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    Effective Use of Calcineurin Inhibitor in Combination Therapy for Interstitial Lung Disease in Patients With Dermatomyositis and Polymyositis.
    J Clin Rheumatol 2017 Mar;23(2):87-93
    From the Department of Medicine (Neurology & Rheumatology), Shinshu University School of Medicine, Matsumoto, Japan.
    Objective: The current study demonstrated the potential use of calcineurin inhibitor (CNI) in combination therapy for interstitial lung disease (ILD) complicated with dermatomyositis (DM) and polymyositis (PM).

    Methods: Thirty DM/PM patients with ILD were enrolled in this study. Continuous intravenous administration of cyclosporine A (IV-CsA) was simultaneously started with corticosteroid in patients presenting more than two respiratory distress factors as follows: <70 mmHg of PaO2, percentage of vital capacity <70%, and/or exertional dyspnea. Read More

    Infections and Vaccinations as Possible Triggers of Inflammatory Myopathies.
    Muscle Nerve 2017 Feb 22. Epub 2017 Feb 22.
    SA Pathology, Frome Rd, Adelaide, SA, 5000, Australia.
    Introduction: The role of vaccinations and infections in triggering idiopathic inflammatory myopathies (IIM) has not been confirmed.

    Methods: Among patients with histologically-confirmed myositis, infections or vaccinations administered prior to myositis onset were determined. The characteristics of this group were compared with controls (myositis patients without prior infection or vaccination). Read More

    Human herpes virus-6 encephalitis causing severe anterograde amnesia associated with rituximab, azathioprine and prednisolone combination therapy for dermatomyositis.
    J Neurovirol 2017 Feb 21. Epub 2017 Feb 21.
    Department of Neurosciences, North Bristol NHS Trust, Southmead Hospital, Bristol, UK.
    Human herpes virus-6 (HHV-6) reactivation is a well-recognised complication following haematological stem cell transplantation, but it is novel in the context of combination immunomodulatory therapy for autoimmune disease. We report a case of severe anterograde amnesia caused by HHV-6 encephalitis in a young female patient on rituximab, azathioprine and prednisolone for dermatomyositis (DM). The use of targeted biologic treatments for systemic autoimmune connective tissue diseases (CTDs) is increasing, particularly when refractory to conventional management. Read More

    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

    Paraneoplastic Dermatomyositis with Cutaneous and Myopathic Disease Responsive to Adrenocorticotropic Hormone Therapy.
    J Clin Aesthet Dermatol 2017 Jan 1;10(1):57-62. Epub 2017 Jan 1.
    St. Barnabas Hospital Dermatology Residency, Bronx, New York;; Mount Sinai School of Medicine Manhattan, New York.
    Dermatomyositis is a myopathic or amyopathic autoimmune connective tissue disease that presents with classic dermatologic findings ranging from: poikilodermatous photosensitivity (shawl sign), eyelid edema and violaceous-pigmentation (heliotrope sign), lichenoid eruptions on the knuckles and elbows (Gottron's sign), periungual telangiectasias, and ragged cuticles (Samitz sign). Up to 30 percent of adult-onset cases of dermatomyositis may represent a paraneoplastic syndrome warranting a thorough work-up for malignancy. The authors present a case report of paraneoplastic dermatomyositis associated with triple negative, BRCA-1 positive, invasive intraductal carcinoma of the breast, whose myopathic and cuteanous symptoms were recalcitrant to high-dose corticosteroid therapy. 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

    Acute Presentation of Juvenile Dermatomyositis with Subclinical Cardiac Involvement: A Rare Case.
    J Clin Diagn Res 2016 Dec 1;10(12):SD01-SD03. Epub 2016 Dec 1.
    Junior Resident, Department of Pediatrics, Sarojini Naidu Medical College , Agra, Uttar Pradesh, India .
    Cardiac involvements are common in patients with Dermatomyositis, most of which are not severe enough to present definite or readily observable symptoms. However, Cardiovascular (CVS) manifestations constitute a major cause of death in these patients. The most frequently reported clinically evident of CVS manifestations in-patient of dermatomyositis are Congestive Heart Failure (CHF), conduction aberrations, that may predispose to complete heart block and coronary artery disease. Read More

