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    An Unusual Suspect Causing Hypoxemic Respiratory Failure.
    J Investig Med High Impact Case Rep 2017 Jan-Mar;5(1):2324709616687587. Epub 2017 Jan 1.
    Medical College of Wisconsin, Milwaukee, WI, USA.
    Introduction: Antisynthetase syndrome (ASS) is characterized by the presence of anti-Jo-1 antibodies in conjunction with clinical findings of fever, polymyositis-dermatomyositis, and interstitial lung disease (ILD). Inflammatory myopathies carry a high risk of malignancy, but this association is less well outlined in ASS. We present the case of a patient with ASS who developed non-Hodgkin's lymphoma with acute hypoxemic respiratory failure. Read More

    Isolated Ro52 Antibodies as Immunological Marker of a Mild Phenotype of Undifferentiated Connective Tissue Diseases.
    Int J Rheumatol 2017 22;2017:3076017. Epub 2017 Jan 22.
    Immunology Unit, Central Laboratory, Complejo Hospitalario Universitario de Canarias, Tenerife, Spain.
    The term undifferentiated connective tissue disease (UCTD) is used to describe undiagnosed patients that do not fulfill classification criteria for definite connective tissue disease (Systemic Lupus, Systemic Sclerosis, Sjögren Syndrome, and Dermatomyositis/Polymyositis). It is important to find serological markers as predictors of the evolution or severity of these diseases. The objective of this retrospective study was to investigate if there was a milder subgroup of UCTD with a special clinical profile consisting only in the presence of anti-Ro52 autoantibodies. Read More

    Prominent subcutaneous oedema as a masquerading symptom of an underlying inflammatory myopathy.
    Intern Med J 2017 Feb;47(2):217-221
    Department of Clinical Immunology, Westmead Hospital, Sydney, New South Wales, Australia.
    The inflammatory myopathies are a group of immune-mediated inflammatory muscle disorders that typically present with marked proximal muscle weakness. We report four cases of inflammatory myopathies with marked subcutaneous oedema as their main complaint. Three of the four patients had normal or low levels of creatine kinase, an enzyme often markedly elevated in these disorders. Read More

    Identification of Palmitoleic Acid Controlled by mTOR Signaling as a Biomarker of Polymyositis.
    J Immunol Res 2017 17;2017:3262384. Epub 2017 Jan 17.
    Department of Rheumatology and Immunology, West China Hospital of Sichuan University, Chengdu 610041, China.
    Polymyositis (PM) is a chronic disease characterized by muscle pain, weakness, and increase in muscle-related enzymes, accompanied with inflammations in lymphocytes. However, it is not well understood how the molecular alternations in lymphocytes contribute to the development of polymyositis. The mechanistic target of rapamycin (mTOR) signaling is the central regulator of metabolism and inflammation in mammalian cells. Read More

    Therapeutic Plasma Exchange in Patients with Neurologic Disorders: Review of 63 Cases.
    Indian J Hematol Blood Transfus 2017 Mar 22;33(1):97-105. Epub 2016 Feb 22.
    0000 0001 0694 8546grid.411691.aDepartment of Hematology, Mersin University Faculty of Medicine, Çiftlikköy Kampüsü, Mersin, Turkey.
    Therapeutic plasma exchange (TPE) is a procedure that reduces circulating autoantibodies of the patients. TPE is commonly used in neurological disorders where autoimmunity plays a major role. We report our experience with regard to the indications, adverse events and outcomes of plasma exchange in neurological disorders. Read More

    Pyruvate kinase M2 and the mitochondrial ATPase Inhibitory Factor 1 provide novel biomarkers of dermatomyositis: a metabolic link to oncogenesis.
    J Transl Med 2017 Feb 10;15(1):29. Epub 2017 Feb 10.
    Departamento de Biología Molecular, Centro de Biología Molecular Severo, Ochoa, CSIC-UAM, Universidad Autónoma de Madrid, c/Nicolás Cabrera 1, 28049, Madrid, Spain.
    Background: Metabolic alterations play a role in the development of inflammatory myopathies (IMs). Herein, we have investigated through a multiplex assay whether proteins of energy metabolism could provide biomarkers of IMs.

    Methods: A cohort of thirty-two muscle biopsies and forty plasma samples comprising polymyositis (PM), dermatomyositis (DM) and sporadic inclusion body myositis (sIBM) and control donors was interrogated with monoclonal antibodies against proteins of energy metabolism using reverse phase protein microarrays (RPPA). Read More

    Association between rheumatic diseases and cancer: results from a clinical practice cohort study.
    Intern Emerg Med 2017 Feb 8. Epub 2017 Feb 8.
    Division of Internal Medicine, Immunorheumatology Unit, "Maggiore della Carità" Hospital, Corso Mazzini 18, 28100, Novara, Italy.
    The association between cancer and immune-mediated rheumatic conditions is controversial, especially as far as polymyalgia rheumatica (PMR) is concerned. Furthermore, no clinical feature has been shown to be suggestive of a paraneoplastic rheumatic syndrome. With the present study, we aim to address both these issues. Read More

