.
    The group of idiopathic inflammatory myopathies (IIM) include various disorders of skeletal muscles with or without skin involvement. The most common types are dermatomyositis (DM), polymyositis (PM), inclusion body myositis (IBM) and necrotizing autoimmune myopathy (NAM). Dermatomyositis subdivides into various clinical forms such as juvenile, amyopathic or paraneoplastic dermatomyositis, scleromyositis, overlap or anti-synthetase syndromes, etc. Read More

    2016 American College of Rheumatology/European League Against Rheumatism Criteria for Minimal, Moderate, and Major Clinical Response in Adult Dermatomyositis and Polymyositis: An International Myositis Assessment and Clinical Studies Group/Paediatric Rheumatology International Trials Organisation Collaborative Initiative.
    Arthritis Rheumatol 2017 May 6;69(5):898-910. Epub 2017 Apr 6.
    Charles University, Prague, Czech Republic.
    Objective: To develop response criteria for adult dermatomyositis (DM) and polymyositis (PM).

    Methods: Expert surveys, logistic regression, and conjoint analysis were used to develop 287 definitions using core set measures. Myositis experts rated greater improvement among multiple pairwise scenarios in conjoint analysis surveys, where different levels of improvement in 2 core set measures were presented. Read More

    2016 American College of Rheumatology/European League Against Rheumatism Criteria for Minimal, Moderate, and Major Clinical Response in Juvenile Dermatomyositis: An International Myositis Assessment and Clinical Studies Group/Paediatric Rheumatology International Trials Organisation Collaborative Initiative.
    Arthritis Rheumatol 2017 May 6;69(5):911-923. Epub 2017 Apr 6.
    Istituto Giannina Gaslini, Pediatria II - Reumatologia, PRINTO, Genoa, Italy.
    Objective: To develop response criteria for juvenile dermatomyositis (DM).

    Methods: We analyzed the performance of 312 definitions that used core set measures from either the International Myositis Assessment and Clinical Studies Group (IMACS) or the Paediatric Rheumatology International Trials Organisation (PRINTO) and were derived from natural history data and a conjoint analysis survey. They were further validated using data from the PRINTO trial of prednisone alone compared to prednisone with methotrexate or cyclosporine and the Rituximab in Myositis (RIM) trial. Read More

    Increased risk of comorbid rheumatic disorders in vitiligo patients: A nationwide population-based study.
    J Dermatol 2017 Mar 31. Epub 2017 Mar 31.
    Department of Dermatology, Veterans Health Service Medical Center, Seoul, Korea.
    Vitiligo is a common acquired depigmentation disorder. Previous studies have shown that vitiligo is associated with a variety of autoimmune disorders. However, a large-scale epidemiological study focused on comorbid rheumatic disorders has not been undertaken. Read More

    Subclinical Cardiac Dysfunction in Polymyositis and Dermatomyositis: A Speckle-tracking Case-control Study.
    J Rheumatol 2017 Apr 1. Epub 2017 Apr 1.
    From the Cardiology and Arrhythmology Clinic, and the Clinical Medicine, Clinical and Molecular Sciences Department, Marche Polytechnic University, University Hospital "Ospedali Riuniti," Ancona, Italy. F. Guerra, MD, Cardiology and Arrhythmology Clinic, Marche Polytechnic University, University Hospital Ospedali Riuniti; C. Gelardi, MD, Clinical Medicine, Clinical and Molecular Sciences Department, Marche Polytechnic University, University Hospital Ospedali Riuniti; A. Capucci, MD, Professor, Cardiology and Arrhythmology Clinic, Marche Polytechnic University, University Hospital Ospedali Riuniti; A. Gabrielli, MD, Professor, Clinical Medicine, Clinical and Molecular Sciences Department, Marche Polytechnic University, University Hospital Ospedali Riuniti; M.G. Danieli, MD, PhD, Associate Professor, Clinical Medicine, Clinical and Molecular Sciences Department, Marche Polytechnic University, University Hospital Ospedali Riuniti. Address correspondence to Prof. M.G. Danieli, Clinica Medica, Dipartimento di Scienze Cliniche e Molecolari, Via Tronto 10, Torrette di Ancona, 60126 Italy. E-mail: Accepted for publication February 14, 2017.
    Objective: Subclinical heart disease occurs in up to 50% of patients with idiopathic inflammatory myopathies (IIM) and is difficult to detect through conventional imaging. We investigated the usefulness of global longitudinal strain (GLS) measurement to detect a subclinical systolic ventricular dysfunction in patients with IIM.