    Recurrent Multiple Squamous Cell Carcinomas on the Scalp in a Patient with Juvenile Dermatomyositis.
    Case Rep Oncol 2017 Jan-Apr;10(1):106-111. Epub 2017 Jan 20.
    Department of Dermatology, Tohoku University Graduate School of Medicine, Sendai, Japan.
    Both long-term administration of immunosuppressive agents and chronic inflammatory conditions, such as autoimmune disease, could be risk factors for the development of cutaneous squamous cell carcinoma (cSCC). In this report, we present a case of recurrent multiple cSCC on the scalp in a patient with juvenile dermatomyositis who had been administered cyclosporine and Predonine since she was a 1-year-old infant. Interestingly, immunohistochemical staining revealed IL-17-producing cells adjacent to IL-17R-expressing atypical keratinocytes. Read More

    Anti-HMGCR Autoantibodies in Juvenile Idiopathic Inflammatory Myopathies Identify a Rare but Clinically Important Subset of Patients.
    J Rheumatol 2017 Feb 15. Epub 2017 Feb 15.
    From the Royal National Hospital for Rheumatic Diseases, Royal United Hospitals Foundation Trust; University of Bath, Bath; University College London (UCL) Institute of Child Health; Arthritis Research UK Centre for Adolescent Rheumatology at UCL, University College London Hospitals (UCLH), and Great Ormond Street Hospital (GOSH), London; Leeds Children's Hospital, Leeds; Nottingham University Hospitals UK National Health Service (NHS) Trust, Nottingham, UK. Funding for the UK Juvenile Dermatomyositis Cohort and Biomarker study has been provided by the Wellcome Trust UK (085860), Action Medical Research UK (SP4252), The Myositis Support Group UK, Arthritis Research UK (14518, 20164), The Henry Smith Charity and Great Ormond Street Children's Charity (V1268), and the National Institute for Health Research (NIHR) Translational Research Collaboration Rare Diseases. This research was supported by the NIHR Biomedical Research Centre at GOSH for Children NHS Foundation Trust and Institute of Child Health UCL. SLT is supported by a fellowship from The Bath Institute for Rheumatic Diseases and has received previous support from the BMA Doris Hillier Grant (2012). S.L. Tansley, MBChB, MRCP, Royal National Hospital for Rheumatic Diseases, Royal United Hospitals Foundation Trust, and University of Bath; Z.E. Betteridge, BSc, PhD, University of Bath; S. Simou, MSc, UCL Institute of Child Health; T.S. Jacques, PhD, MRCP, FRCPath, UCL Institute of Child Health, and GOSH; C. Pilkington, MBBS, FRCPCH, FRCP, GOSH; M. Wood, MBBS, MRCPCH, Leeds Children's Hospital; K. Warrier, MBBS, MRCPCH, Nottingham University Hospitals NHS Trust; L.R. Wedderburn, MD, PhD, FRCP, UCL Institute of Child Health, and GOSH, and Arthritis Research UK Centre for Adolescent Rheumatology at UCL, UCLH, and GOSH; N.J. McHugh, MD, FRCP, FRCPath, Royal National Hospital for Rheumatic Diseases, Royal United Hospitals Foundation Trust, and University of Bath. Address correspondence to Dr. S.L. Tansley, Royal National Hospital For Rheumatic Diseases NHS Foundation Trust, Upper Borough Walls, Bath BA1 1RL, UK. E-mail: Accepted for publication December 23, 2016.
    Objective: We aimed to establish the prevalence and clinical associations of anti-HMG-CoA-reductase (anti-HMGCR) in a large UK cohort with juvenile myositis.

    Methods: There were 381 patients investigated for anti-HMGCR using ELISA.

    Results: Anti-HMGCR autoantibodies were detected in 4 patients (1%). 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

    Dermatomyositis Leading to Necrotizing Vasculitis: A Perfect Response to Applied Therapy.
    Int J Biomed Sci 2016 Dec;12(4):125-129
    Fellowship of Rheumatology, Rheumatologic Research Center, Tehran University of Medical Science, Tehran, Iran.
    Dermatomyositis is an idiopathic inflammatory myopathy that cause skin and muscle complications. The ethiology is not understood well yet. Released cytokines including interferon and interleukins are suggested to make inflammatory responses in the skin or muscle. Read More

    Cardiovascular magnetic resonance imaging pattern at the time of diagnosis of treatment naïve patients with connective tissue diseases.
    Int J Cardiol 2017 Feb 1. Epub 2017 Feb 1.
    Onassis Cardiac Surgery Center, Athens, Greece.
    Background-aim: Cardiac involvement at diagnosis of connective tissue disease (CTD) has been described by echocardiography. We hypothesized that cardio-vascular magnetic resonance (CMR) detects occult lesions at CTD diagnosis.