    Myalgia in Patients with Dermatomyositis and Polymyositis Is Attributable to Fasciitis Rather Than Myositis: A Retrospective Study of 32 Patients who Underwent Histopathological Examinations.
    J Rheumatol 2017 Feb 1. Epub 2017 Feb 1.
    From the Division of Rheumatology, Department of Internal Medicine, the Jikei University School of Medicine, Tokyo, Japan. Supported by The Jikei University Research Fund. K. Noda, MD, PhD; K. Yoshida, MD, PhD; T. Ukichi, MD; K. Furuya, MD, PhD; K. Hirai, MD, PhD; I. Kingetsu, MD, PhD; D. Kurosaka, MD, PhD, Division of Rheumatology, Department of Internal Medicine, the Jikei University School of Medicine. Address correspondence to Dr. K. Noda, Division of Rheumatology, Department of Internal Medicine, the Jikei University School of Medicine, 3-25-8 Nishi-shimbashi, Minato-ku, Tokyo, Japan. E-mail: Accepted for publication December 15, 2016.
    Objective: To determine the association between fasciitis and the clinical variables in patients with dermatomyositis (DM) and polymyositis (PM).

    Methods: We retrospectively reviewed the medical records of 32 patients (24 DM, 8 PM) with newly diagnosed DM and PM and in whom fascia and muscle specimens were histopathologically examined. The relationship between fasciitis and the clinical variables was statistically analyzed. Read More

    The Correlation of Muscle Biopsy Scores with the Clinical Variables in Idiopathic Inflammatory Myopathies.
    Open Rheumatol J 2016 16;10:141-149. Epub 2016 Dec 16.
    Division of Rheumatology, Department of Internal Medicine, Chiang Mai University, Chiang Mai, Thailand.
    Objectives: To compare the muscle pathology findings among subgroups of idiopathic inflammatory myopathies (IIM) patients, and to determine the correlations of muscle biopsy scores with muscle power and creatine kinase (CK).

    Methods: The medical records of IIM patients consisting of the demographic data, clinical parameters and laboratory conducted were retrospectively reviewed. Their initial muscle biopsies were reviewed, and four domains were scored: inflammation, vascular, muscle, and connective tissue. Read More

    Spectrum of paediatric rheumatic diseases in Nigeria.
    Pediatr Rheumatol Online J 2017 Jan 31;15(1). Epub 2017 Jan 31.
    Department of Medicine, University of Ilorin Teaching Hospital, Ilorin, Kwara State, Nigeria.
    Background: Paediatric rheumatology service in Sub-Sahara African is virtually not available as there is a shortage of paediatric rheumatologists and other rheumatology health professionals. We aim to describe the clinical spectrum and the frequencies of paediatric rheumatic diseases (PRDs) in Lagos State University Teaching Hospital (LASUTH), Lagos, Nigeria.

    Methods: This is a retrospective review of patients with PRDs seen over a five year period (March 2010 to February 2016) at the rheumatology clinic and children ward of LASUTH. Read More

    Direct suppression of autoaggressive CD8+ T cells with CD80/86 blockade in CD8+ T cell-mediated polymyositis models of mice.
    Clin Exp Rheumatol 2017 Jan 27. Epub 2017 Jan 27.
    Department of Rheumatology, Graduate School of Medical and Dental Sciences, Tokyo Medical and Dental University (TMDU), Tokyo, Japan.
    Objectives: CD80/86 blockade to inhibit CD28 costimulation suppressed alloreactive human and murine CD4+ T cells but not alloreactive CD8+ T cells. In contrast, CD28 costimulation augments CD8+ T cell-mediated cell lysis in antigen-nonspecific stimulation. The present study was conducted to discern whether the CD80/86 blockade exerts therapeutic effects on CD8+ T cell-mediated polymyositis (PM) models of mice and whether the effects could be attributable to direct suppression of autoantigen-specific CD8+ T cells. Read More

    Inflammatory lung disease a potential risk factor for onset of idiopathic inflammatory myopathies: results from a pilot study.
    RMD Open 2016 26;2(2):e000342. Epub 2016 Dec 26.
    Unit of Cardiovascular Epidemiology, Karolinska Institutet, Institute of Environmental Medicine, Stockholm, Sweden; Centre for Occupational and Environmental Medicine, Stockholm County Council, Stockholm, Sweden.
    Objectives: To assess the association between inflammatory lung disease and the risk of developing idiopathic inflammatory myopathies.