    Methods: We enrolled 28 patients with IIM and 28 matched controls in a 1:1 fashion. Read More

    [Thymoma and autoimmune diseases].
    Rev Med Interne 2017 Mar 29. Epub 2017 Mar 29.
    Service de médecine interne, hospices civils de Lyon, hôpital de la Croix-Rousse, université Claude-Bernard - Lyon 1, 103, grande rue de la Croix-Rousse, 69004 Lyon, France.
    The association between thymoma and autoimmunity is well known. Besides myasthenia gravis, which is found in 15 to 20% of patients with thymoma, other autoimmune diseases have been reported: erythroblastopenia, systemic lupus erythematosus, inflammatory myopathies, thyroid disorders, Isaac's syndrome or Good's syndrome. More anecdotally, Morvan's syndrome, limbic encephalitis, other autoimmune cytopenias, autoimmune hepatitis, and bullous skin diseases (pemphigus, lichen) have been reported. Read More

    MASTICATORY MUSCLE MYOSITIS IN A GRAY WOLF (CANIS LUPUS).
    J Zoo Wildl Med 2017 Mar;48(1):245-249
    A 10-yr-old male, neutered gray wolf ( Canis lupus ) was presented for atrophy of the temporalis and masseter muscles. Clinical signs and magnetic resonance imaging were consistent with a myopathy. Positive serology for antibody titers directed against Type 2M myofibers, and the observation of a mixed mononuclear inflammatory cell infiltrate along with eosinophils and neutrophils within the temporalis muscle, were diagnostic for masticatory muscle myositis. Read More

    Value of whole-body magnetic resonance imaging for screening multifocal osteonecrosis in patients with polymyositis/dermatomyositis.
    Br J Radiol 2017 May 29;90(1073):20160780. Epub 2017 Mar 29.
    2 Department of Rheumatology, China-Japan Friendship Hospital, Beijing, China.
    Objective: To assess the value of coronal short-tau inversion recovery whole-body MRI (STIR-WBMRI) for screening osteonecrosis in patients with polymyositis (PM)/dermatomyositis (DM).

    Methods: The imaging and medical records of 129 patients with PM/DM who met the Bohan and Peter diagnostic criteria were retrospectively analyzed. STIR-WBMRI was performed in all patients. Read More

    DEPTOR-mTOR Signaling Is Critical for Lipid Metabolism and Inflammation Homeostasis of Lymphocytes in Human PBMC Culture.
    J Immunol Res 2017 27;2017:5252840. Epub 2017 Feb 27.
    Department of General Practice, West China Hospital of Sichuan University, Chengdu 610041, China.
    Abnormal immune response of the body against substances and tissues causes autoimmune diseases, such as polymyositis, dermatomyositis, and rheumatoid arthritis. Irregular lipid metabolism and inflammation may be a significant cause of autoimmune diseases. Although much progress has been made, mechanisms of initiation and proceeding of metabolic and inflammatory regulation in autoimmune disease have not been well-defined. Read More

    Red blood cell distribution width and neutrophil to lymphocyte ratio are correlated with disease activity of dermatomyositis and polymyositis.
    J Clin Lab Anal 2017 Mar 27. Epub 2017 Mar 27.
    Department of Rheumatology, Wuxi Second People's Hospital of Nanjing Medical University, Wuxi, China.
    Background: Previous studies indicated that both red blood cell distribution width (RDW) and neutrophil to lymphocyte ratio (NLR) were useful indices in assessing the disease activity of autoimmune diseases. However, the evidence for the association between RDW, NLR and dermatomyositis (DM) and polymyositis (PM) is limited. The aim of this study is to investigate the association between the disease activity of PM/DM and both RDW and NLR. Read More

    Analysis of sexual function of patients with dermatomyositis and polymyositis through self-administered questionnaires: a cross-sectional study.
    Rev Bras Reumatol Engl Ed 2017 Mar - Apr;57(2):134-140. Epub 2016 Dec 5.
    Universidade de São Paulo, Faculdade de Medicina, Disciplina de Reumatologia, São Paulo, SP, Brazil. Electronic address:
    Introduction: To date, there are no descriptions in the literature on gynecologic and sexual function evaluation in female patients with dermatomyositis (DM) and polymyositis (PM).

    Objective: To assess sexual function in female patients with DM/PM.

    Patients And Methods: This is a monocentric, cross-sectional study in which 23 patients (16 DM and 7 PM), with ages between 18 and 40 years, were compared to 23 healthy women of the same age group. Read More

    Inflammatory myopathies: A new landscape.
    Joint Bone Spine 2017 Mar 22. Epub 2017 Mar 22.
    Service de rhumatologie, hôpitaux universitaires de Strasbourg, 67000 Strasbourg, France; Centre de référence des maladies auto-immunes rares, hôpitaux universitaires de Strasbourg, 67000 Strasbourg, France; Fédération de médecine translationnelle de Strasbourg, université de Strasbourg, 67000 Strasbourg, France.
    Greater accuracy in clinical descriptions combined with advances in muscle histology and immunology have established that inflammatory myopathies (IMs), similarly to inflammatory rheumatic diseases, constitute a highly heterogeneous group of conditions. The topographic distribution, severity, and tempo of onset of the myopathy vary widely, and the histological findings distinguish at least five different profiles, which may reflect different pathophysiological processes. Most IMs are connective tissue diseases that can affect multiple organs, among which the most common targets are the skin, joints, and lungs. Read More