    Patients-methods: CMR was performed early after diagnosis in 78 treatment-naïve CTDs (aged 43±11, 59F/19M) without cardiac involvement [5 Takayasu arteritis (TA), 4 Churg Strauss syndrome (CSS), 5 Wegener granulomatosis (WG), 16 systemic lupus erythematosus (SLE), 12 rheumatoid arthritis (RA), 8 mixed connective tissue diseases (MCTD), 12 ankylosing spondylitis (AS), 3 polymyalgia rheumatica (PMR), 8 systemic sclerosis (SSc) and 5 dermatomyositis (DM)]. 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

    Juvenile dermatomyositis in a 4-year-old Kenyan girl.
    Clin Case Rep 2017 Feb 17;5(2):134-138. Epub 2017 Jan 17.
    Department of Internal Medicine Kilimanjaro Christian Medical Centre Moshi United Republic of Tanzania.
    To our knowledge, this is the first case report of juvenile dermatomyositis (JDM) in Tanzania. It demonstrates that the characteristic cutaneous findings of JDM may easily be overlooked, especially on dark skin, and the difficulty of clinical management in resource-constrained settings. Read More

    A Report of Two Cases of Solid Facial Edema in Acne.
    Dermatol Ther (Heidelb) 2017 Feb 6. Epub 2017 Feb 6.
    Department of Dermatology, University Hospital Zurich, Zurich, Switzerland.
    Introduction: Solid facial edema (SFE) is a rare complication of acne vulgaris. To examine the clinical features of acne patients with solid facial edema, and to give an overview on the outcome of previous topical and systemic treatments in the cases so far published.

    Methods: We report two cases from Switzerland, both young men with initially papulopustular acne resistant to topical retinoids. Read More

    Beyond the MHC: A canine model of dermatomyositis shows a complex pattern of genetic risk involving novel loci.
    PLoS Genet 2017 Feb 3;13(2):e1006604. Epub 2017 Feb 3.
    Department of Genetics and Biochemistry, Clemson University, Clemson, South Carolina, United States of America.
    Juvenile dermatomyositis (JDM) is a chronic inflammatory myopathy and vasculopathy driven by genetic and environmental influences. Here, we investigated the genetic underpinnings of an analogous, spontaneous disease of dogs also termed dermatomyositis (DMS). As in JDM, we observed a significant association with a haplotype of the major histocompatibility complex (MHC) (DLA-DRB1*002:01/-DQA1*009:01/-DQB1*001:01), particularly in homozygosity (P-val = 0. Read More

    An Atypical Clinical Course of Anti-MDA5 Antibody-positive Interstitial Lung Disease in a Patient with Three Deteriorations in 9 years.
    Intern Med 2017 1;56(3):341-346. Epub 2017 Feb 1.
    Department of Respiratory Medicine, Kobe City Medical Center General Hospital, Japan.
    Anti-MDA5 antibody-positive patients with clinically amyopathic dermatomyositis (CADM) are at high risk of developing rapidly progressive interstitial lung disease (ILD), which is associated with a high mortality rate. Approximately half of the patients with ILD recover; however, the long-term clinical course of these patients has not been fully reported and is not completely understood. This report describes the atypical clinical course of an anti-MDA5 antibody-positive CADM patient who experienced three deteriorations of ILD in 9 years. 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

    Contraception for adolescents with chronic rheumatic diseases.
    Rev Bras Reumatol Engl Ed 2017 Jan - Feb;57(1):73-81. Epub 2016 Aug 6.
    Universidade de São Paulo (USP), Faculdade de Medicina, Unidade do Adolescente, São Paulo, SP, Brazil; Universidade de São Paulo (USP), Faculdade de Medicina, Unidade de Reumatologia Pediátrica, São Paulo, SP, Brazil; Universidade de São Paulo (USP), Faculdade de Medicina, Divisão de Reumatologia, São Paulo, SP, Brazil.
    Contraception is an important issue and should be a matter of concern in every medical visit of adolescent and young patients with chronic rheumatic diseases. This narrative review discusses contraception methods in adolescents with juvenile systemic lupus erythematosus (JSLE), antiphospholipid syndrome (APS), juvenile idiopathic arthritis (JIA) and juvenile dermatomyositis (JDM). Barrier methods are safe and their use should be encouraged for all adolescents with chronic rheumatic diseases. Read More