    Methods: A population-based case-control study was conducted. Adult myositis cases, identified from the Swedish inpatient registry (diagnosed between 1995 and 1997), and randomly selected controls matched to cases on the date of birth, gender and residency, were asked to fill out a questionnaire with questions on lifestyle, environmental exposures and health. Read More

    Cytosolic 5'-nucleotidase 1A autoantibody profile and clinical characteristics in inclusion body myositis.
    Ann Rheum Dis 2017 Jan 25. Epub 2017 Jan 25.
    Department of Neurology, Center for Neuroscience Donders Institute for Brain, Cognition and Behaviour, Radboud University Medical Center, Nijmegen, The Netherlands.
    Objectives: Autoantibodies directed against cytosolic 5'-nucleotidase 1A have been identified in many patients with inclusion body myositis. This retrospective study investigated the association between anticytosolic 5'-nucleotidase 1A antibody status and clinical, serological and histopathological features to explore the utility of this antibody to identify inclusion body myositis subgroups and to predict prognosis.

    Materials And Methods: Data from various European inclusion body myositis registries were pooled. Read More

    Clinical significance of soluble CD163 in polymyositis-related or dermatomyositis-related interstitial lung disease.
    Arthritis Res Ther 2017 Jan 19;19(1). Epub 2017 Jan 19.
    Second Division, Department of Internal Medicine, Hamamatsu University School of Medicine, 1-20-1 Handayama, Hamamatsu, Shizuoka, 431-3192, Japan.
    Background: Macrophage activation is involved in the pathogenesis of polymyositis (PM)/dermatomyositis (DM). CD163, a scavenger receptor expressed on the surface of activated macrophages, mediates anti-inflammatory functions. This study aimed to evaluate the clinical significance of soluble CD163 (sCD163) in PM/DM-related interstitial lung disease (ILD). Read More

    Hypokalemic myopathy in primary aldosteronism: A case report.
    Exp Ther Med 2016 Dec 2;12(6):4064-4066. Epub 2016 Nov 2.
    Department of Nephrology, Lishui Hospital Affiliated to Zhejiang University, Lishui, Zhejiang 323000, P.R. China.
    Primary aldosteronism (PA) is a rare disorder. The majority of patients with PA present with typical features and are easily diagnosed. This disorder is usually diagnosed with hypokalemia, hypertension or an adrenal mass. Read More

    Atorvastatin-induced necrotizing autoimmune myositis: An emerging dominant entity in patients with autoimmune myositis presenting with a pure polymyositis phenotype.
    Medicine (Baltimore) 2017 Jan;96(3):e5694
    aDivisions of Rheumatology, Department of Medicine bInternal Medicine, Hôpital du Sacré-Coeur cDivision of Rheumatology, Department of Medicine, Centre Hospitalier de l'Université de Montréal, University of Montreal Faculty of Medicine, Montreal, QC dMitogen Advanced Diagnostics Laboratory, Cumming School of Medicine, University of Calgary, Calgary, AB eDepartment of Pathology, Hôpital Maisonneuve-Rosemont, University of Montreal Faculty of Medicine, Montreal, QC, Canada fVeterans Affairs Medical Center, University of Oklahoma Health Sciences Center gOklahoma Medical Research Foundation, Oklahoma City, OK hDepartment of Pathology, Hôpital Sainte-Justine iDepartment of Pathology and Cell Biology, University of Montreal Faculty of Medicine jDivision of Rheumatology, Department of Medicine, Centre hospitalier affilié universitaire régional de Trois-Rivières, University of Montreal Faculty of Medicine, Montreal kDivision of Rheumatology, Centre Hospitalier de l'Université Laval, Laval University Faculty of Medicine, Québec, QC, Canada.
    The general aim of this study was to evaluate the disease spectrum in patients presenting with a pure polymyositis (pPM) phenotype. Specific objectives were to characterize clinical features, autoantibodies (aAbs), and membrane attack complex (MAC) in muscle biopsies of patients with treatment-responsive, statin-exposed necrotizing autoimmune myositis (NAM). Patients from the Centre hospitalier de l'Université de Montréal autoimmune myositis (AIM) Cohort with a pPM phenotype, response to immunosuppression, and follow-up ≥3 years were included. Read More

    Development of myasthenia gravis 8 years after interstitial lung disease associated with antisynthetase (anti-EJ antibody) syndrome.
    Clin Case Rep 2017 Jan 20;5(1):61-65. Epub 2016 Dec 20.
    Department of Respiratory Medicine Saitama Cardiovascular and Respiratory Center Kumagaya Saitama Prefecture Japan.
    Patients with antisynthetase-positive interstitial lung disease (ILD) alone sometimes develop myositis during follow-up, but myasthenia gravis (MG) overlapping on antisynthetase syndrome is unusual. A 56-year-old woman with ILD and anti-EJ antibody treated for 8 years developed MG. Physicians should consider myositis and MG when patients develop muscle symptoms during follow-up. Read More

    More severe disease and slower recovery in younger patients with anti-3-hydroxy-3-methylglutaryl-coenzyme A reductase-associated autoimmune myopathy.
    Rheumatology (Oxford) 2017 Jan 17. Epub 2017 Jan 17.
    Department of Medicine, Johns Hopkins University School of Medicine, Baltimore
    Objective: To study disease severity and response to therapy in a large cohort of patients with anti-hydroxy-3-methylglutaryl-coenzyme A reductase (HMGCR)-associated myositis.

