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    Treatment of Juvenile Dermatomyositis: An Update.
    Paediatr Drugs 2017 May 26. Epub 2017 May 26.
    UCL Great Ormond Street Institute of Child Health, 30 Guilford St, London, WC1N 1EH, UK.
    The idiopathic inflammatory myopathies of childhood consist of a heterogeneous group of autoimmune diseases characterised by proximal muscle weakness and pathognomonic skin rashes. The overall prognosis of juvenile myositis has improved significantly over recent years, but the long-term outcome differs substantially from patient to patient, suggestive of distinct clinical phenotypes with variable responses to treatment. High doses of corticosteroids remain the cornerstone of therapy along with other immunosuppressant therapies depending on disease severity and response. Read More

    Clinically amyopathic dermatomyositis with rapidly progressive interstitial pneumonia: The relation between the disease activity and the serum interleukin-6 level.
    J Dermatol 2017 May 23. Epub 2017 May 23.
    Department of Dermatology, Wakayama Medical University Graduate School of Medicine, Wakayama, Japan.
    A 67-year-old woman exhibited chilblain-like erythema on the bilateral auricles, erythema with hyperkeratosis on the sides of fingers and the bilateral elbows, and erythema on the extensor/flexor sides of the finger joints and the iliac regions. Fine crackles were audible on the dorsal side of the bilateral lower lung regions. We diagnosed clinically amyopathic dermatomyositis accompanied by rapidly progressive interstitial pneumonia. Read More

    The pathogenesis of dermatomyositis.
    Br J Dermatol 2017 May 24. Epub 2017 May 24.
    Department of Rheumatology, University Hospital of Wales, Cardiff, U.K.
    This review looks at the many different factors thought to play a role in idiopathic inflammatory myopathies (IIM), concentrating mainly on the dermatomyositis (DM) subtype. Subject areas addressed include looking at the different clinical features of IIM, paying particular attention to the skin manifestations. There is a discussion around investigations needed with their perceived value, followed by a description of the immunohistochemical findings of DM. Read More

    What's in the Literature?
    J Clin Neuromuscul Dis 2017 Jun;18(4):246-259
    *Department of Neurology, University of Utah School of Medicine, Salt Lake City, UT; †Department of Neurology, University at Buffalo Jacobs School of Medicine and Biomedical Sciences, SUNY, Buffalo, NY; and Departments of ‡Neurology and §Pathology, University of Pittsburgh School of Medicine, Pittsburgh, PA.
    In this edition, we provide a detailed summary of an informative book, "GBS100: Celebrating a Century of Progress in Guillain-Barré Syndrome" developed by the Peripheral Nerve Society to honor the centenary of the original paper on Guillain-Barré Syndrome. We also review various studies in myasthenia gravis including: management with rituximab; the efficacy of early fast-acting treatment with corticosteroids; and various dosing strategies for tacrolimus. Finally, we review new studies including: the potential pathogenesis, risk factors, and functional decline of patients with inclusion body myositis; MxA immunoreactivity in dermatomyositis; diagnostic approaches for evaluating patients with myalgia, fatigue, and exercise intolerance; MRI patterns in genetic muscle disease; and MRI as an outcome measure in facioscapulohumeral muscular dystrophy. Read More

    [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

    Association between demyelinating disease and autoimmune rheumatic disease in a pediatric population.
    Rev Bras Reumatol Engl Ed 2017 May - Jun;57(3):224-228. Epub 2016 Oct 25.
    Universidade Federal de São Paulo (Unifesp), Departamento de Pediatria, Setor de Reumatologia Pediátrica, São Paulo, SP, Brazil. Electronic address:
    Introduction: Multiple sclerosis (MS) and neuromyelitis optica (NMO) are demyelinating diseases of the central nervous system. Autoimmunity in patients with demyelinating disease and in their families has been broadly investigated and discussed. Recent studies show a higher incidence of rheumatic autoimmune diseases among adult patients with MS or NMO and their families, but there are no studies in the pediatric population. Read More

    Unsuspected Active Ulcerative Colitis in a Patient With Dermatomyositis: A Rare Association Detected on (18)F-FDG PET/CT During the Search for an Occult Malignancy.
    Indian J Nucl Med 2017 Apr-Jun;32(2):130-132
    Department of Nuclear Medicine, PGIMER, Chandigarh, India.
    Dermatomyositis is an inflammatory myopathy with the characteristic features of skin rash and myopathy. We here present a known case of dermatomyositis evaluated with (18)F-FDG PET/CT for the presence of any occult malignancy. The scan was negative for the presence of any malignancy. Read More

    Physical activity for paediatric rheumatic diseases: standing up against old paradigms.
    Nat Rev Rheumatol 2017 May;13(6):368-379
    Division of Rheumatology, Hospital das Clinicas da Faculdade de Medicina da Universidade de São Paulo.
    Over the past 50 years it has become clear that physical inactivity is associated with chronic disease risk. For several rheumatic diseases, bed rest was traditionally advocated as the best treatment, but several levels of evidence support the imminent paradigm shift from the prescription of bed rest to physical activity in individuals with paediatric rheumatic diseases, in particular juvenile systemic lupus erythematosus, juvenile idiopathic arthritis, juvenile fibromyalgia, and juvenile dermatomyositis. Increasing levels of physical activity can alleviate several symptoms experienced by patients with paediatric rheumatic diseases, such as low aerobic fitness, pain, fatigue, muscle weakness and poor health-related quality of life. 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