    The family impact of caring for a child with juvenile dermatomyositis.
    Chronic Illn 2017 Jan 1:1742395317690034. Epub 2017 Jan 1.
    Pacific Graduate School of Psychology, Palo Alto University, Palo Alto, CA, USA.
    Background Juvenile dermatomyositis (JDM), a rare autoimmune disease, accounts for more than 80% of idiopathic inflammatory myopathy childhood cases, making it the most common idiopathic inflammatory myopathy among children. The average age of onset is approximately 7 years and commonly leads a chronic course. Symptoms of JDM include cutaneous features (Gottron's rash, heliotrope rash, or nail fold capillary changes), musculoskeletal features, calcinosis and lipodystrophy (a symmetrical deficit of subcutaneous fatty tissue), and acanthosis (thickening of the skin). Read More

    [A Case of Rectal Metastasis of Gastric Cancer Associated with Dermatomyositis].
    Gan To Kagaku Ryoho 2016 Nov;43(12):2401-2403
    Dept. of Surgery, National Hospital Organization Osaka National Hospital.
    We report a case of rectal metastasis of gastric cancer associated with dermatomyositis showing paraneoplastic syndrome. The patient was a 70-year-old man who had undergone curative total gastrectomy for Stage III Agastric cancer in March 2005. He was diagnosed with dermatomyositis and treated with prednisolone after gastrectomy. Read More

    Panniculitis: A summary.
    Semin Diagn Pathol 2016 Dec 27. Epub 2016 Dec 27.
    Section of Dermatopathology,Dermatopathology, Division of Surgical Pathology & Cytopathology, University of Virginia Medical Center, Charlottesville, VA, USA. Electronic address:
    The diagnosis of panniculitis is felt to be a confusing topic by some pathologists. This summarical article presents inflammatory diseases of the subcutis in a systematic fashion, based on whether they are centered on fibrovascular septa or the adipose lobules, and whether morphologic vasculitis is present or not. Septocentric, non-vasculitis disorders include erythema nodosum, panniculitis that follows the use of "biological" therapeutic agents, lipodermatosclerosis, post-irradiation panniculitis, morphea profunda, and necrobiosis lipodica profunda. Read More

    The cutaneous and systemic findings associated with nuclear matrix protein-2 antibodies in adult dermatomyositis patients.
    Arthritis Care Res (Hoboken) 2017 Jan 27. Epub 2017 Jan 27.
    Department of Dermatology, Stanford University School of Medicine, Stanford, CA.
    Objective: To characterize the cutaneous and systemic clinical phenotype of dermatomyositis patients with anti-NXP-2 antibodies.

    Methods: We conducted a retrospective cohort analysis of 178 dermatomyositis patients seen at the Stanford University Clinic. Electronic chart review employing a keyword search strategy was performed to collect clinical and laboratory data. Read More

    Anti-3-Hydroxy-3-Methylglutaryl-Coenzyme A Reductase Autoantibodies are Associated with DRB1*07:01 and Severe Myositis in Pediatric Myositis Patients.
    Arthritis Care Res (Hoboken) 2017 Jan 27. Epub 2017 Jan 27.
    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, MD.
    Objective: Autoantibodies recognizing 3-hydroxy-3-methylglutaryl-coenzyme A reductase (HMGCR) are associated with statin exposure, the HLA allele DRB1*11:01, and necrotizing muscle biopsies in adult myositis patients. The aim of this study was to characterize the features of pediatric anti-HMGCR-positive myositis patients.

    Methods: The sera of 440 juvenile myositis patients were screened for anti-HMGCR autoantibodies. Read More

    Fibrocellular non-specific interstitial pneumonia with favourable prognosis in clinically amyopathic dermatomyositis.
    Respirol Case Rep 2016 Sep 9;4(5):e00180. Epub 2016 Aug 9.
    Department of Respiratory Medicine Toho University Omori Medical Center Tokyo Japan.
    We report the case of a 49-year-old man who experienced dry cough and itching eruption for 2 months. The patient had Gottoron's sign, mechanic's hand, and high levels of serum Krebs von den Lungen-6 and surfactant protein D, but was negative for anti-aminoacyl transfer RNA synthetase antibodies. High-resolution chest computed tomography revealed subpleural band-like or patchy consolidation with predominance in the bilateral lower lobe. 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

    A Rare Case of Amyopathic Juvenile Dermatomyositis Associated With Psoriasis Successfully Treated With Ustekinumab.
    J Clin Rheumatol 2017 Mar;23(2):129-130
    Departments of Dermatology, Fundación Valle del Lili, School of Medicine, Icesi University, Cali, Colombia. Departments of Rheumatology, Fundación Valle del Lili, School of Medicine, Icesi University, Cali, Colombia.