    Methods: Muscle strength, creatine kinase levels and treatments were assessed in anti-HMGCR-positive patients at each clinical visit. Univariate and multivariate analyses were used to analyse the influence of clinical characteristics on strength and the change in strength over time. Read More

    Immune-array Analysis in Sporadic Inclusion Body Myositis Reveals HLA-DRB1 Amino Acid Heterogeneity across the Myositis Spectrum.
    Arthritis Rheumatol 2017 Jan 13. Epub 2017 Jan 13.
    National Institute of Health Research Manchester Musculoskeletal Biomedical Research Unit, Central Manchester University Hospitals NHS Foundation Trust, University of Manchester, UK.
    Objective: Inclusion body myositis (IBM) is characterised by a combination of inflammatory and degenerative changes affecting muscle. While the primary cause of IBM is unknown, genetic factors may influence disease susceptibility. We conducted the largest genetic association study to date in IBM, investigating immune-related genes using the Immunochip. Read More

    Mixed Connective Tissue Disorder Complicated by Polymyositis, Sjogren's Syndrome, Pleural Effusion and Pericarditis.
    Cureus 2016 Dec 1;8(12):e906. Epub 2016 Dec 1.
    Department of Internal Medicine, Carthage Area Hospital, New York, USA.
    We report a case of a 24-year-old female with a history of asthma and gastroesophageal reflux disease (GERD). She presented to the emergency room with severe chest pain, chest tightness, and shortness of breath following an upper respiratory tract infection. The patient reported that she had a cough and runny nose one week prior to this presentation, followed by a sudden sharp pain in the center of the chest 8/10 in intensity on the visual analog scale and pleuritic in nature, which aggravated by deep breathing and lying down flat. Read More

    Use of intravenous immunoglobulin therapy for myositis: an audit in South Australian patients.
    Intern Med J 2017 Jan;47(1):112-115
    Rheumatology Department, Royal Adelaide Hospital, Adelaide, South Australia, Australia.
    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. Read More

    Polymyositis without Beneficial Response to Steroid Therapy: Should Miyoshi Myopathy be a Differential Diagnosis?
    Am J Case Rep 2017 Jan 5;18:17-21. Epub 2017 Jan 5.
    MRC Centre for Neuromuscular Diseases and Division of Molecular Neuroscience, University College London (UCL) Institute of Neurology, London, United Kingdom.
    BACKGROUND Miyoshi myopathy (MM) is an autosomal-recessive muscle disorder caused by mutations in the DYSF gene. Clinical features and histopathological changes in dysferlinopathies may mimic inflammatory myopathies and a high degree of clinical suspicion is required to guide the genetic investigation. CASE REPORT We report the case of a 16-year-old male who presented with severe bilateral calf pain and elevated CK levels (15 000 IU/l) who was on prolonged steroid therapy prompted by the clinical suspicion of inflammatory myopathy. Read More

    Anti-NXP2-Positive Paraneoplastic Dermatomyositis With Histopathologic Changes Confined to the Acrosyringia.
    Am J Dermatopathol 2017 Jan;39(1):e3-e7
    Departments of*Dermatology, †Immunology, and ‡Pathology, Fundación Jimenez Diaz, Universidad Autónoma, Madrid, Spain.
    Background: Paraneoplastic syndromes consist of a group of disorders that are not related to the extension of the primary tumor or its metastases and that might be the first manifestation of a hidden neoplasm. It is a well-known association between dermatomyositis (DM) and cancer, especially gynecological tumors in women and lung cancer in men.

    Methods: We describe the case of a 67-year-old male who developed muscular weakness and pruritic skin lesions. Read More

    Serum level of DNase1l3 in patients with dermatomyositis/polymyositis, systemic lupus erythematosus and rheumatoid arthritis, and its association with disease activity.
    Clin Exp Med 2016 Dec 30. Epub 2016 Dec 30.
    Department of Rheumatology and Immunology, First Affiliated Hospital, China Medical University, Shenyang, 110001, People's Republic of China.
    DNase1l3 is an endonuclease to degrade the chromatin of apoptotic or necrotic cells. Serum DNase1l3 may fulfill the function of clearance of chromatin released into the circulation by dying cells, which can trigger autoimmune responses. To date, it remains unclear whether serum DNase1l3 level associates with the pathogenesis of autoimmune diseases. Read More

    Sarcoplasmic MxA expression: A valuable marker of dermatomyositis.
    Neurology 2017 Jan 30;88(5):493-500. Epub 2016 Dec 30.
    From the Department of Genome Medicine Development (A.U., A.N., K.H., I.N.), Medical Genome Center, and Department of Neuromuscular Research (A.U., A.N., R.S.T., K.H., I.N.), National Institute of Neuroscience, National Center of Neurology and Psychiatry, Tokyo; Department of Education (A.N.), Interdisciplinary Graduate School of Medicine and Engineering, University of Yamanashi; Department of Pediatric Neurology (R.S.T.), National Hospital Organization, Utano National Hospital; Department of Neurology (K.H.), Graduate School of Medicine, Kyoto University; Department of Allergy and Rheumatology (M.K.), Nippon Medical School Graduate School of Medicine; and Division of Rheumatology (M.K.), Department of Internal Medicine, and Department of Neurology (Y.W., S.S., N.S.), Keio University School of Medicine, Tokyo, Japan.
    Objective: To evaluate the diagnostic value of myxovirus resistance A (MxA) expression in the cytoplasm of myofibers in the diagnosis of dermatomyositis (DM).