    A longitudinal cohort study of the anti-synthetase syndrome: increased severity of interstitial lung disease in black patients and patients with anti-PL7 and anti-PL12 autoantibodies.
    Rheumatology (Oxford) 2017 Mar 4. Epub 2017 Mar 4.
    Johns Hopkins University School of Medicine, Baltimore, MD, USA.
    Objective.: The aim was to study the prevalence, rate of appearance and severity of clinical features in patients with different anti-synthetase syndrome (ASyS) autoantibodies.

    Methods. Read More

    Dermatomyositis Associated with Lung Neuroendocrine Carcinoma.
    Intern Med 2017 17;56(6):719-724. Epub 2017 Mar 17.
    Respiratory Disease Center, Division of Respiratory Medicine, The Tazuke-Kofukai Medical Research Institute, Kitano Hospital, Japan.
    Dermatomyositis is associated with various types of malignancy. However, the association of dermatomyositis with lung neuroendocrine carcinoma is rare. We herein report a case of dermatomyositis with lung neuroendocrine carcinoma. Read More

    A study of acute muscle dysfunction with particular reference to dengue myopathy.
    Ann Indian Acad Neurol 2017 Jan-Mar;20(1):13-22
    Department of Neurology, King George Medical University, Lucknow, Uttar Pradesh, India.
    Background: Acute myopathy is a common cause of acute motor quadriparesis which has various etiologies with different courses of illness and prognosis depending on the cause. Understanding this diversity helps us in proper approach toward diagnosis, predicting the prognosis, and possible complications and in improving the treatments that are being provided. This study was planned to study the clinical, electrophysiological, and etiological profile of patients presenting with acute myopathy. Read More

    Relevance of clinical and autoantibody profiles in systemic sclerosis among Thais.
    Int J Rheum Dis 2017 Mar 10. Epub 2017 Mar 10.
    Department of Medicine, Faculty of Medicine, Khon Kaen University, Khon Kaen, Thailand.
    Objective: Autoantibody profiles in systemic sclerosis (SSc) and their relative clinical association vary between studies. The rate for being anti-topoisomerase-I (ATA) positive and the association with diffuse cutaneous the SSc subset (dcSSc) is higher among Thais than among Caucasians. The objective was to evaluate the relevance of clinical presentation, namely being positive for one or more autoantibodies among Thai SSc patients. Read More

    Subcutaneous IgG in the Myositis Spectrum Disorders.
    Curr Rheumatol Rev 2017 Mar 14. Epub 2017 Mar 14.
    Clinica Medica - Dipartimento di Scienze Cliniche e Molecolari, Università Politecnica delle Marche e Ospedali Riuniti Ancona. Italy.
    The efficacy of subcutaneous immunoglobulin is reported in several neurological disorders and, more recently, its use has been extended to other inflammatory diseases, such as the idiopathic inflammatory myopathies, including polymyositis and dermatomyositis. Due to the rarity of these disorders, the role of immunoglobulin, administered intravenously or subcutaneously, remains unclear and poorly investigated. We report our experience about the use of subcutaneous immunoglobulin in myositis spectrum disorders, from idiopathic inflammatory myopathies to more complex conditions, such as overlap and cancer-associated myositis or pregnancy. Read More

    Protecting Bone Health in Pediatric Rheumatic Diseases: Pharmacological Considerations.
    Paediatr Drugs 2017 Jun;19(3):193-211
    Pediatric Rheumatology Division, Floating Hospital for Children at Tufts Medical Center, 800 Washington Street, #190, Boston, MA, 02111, USA.
    Bone health in children with rheumatic conditions may be compromised due to several factors related to the inflammatory disease state, delayed puberty, altered life style, including decreased physical activities, sun avoidance, suboptimal calcium and vitamin D intake, and medical treatments, mainly glucocorticoids and possibly some disease-modifying anti-rheumatic drugs. Low bone density or even fragility fractures could be asymptomatic; therefore, children with diseases of high inflammatory load, such as systemic onset juvenile idiopathic arthritis, juvenile dermatomyositis, systemic lupus erythematosus, and those requiring chronic glucocorticoids may benefit from routine screening of bone health. Most commonly used assessment tools are laboratory testing including serum 25-OH-vitamin D measurement and bone mineral density measurement by a variety of methods, dual-energy X-ray absorptiometry as the most widely used. Read More

    Overlapping features of polymyositis and inclusion body myositis in HIV-infected patients.
    Neurology 2017 Apr 10;88(15):1454-1460. Epub 2017 Mar 10.
    From the Johns Hopkins University School of Medicine (T.E.L., E.H.M., L.C.-S., N.S., A.L.M.), Baltimore; and the National Institute of Arthritis and Musculoskeletal and Skin Diseases (I.P.-F., K.P., A.L.M.), National Institutes of Health, Bethesda, MD.
    Objective: To characterize patients with myositis with HIV infection.