    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

    Antimelanoma Differentiation-associated Gene 5 Antibody: Expanding the Clinical Spectrum in North American Patients with Dermatomyositis.
    J Rheumatol 2017 Jan 15. Epub 2017 Jan 15.
    From the Myositis Center and Division of Rheumatology and Clinical Immunology, Department of Medicine, University of Pittsburgh School of Medicine, Pittsburgh, Pennsylvania, USA; Division of Rheumatology, Department of Internal Medicine, Tokai University, School of Medicine, Isehara; Department of Allergy and Rheumatology, Nippon Medical School Graduate School of Medicine, Tokyo, Japan. Drs. Kuwana and Sato hold a patent on an anti-MDA5 ELISA kit. S. Moghadam-Kia, MPH, MSc, Myositis Center and Division of Rheumatology and Clinical Immunology, Department of Medicine, University of Pittsburgh School of Medicine; C.V. Oddis, MD, Myositis Center and Division of Rheumatology and Clinical Immunology, Department of Medicine, University of Pittsburgh School of Medicine; S. Sato, MD, Division of Rheumatology, Department of Internal Medicine, Tokai University, School of Medicine; M. Kuwana, MD, Department of Allergy and Rheumatology, Nippon Medical School Graduate School of Medicine; R. Aggarwal, MD, MS, Myositis Center and Division of Rheumatology and Clinical Immunology, Department of Medicine, University of Pittsburgh School of Medicine. Address correspondence to Dr. R. Aggarwal, Associate Professor of Medicine, Division of Rheumatology and Clinical Immunology, Department of Medicine, University of Pittsburgh, 3601 5th Avenue, Suite 2B, Pittsburgh, Pennsylvania 15261, USA. E-mail: Accepted for publication November 9, 2016.
    Objective: To determine the clinical features associated with the antimelanoma differentiation-associated gene 5 antibody (anti-MDA5) in US patients with clinically amyopathic dermatomyositis (CADM) and classic DM.

    Methods: Patients with CADM were consecutively selected from the University of Pittsburgh Myositis Database from 1985 to 2013. CADM was defined by a typical DM rash without objective muscle weakness and no or minimal abnormalities of muscle enzymes, electromyography, or muscle biopsy. 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

    Dermatomyositis Patients with Anti-Nuclear Matrix Protein-2 Autoantibodies Have More Edema, More Severe Muscle Disease, and Increased Malignancy Risk.
    Arthritis Care Res (Hoboken) 2017 Jan 13. Epub 2017 Jan 13.
    Johns Hopkins University School of Medicine, Baltimore, MD.
    Objectives: Dermatomyositis (DM) patients typically present with proximal weakness and autoantibodies that are associated with distinct clinical phenotypes. We observed that DM patients with autoantibodies recognizing the nuclear matrix protein NXP-2 often presented with especially severe weakness. The aim of this study was to characterize clinical features associated with anti-NXP-2 autoantibodies. Read More

    Intravenous Immunoglobulin in Pediatric Rheumatology: When to Use It and What Is the Evidence.
    Pediatr Ann 2017 Jan;46(1):e19-e24
    Intravenous immunoglobulin (IVIG) is given to children with a variety of rheumatologic illnesses. The mechanism of action by which it exerts therapeutic effects is not well understood and likely differs in the medical conditions for which it is given. IVIG is approved by the US Food and Drug Administration and is the standard of care for Kawasaki disease, but most IVIG use in pediatric rheumatology is "off-label. Read More

    Anti-MDA5 Antibody Dermatomyositis Overlap with Systemic Lupus Erythematosus: A Case Report and Review of the Literature.
    Open Rheumatol J 2016 30;10:122-128. Epub 2016 Nov 30.
    Ronald O. Perelman Department of Dermatology, New York University School of Medicine, New York, USA; Department of Medicine, Division of Rheumatology, New York University School of Medicine, New York, USA.
    Background: Dermatomyositis (DM) is an autoimmune connective tissue disease that primarily targets the muscle, skin, and lungs. Many patients have autoantibodies that correspond to distinct clinical phenotypes. Melanoma differentiation-associated gene 5 (anti-MDA5) antibody, a specific antibody that targets the melanoma differentiation-associated gene 5 (MDA5), has been reported in DM cases and is significant for a distinct cutaneous presentation and rapidly progressive interstitial lung disease. Read More