    Methods: We assessed the sensitivity and specificity of the sarcoplasmic expression of MxA in muscles with DM by immunohistochemistry in consecutive cases of DM (n = 34) and other idiopathic inflammatory myopathies (n = 120: 8 with polymyositis, 16 with anti-tRNA-synthetase antibody-associated myositis, 46 with immune-mediated necrotizing myopathy, and 50 with inclusion body myositis) and compared them with conventional pathologic hallmarks of DM, including perifascicular atrophy (PFA) and membrane attack complex (MAC) deposition on endomysial capillaries.

    Results: The sensitivity and specificity of sarcoplasmic MxA expression were 71% and 98%, respectively. Read More

    Sphingosine 1-Phosphate Receptor Modulators and Drug Discovery.
    Biomol Ther (Seoul) 2017 Jan;25(1):80-90
    Molecular Inflammation Research Center for Aging Intervention (MRCA) and College of Pharmacy, Pusan National University, Busan 46241, Republic of Korea.
    Initial discovery on sphingosine 1-phosphate (S1P) as an intracellular second messenger was faced unexpectedly with roles of S1P as a first messenger, which subsequently resulted in cloning of its G protein-coupled receptors, S1P₁₋₅. The molecular identification of S1P receptors opened up a new avenue for pathophysiological research on this lipid mediator. Cellular and molecular in vitro studies and in vivo studies on gene deficient mice have elucidated cellular signaling pathways and the pathophysiological meanings of S1P receptors. Read More

    The host defense peptide LL-37 a possible inducer of the type I interferon system in patients with polymyositis and dermatomyositis.
    J Autoimmun 2016 Dec 22. Epub 2016 Dec 22.
    Department of Medicine, Unit of Rheumatology, Karolinska Institutet, Karolinska University Hospital Solna, Stockholm, Sweden. Electronic address:
    The type I interferon (IFN) system has recently been suggested to play important and essential roles in the pathogenesis of myositis. However, a clarification of how type I IFNs could function as triggering factor(s) in the pathogenesis of myositis has yet failed. Through activation of the type I IFN system, the host defense peptide LL-37 carries numerous immunomodulatory properties and is implicated in the pathogenesis of several other autoimmune diseases, including systemic lupus erythematosus (SLE). Read More

    Association of anti-aminoacyl-transfer RNA synthetase antibody and anti-melanoma differentiation-associated gene 5 antibody with the therapeutic response of polymyositis/dermatomyositis-associated interstitial lung disease.
    Respir Investig 2017 Jan 30;55(1):24-32. Epub 2016 Sep 30.
    Division of Respirology, Neurology, and Rheumatology, Department of Internal Medicine, Kurume University School of Medicine, Asahi-machi 77, Kurume, Fukuoka 830-0011, Japan. Electronic address:
    Background: We attempted to clarify whether the presence of anti-aminoacyl-transfer RNA synthetase antibody (anti-ARS Ab) or anti-melanoma differentiation-associated gene 5 antibody (anti-MDA5 Ab) is associated with the therapeutic response of polymyositis/dermatomyositis-associated interstitial lung disease (PM/DM-ILD).

    Methods: We retrospectively investigated 22 patients with PM/DM-ILD (10 positive for anti-ARS Ab and nine positive for anti-MDA5 Ab) for whom antibody analysis of conserved serum was possible. We assessed mortality in the first three months as the therapeutic response in the acute phase and compared changes in clinical data for up to one year considered as the chronic phase. Read More

    Do dermatomyositis and polymyositis affect similar thigh muscles? A comparative MRI-based study.
    Clin Exp Rheumatol 2016 Nov-Dec;34(6):1098-1100. Epub 2016 Oct 27.
    Department of Rheumatology, Department of Internal Medicine, Azienda Ospedaliera ASMN, IRCSS, Reggio Emilia, Italy.
    Objectives: Dermatomyositis (DM) and polymyositis (PM) commonly cause weakness of the thigh muscles. However, it is debated whether DM and PM affect similar thigh muscles. Muscle oedema on fat-suppressed MRI sequences is thought to represent active inflammation. Read More

    Neutrophil-lymphocyte ratio and platelet-lymphocyte ratio are 2 new inflammatory markers associated with pulmonary involvement and disease activity in patients with dermatomyositis.
    Clin Chim Acta 2017 Feb 10;465:11-16. Epub 2016 Dec 10.
    Department of Clinical Laboratory, The Second Affiliated Hospital of Nanchang University, Nanchang 330006, China. Electronic address:
    Background: The neutrophil-lymphocyte ratio (NLR) and platelet-lymphocyte ratio (PLR) have emerged as useful biomarkers to predict systemic inflammation. However, there is no study to investigate the relationship between the biomarkers and dermatomyositis (DM).