    Methods: All HIV-positive patients with myositis seen at the Johns Hopkins Myositis Center from 2003 to 2013 were included in this case series. Muscle biopsy features, weakness pattern, serum creatine kinase (CK) level, and anti-nucleotidase 1A (NT5C1A) status of HIV-positive patients with myositis were assessed. Read More

    Follistatin Gene Therapy for Sporadic Inclusion Body Myositis Improves Functional Outcomes.
    Mol Ther 2017 Apr 6;25(4):870-879. Epub 2017 Mar 6.
    Center for Gene Therapy, Nationwide Children's Hospital, Columbus, OH 43205, USA; Department of Pediatrics, The Ohio State University, Columbus, OH 43205, USA.
    Sporadic inclusion body myositis, a variant of inflammatory myopathy, has features distinct from polymyositis/dermatomyositis. The disease affects men more than women, most commonly after age 50. Clinical features include weakness of the quadriceps, finger flexors, ankle dorsiflexors, and dysphagia. Read More

    Predictive factors for long-term outcome in polymyositis/dermatomyositis-associated interstitial lung diseases.
    Respir Investig 2017 Mar 8;55(2):130-137. Epub 2017 Feb 8.
    Second Division, Department of Internal Medicine, Hamamatsu University School of Medicine, 1-20-1 Handayama Higashi-ku, Hamamatsu 431-3192, Japan. Electronic address:
    Background: Interstitial lung disease (ILD) is strongly associated with polymyositis (PM), dermatomyositis (DM), and clinically amyopathic dermatomyositis (CADM). It is also related to mortality. Previous studies have highlighted that the acute form of PM/DM/CADM-associated ILD (PM/DM/CADM-ILD) has a poor short-term prognosis. Read More

    Neurological manifestations in dogs naturally infected by Leishmania infantum: descriptions of 10 cases and a review of the literature.
    J Small Anim Pract 2017 Mar;58(3):125-138
    Pingry Veterinary Hospital, Bari, 70126, Italy.
    In order to evaluate possible nervous system involvement in canine leishmaniasis, retrospective evaluation of all medical records of leishmaniotic dogs exhibiting neurological signs referred to our hospital over a 5-year period was performed. The records of 10 dogs were reviewed. Depending on the neuroanatomical localisation, the dogs underwent advanced diagnostic imaging, cerebrospinal fluid analysis, electrodiagnostic testing and histopathologic evaluations. Read More

    The clinical and pathological characteristics of nephropathies in connective tissue diseases in the Japan Renal Biopsy Registry (J-RBR).
    Clin Exp Nephrol 2017 Mar 2. Epub 2017 Mar 2.
    Division of Nephrology, Kanazawa Medical University School of Medicine, Uchinada, Japan.
    Background: In connective tissue diseases, a wide variety of glomerular, tubulointerstitial, and vascular lesions of the kidney are observed. Nonetheless, recent information is limited regarding renal lesions in connective tissue diseases, except in systemic lupus erythematosus (SLE).

    Methods: In this study, we used a nationwide database of biopsy-confirmed renal diseases in Japan (J-RBR) (UMIN000000618). Read More

    Sex differential association of dermatomyositis with Sjögren syndrome.
    CMAJ 2017 Feb;189(5):E187-E193
    Department of Internal Medicine (Tseng), Kaohsiung Municipal Ta-Tung Hospital; Department of Kinesiology (Chang), Health and Leisure Studies, National University of Kaohsiung; Division of Rheumatology (Tsai, Ou, Wu, Sung, Yen), Department of Internal Medicine, Kaohsiung Medical University Hospital; Graduate Institute of Medicine (Sung, Yen), College of Medicine, Kaohsiung Medical University; Division of Endocrinology and Metabolism, Department of Internal Medicine (Hsieh), Changhua Christian Hospital, Changhua, Taiwan; Graduate Institute of Integrated Medicine (Hsieh), China Medical University, Taichung, Taiwan
    Background: Although dermatomyositis and Sjögren syndrome share serologic autoantibodies and genetic polymorphisms, population data about the incidence of Sjögren syndrome in patients with dermatomyositis is unavailable. We performed a nationwide cohort study to explore the potential relation between dermatomyositis and Sjögren syndrome and, if an association exists, to elucidate whether it varies by sex.

    Methods: We identified all patients with newly diagnosed dermatomyositis from the Registry of Catastrophic Illness Database in Taiwan between Jan. Read More

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