    Childhood Arthritis and Rheumatology Research Alliance consensus clinical treatment plans for juvenile dermatomyositis with skin predominant disease.
    Pediatr Rheumatol Online J 2017 Jan 11;15(1). Epub 2017 Jan 11.
    Department of Pediatrics, IWK Health Centre and Dalhousie University, 5980 University Ave, Halifax, NS, Canada.
    Background: Juvenile dermatomyositis (JDM) is the most common form of the idiopathic inflammatory myopathies in children. A subset of children have the rash of JDM without significant weakness, and the optimal treatments for these children are unknown. The goal of this study was to describe the development of consensus clinical treatment plans (CTPs) for children with JDM who have active skin rashes, without significant muscle involvement, referred to as skin predominant JDM in this manuscript. 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

    Serum adipocytokine profile and metabolic syndrome in young adult female dermatomyositis patients.
    Clinics (Sao Paulo) 2016 Dec 1;71(12):709-714. Epub 2016 Dec 1.
    Faculdade de Medicina da Universidade de São Paulo, Clínica Médica, Reumatologia, São Paulo/SP, Brazil.
    Objectives:: To analyse the frequency of metabolic syndrome in young adult female dermatomyositis patients and its possible association with clinical and laboratory dermatomyositis-related features and serum adipocytokines.

    Method:: This cross-sectional study included 35 dermatomyositis patients and 48 healthy controls. Metabolic syndrome was defined according to the 2009 Joint Interim Statement. Read More

    Establishment of a New Conditionally Immortalized Human Skeletal Muscle Microvascular Endothelial Cell Line.
    J Cell Physiol 2017 Jan 7. Epub 2017 Jan 7.
    Department of Neurology and Clinical Neuroscience, Yamaguchi University Graduate School of Medicine, 1-1-1 Minami-kogushi, Ube, Yamaguchi, 7558505, Japan.
    In skeletal muscle, the capillaries have tight junctions (TJs) that are structurally similar to those in the blood-brain barrier (BBB) and blood-nerve barrier (BNB). Although many findings have been clarified in the territory of BBB and BNB, few have so far examined the TJs of capillaries in the skeletal muscle. In addition, no in vitro human skeletal muscle microvasculature models have been reported thus far. Read More

    Juvenile dermatomyositis: a tertiary center experience.
    Clin Rheumatol 2017 Feb 5;36(2):361-366. Epub 2017 Jan 5.
    Department of Pediatric Rheumatology, Cerrahpasa Medical School, Istanbul University, Istanbul, Turkey.
    Juvenile dermatomyositis (JDM) is a rare chronic inflammatory disease of unknown etiology and primarily involves muscle and skin. It is the most common idiopathic inflammatory myopathy of childhood. This study aimed to evaluate demographic and clinical features, laboratory data, treatment modalities, and outcome of patients with JDM at a referral pediatric rheumatology center in Turkey. Read More

    Successful resection of liver metastasis detected by exacerbation of skin symptom in a patient with dermatomyositis accompanied by rectal cancer: a case report and literature review.
    Surg Case Rep 2017 Dec 4;3(1). Epub 2017 Jan 4.
    Department of Surgery, National Hospital Organization Higashihiroshima Medical Center, 513, Jike, Saijyo-cho, Higashihiroshima, Hiroshima, 739-0041, Japan.
    Background: Dermatomyositis (DM) is a rare syndrome that belongs to the group of idiopathic inflammatory myopathies. The association between DM and malignancy is well recognized, and the severity of DM symptoms has been linked to the progression of metastatic disease.

    Case Presentation: We report the case of a 42-year-old man that was diagnosed with dermatomyositis (DM) and rectal cancer. Read More

    Recurrence of juvenile dermatomyositis 8 years after remission.
    JAAD Case Rep 2017 Jan 26;3(1):29-32. Epub 2016 Dec 26.
    Center for Pediatric Allergy and Rheumatology, KKR Sapporo Medical Center, Sapporo, Japan; Department of Pediatrics, Hokkaido University Graduate School of Medicine, Sapporo, Japan.

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