    Methods: Seventy-three newly diagnosed patients with DM and 147 healthy subjects were selected in this retrospective study. Read More

    [Relapses in inflammatory myopathies].
    Medicina (B Aires) 2016 ;76(6):379-382
    Unidad de Alergia, Asma e Inmunología Clínica, Buenos Aires, Argentina.
    Most studies about treatment of inflammatory myopathies consist of cross-sectional analyses that do not assess long-term efficacy. In the present study we describe the follow-up of seven patients with inflammatory myopathies, 5 polymyositis and 2 dermatomyositis. We describe their clinical features, follow-up, muscle enzyme levels, and treatment responses. Read More

    Febuxostat-associated eosinophilic polymyositis in marginal zone lymphoma.
    Joint Bone Spine 2016 Dec 7. Epub 2016 Dec 7.
    Department of Rheumatology, Faculty of Medicine, Saint-Joseph university, Hotel-Dieu de France, Beirut, Lebanon.
    Febuxostat is an orally administered selective inhibitor of xanthine oxidase approved for the treatment of gout and prevention of tumor lysis syndrome. It is a relatively safe medication. Hypersensitivity reactions associated with the use of febuxostat are quite rare with only one reported case of DRESS syndrome. Read More

    High resolution manometry in patients with idiopathic inflammatory myopathy: Elevated prevalence of esophageal involvement and differences according to autoantibody status and clinical subset.
    Muscle Nerve 2016 Dec 9. Epub 2016 Dec 9.
    Autoimmune Systemic Diseases Unit (Department of Internal Medicine).
    Introduction: We studied the high-resolution manometry (HRM) findings in patients with dermatomyositis and polymyositis.

    Methods: From 2008 to 2015, we performed a cross-sectional study of myositis patients. A survey of esophageal symptoms and a HRM were analyzed and compared among different clinical and serologic groups. Read More

    Specific autoantibodies in dermatomyositis: a helpful tool to classify different clinical subsets.
    Arch Dermatol Res 2017 Mar 7;309(2):87-95. Epub 2016 Dec 7.
    Di.S.Sal. Section of Dermatology, IRCCS Azienda Ospedaliera Universitaria San Martino-IST, Via Pastore 1, 16132, Genoa, Italy.
    Autoantibodies are important in the diagnosis of dermatomyositis. They can be divided in two different groups: myositis-associated autoantibodies (MAA) prevailing in overlap syndromes, and myositis-specific autoantibodies (MSA), with diagnostic specificity exceeding 90%. Our purpose was to detect retrospectively the prevalence of the most common MSAs in a group of 19 adult DM patients (13 women, 6 men). Read More

    Autoimmune Myopathies.
    Continuum (Minneap Minn) 2016 Dec;22(6, Muscle and Neuromuscular Junction Disorders):1852-1870
    Purpose Of Review: This article provides guidelines for diagnosing and treating the different subtypes of autoimmune myopathies.

    Recent Findings: The most common subtypes of autoimmune myopathies are dermatomyositis, immune-mediated necrotizing myopathy, antisynthetase syndrome, and overlap syndromes; isolated polymyositis is an exceptionally rare disease. Specific autoantibodies are associated with unique clinical phenotypes and may be used for diagnostic and prognostic purposes, such as to assess the risk of coexisting malignancy. Read More

    [Autoantibodies of Inflammatory Myopathies: Update].
    Brain Nerve 2016 Dec;68(12):1443-1451
    Department of Neurology, Keio University School of Medicine.
    Inflammatory myopathies are a heterogeneous group of immune-mediated diseases that involve the skeletal muscle as well as many other organs. In addition to a histological diagnosis at muscle biopsy, the clinical phenotypes of inflammatory myopathies can be defined by the presence of various autoantibodies that are originally detected by RNA or protein immunoprecipitation. However, the correlation between histological features and autoantibodies has not been fully elucidated. Read More

    [Diagnosis of Idiopathic Inflammatory Myopathy: A Muscle Pathology Perspective].
    Brain Nerve 2016 Dec;68(12):1431-1441
    Department of Neuromuscular Research, National Institute of Neuroscience, National Center of Neurology and Psychiatry.
    Idiopathic inflammatory myopathies are historically classified into polymyositis and dermatomyositis based on the presence or absence of skin lesions. Recently, however, a more histology-oriented classification into 6 subtypes has been proposed. The subtypes include dermatomyositis, polymyositis, inclusion body myositis, immune-mediated necrotizing myopathy, anti-synthetase syndrome, and non-specific myositis. Read More

    A Negative Antinuclear Antibody Does Not Indicate Autoantibody Negativity in Myositis: Role of Anticytoplasmic Antibody as a Screening Test for Antisynthetase Syndrome.
    J Rheumatol 2017 Feb 1;44(2):223-229. Epub 2016 Dec 1.
    From the Division of Rheumatology and Clinical Immunology, University of Pittsburgh School of Medicine, Pittsburgh, Pennsylvania, USA.
    Objective: To evaluate the utility of anticytoplasmic autoantibody (anti-CytAb) in antisynthetase antibody-positive (anti-SynAb+) patients.

    Methods: Anti-SynAb+ patients were evaluated for antinuclear antibody (ANA) and anti-CytAb [cytoplasmic staining on indirect immunofluorescence (IIF)] positivity. Anti-SynAb+ patients included those possessing anti-Jo1 and other antisynthetase autoantibodies. Read More

    SPARC Interacts with Actin in Skeletal Muscle in Vitro and in Vivo.
    Am J Pathol 2017 Feb 29;187(2):457-474. Epub 2016 Nov 29.
    Department of Pathology, Institute of Clinical Research, University of Southern Denmark Muscle Research Cluster, University of Southern Denmark, Odense, Denmark.
    The cytoskeleton is an integral part of skeletal muscle structure, and reorganization of the cytoskeleton occurs during various modes of remodeling. We previously found that the extracellular matrix protein secreted protein acidic and rich in cysteine (SPARC) is up-regulated and expressed intracellularly in developing muscle, during regeneration and in myopathies, which together suggests that SPARC might serve a specific role within muscle cells. Using co-immunoprecipitation combined with mass spectrometry and verified by staining for direct protein-protein interaction, we find that SPARC binds to actin. Read More

    Critical review of the role of intravenous immunoglobulins in idiopathic inflammatory myopathies.
    Semin Arthritis Rheum 2016 Jul 29. Epub 2016 Jul 29.
    Lady Davis Institute for Medical Research, Jewish General Hospital, Montreal, Quebec, Canada; Department of Medicine, Jewish General Hospital, Montreal, Quebec, Canada; Faculty of Medicine, McGill University, Montreal, Quebec, Canada. Electronic address:
    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. Original and review articles were retrieved for full-text review. Read More

    Calcinosis in poly-dermatomyositis: clinical and laboratory predictors and treatment options.
    Clin Exp Rheumatol 2016 Nov 14. Epub 2016 Nov 14.
    Rheumatology and Clinical Immunology Unit, Rheumatology Chair, Spedali Civili, Brescia, Italy.
    Objectives: We aimed to identify the possible clinical and laboratory predictors of calcinosis in a cohort of patients with a diagnosis of polymyositis (PM) and dermatomyositis (DM).

    Methods: We carried out a retrospective analysis of a cohort of myositis patients attending our clinic between January 2013 and May 2014.

    Results: 74 patients (58 females, 16 males) with PM (30 cases), DM (30 cases), overlap syndrome (13 cases) and inclusion body myositis (1 case) were enrolled. Read More

    Long-term clinical course of anti-glycyl tRNA synthetase (anti-EJ) antibody-related interstitial lung disease pathologically proven by surgical lung biopsy.
    BMC Pulm Med 2016 Dec 1;16(1):168. Epub 2016 Dec 1.
    Department of Respiratory Medicine, Kanagawa Cardiovascular and Respiratory Center, 6-16-1 Tomioka-higashi, Kanazawa-Ku, Yokohama, 236-0051, Japan.
    Background: Anti-glycyl-tRNA synthetase (anti-EJ) antibody is occasionally positive in patients with interstitial lung disease (ILD). We aimed to define the clinical, radiological and pathological features of patients with anti-EJ antibody-positive ILD (EJ-ILD).

    Methods: We retrospectively analyzed the medical records of 12 consecutive patients with EJ-ILD who underwent surgical lung biopsy. Read More

    The sphingosine-1-phosphate receptor: A novel therapeutic target for multiple sclerosis and other autoimmune diseases.
    Clin Immunol 2017 Feb 23;175:10-15. Epub 2016 Nov 23.
    Department of Neurology and Clinical Autoimmunity Center of Excellence, University of Michigan Medical School, 4015 Alfred Taubman Biomedical Sciences Research Bldg., 109 Zina Pitcher Place, Ann Arbor, MI 48109-2200, United States.
    Multiple sclerosis (MS) is a prototype autoimmune disease of the central nervous system (CNS). Currently, there is no drug that provides a cure for MS. To date, all immunotherapeutic drugs target relapsing remitting MS (RR-MS); it remains a daunting medical challenge in MS to develop therapy for secondary progressive MS (SP-MS). Read More

    MiR-146a Regulates Inflammatory Infiltration by Macrophages in Polymyositis/Dermatomyositis by Targeting TRAF6 and Affecting IL-17/ICAM-1 Pathway.
    Cell Physiol Biochem 2016 25;40(3-4):486-498. Epub 2016 Nov 25.
    Cancer Institute, First Affiliated Hospital, China Medical University, Shenyang, China.
    Background/aims: The primary objective of this study was to investigate the role of miR-146a in inducing the inflammatory infiltration of macrophages in polymyositis/dermatomyositis (PM/DM) through targeting TNF receptor associated factor 6 (TRAF6), which may further down-regulate the Interleukin-17 (IL-17)/Intercellular Adhesion Molecule 1 (ICAM-1) pathway.

    Methods: Biopsies were collected from PM/DM patients and healthy volunteers. PM/DM model establishment and macrophage isolation were performed on Sprague Dawley (SD) rats. Read More

    Comprehensive assessment of myositis-specific autoantibodies in polymyositis/dermatomyositis-associated interstitial lung disease.
    Respir Med 2016 Dec 2;121:91-99. Epub 2016 Nov 2.
    Second Division, Department of Internal Medicine, Hamamatsu University School of Medicine, 1-20-1 Handayama Higashiku, Hamamatsu, Shizuoka 431-3192, Japan.
    Objectives: Myositis-specific autoantibodies (MSAs) are associated with clinical phenotypes in polymyositis/dermatomyositis (PM/DM). No study has investigated the clinical features based on comprehensive MSA assessment in PM/DM-associated interstitial lung disease (ILD). We aimed to determine the practical significance of MSAs in PM/DM-ILD. Read More

    Myocardial perfusion in peripheral Raynaud's phenomenon. Evaluation using stress cardiovascular magnetic resonance.
    Int J Cardiol 2017 Feb 12;228:444-448. Epub 2016 Nov 12.
    Onassis Cardiac Surgery Center, Athens, Greece.
    Background: Peripheral Raynaud's phenomenon (RP) is either primary (PRP), without any coexisting disease or secondary (SRP), due to connective tissue diseases (CTD). We hypothesized that adenosine stress cardiovascular magnetic resonance (CMR) can assess myocardial perfusion in a population of PRP and SRP.

    Patients-methods: Twenty CTDs, aged 30. Read More

    Analysis of sexual function of patients with dermatomyositis and polymyositis through self-administered questionnaires: a cross-sectional study.
    Rev Bras Reumatol 2016 Nov 1. Epub 2016 Nov 1.
    Universidade de São Paulo, Faculdade de Medicina, Disciplina de Reumatologia, São Paulo, SP, Brasil. 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

    Anti-HMGCR antibodies in systemic sclerosis.
    Medicine (Baltimore) 2016 Nov;95(44):e5280
    aDepartment of Medicine, McGill UniversitybDivision of RheumatologycLady Davis Institute, Jewish General Hospital, Montréal, QuebecdCumming School of Medicine, University of Calgary, Calgary, Alberta, Canada.
    The objective of this study was to investigate the frequency of autoantibodies to hydroxymethylglutaryl coenzyme A reductase (HMGCR) in systemic sclerosis (SSc) and associations with inflammatory myositis and statin exposure.This was a cross-sectional, multicenter study of 306 subjects from the Canadian Scleroderma Research Group cohort who had complete data on statin exposure and serology for anti-HMGCR antibodies assayed by an addressable laser bead immunoassay (ALBIA). Descriptive statistics were used to summarize the baseline characteristics of the patients and to compare subjects with and without anti-HMGCR antibodies. Read More

    Development and evaluation of a standardized ELISA for the determination of autoantibodies against cN-1A (Mup44, NT5C1A) in sporadic inclusion body myositis.
    Auto Immun Highlights 2016 Dec 17;7(1):16. Epub 2016 Nov 17.
    Institute for Experimental Immunology, Euroimmun AG, Seekamp 31, 23560, Lübeck, Germany.
    Purpose: Sporadic inclusion body myositis (sIBM) is an autoimmune degenerative disease of the muscle, with inflammatory infiltrates and inclusion vacuoles. Its pathogenesis is not fully understood and the diagnosis is hampered by its imprecise characteristics, at times indistinguishable from other idiopathic inflammatory myopathies such as polymyositis and dermatomyositis. The diagnosis may be assisted by the detection of autoantibodies targeting Mup44, a skeletal muscle antigen identified as cytosolic 5'-nucleotidase 1A (cN-1A, NT5C1A). Read More

    Prognostic factors of idiopathic inflammatory myopathies complicated with interstitial lung disease: protocol for a systematic review and meta-analysis.
    BMJ Open 2016 Nov 17;6(11):e012744. Epub 2016 Nov 17.
    Faculty of Engineering, Department of Management Science, Tokyo University of Science, Tokyo, Japan.
    Introduction: Idiopathic inflammatory myopathies may be an overlapping disease complex. Although interstitial lung disease affects the mortality and the morbidity of the disease, a clinical course and the prognosis of the disease complicated with interstitial lung disease are diverse among individuals and prognostic factors have yet to be clarified. This article aims to report the rationale and the methodology of a future intended systematic review and meta-analysis of prognostic factors of idiopathic inflammatory myopathies complicated with interstitial lung disease. Read More

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