    Endothelial progenitor cell number is not decreased in 34 children with Juvenile Dermatomyositis: a pilot study.
    Pediatr Rheumatol Online J 2017 May 17;15(1):42. Epub 2017 May 17.
    Cure JM Program of Excellence in Juvenile Myositis Research at Stanley Manne Children's Research Institute of Ann and Robert H., Lurie Children's Hospital of Chicago, Chicago, IL, USA.
    Objective: A pilot study to determine endothelial progenitor cells (EPC) number in children with Juvenile Dermatomyositis (JDM).

    Methods: After obtaining informed consent, the EPC number from 34 fasting children with definite/probable JDM at various stages of therapy-initially untreated, active disease on medication and clinically inactive, off medication-was compared with 13 healthy fasting pediatric controls. The EPC number was determined by fluorescence activated cell sorting (FACS), CD34(+)/VEGFR2(+)/CD45dim(-), and assessed in conjunction with clinical variables: disease activity scores (DAS), duration of untreated disease (DUD), TNF-α allelic polymorphism (A/G) at the promoter region of -308, number of nailfold capillary end row loop (ERL) and von Willebrand factor antigen (vWF:Ag). 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

    Clinical characteristics and change in the antibody titres of patients with anti-MDA5 antibody-positive inflammatory myositis.
    Rheumatology (Oxford) 2017 May 11. Epub 2017 May 11.
    Department of Internal Medicine and Rheumatology, Juntendo University School of Medicine, Tokyo, Japan.
    Objective.: The aim of this study was to evaluate the clinical characteristics of patients with anti-melanoma differentiation-associated gene 5 (MDA5) antibody-positive inflammatory myositis, and the change in anti-MDA5 antibody titres before and after onset.

    Method. 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

    Evaluation of clinical prognostic factors for interstitial pneumonia in anti-MDA5 antibody-positive dermatomyositis patients.
    Mod Rheumatol 2017 May 11:1-8. Epub 2017 May 11.
    a Department of Internal Medicine (IV) , Osaka Medical College , Takatsuki , Osaka , Japan.
    Objectives: We retrospectively investigated clinical prognostic factors for interstitial pneumonia (IP) in anti-melanoma differentiation-associated gene 5 (MDA5) antibody (Ab)-positive dermatomyositis (DM) patients.

    Methods: Subjects comprised 18 patients with anti-MDA5 Ab-positive DM-IP (9 survivors; 9 deaths).

    Results: Initial serum albumin levels, ferritin levels, and ground-glass opacity (GGO) scores in the right middle lobes were significantly higher in the death group than in the survivor group (p = . Read More

    Hypomyopathic dermatomyositis associated with interstitial lung disease and good response to mycophenolate mofetil: case-based review.
    Clin Rheumatol 2017 May 9. Epub 2017 May 9.
    Institute of Health Sciences, Federal University of Pará, 1 Generalíssimo Deodoro Avenue, Belém, PA, 66050-160, Brazil.
    Hypomyopathic dermatomyositis (HDM) is a rare form of dermatomyositis (DM). Interstitial lung disease (ILD) associated with clinically amyopathic DM (CADM-ILD) or hypomyopathic DM (HDM-ILD) is a rare condition with a more unfavorable prognosis than ILD associated with classic DM (CDM-ILD). There is no effective treatment for HDM-ILD. Read More

    Chemokine profiles of interstitial pneumonia in patients with dermatomyositis: a case control study.
    Sci Rep 2017 May 9;7(1):1635. Epub 2017 May 9.
    Department of Internal Medicine (I), Osaka Medical College, Takatsuki, Osaka, Japan.
    Chemokines play an important role in the pathophysiology of dermatomyositis (DM) with interstitial pneumonia (IP). However, the relation between chemokines and the disease activity or prognosis of DM-IP has not been elucidated. We evaluated the serum C-C motif chemokine ligand (CCL) 2, Th1 chemokines (C-X-C motif chemokine ligand [CXCL] 9, CXCL10, CXCL11), and Th2 chemokine (CCL17) profiles of 30 patients, and examined the relation between these chemokines and the disease activity or prognosis of DM-IP. Read More

    MDA5 autoantibody-another indicator of clinical diversity in dermatomyositis.
    Ann Transl Med 2017 Apr;5(7):160
    University of Utah School of Medicine, Salt Lake City, Utah, USA.
    Allenbach and colleagues have recently reported for the first time the results of an intriguing study of the histopathologic, immunopathologic and gene expression differences in muscle biopsy tissue from adult dermatomyositis (DM) patients who do and do not have circulating MDA5 autoantibodies (anti-MDA5). Anti-MDA5 were originally identified in a clinically-defined subset of DM patients whose disease was expressed predominately in the skin for unusually long periods of time without accompanying muscle weakness [i.e. Read More

    Integrated Diagnosis Project for Inflammatory Myopathies: An association between autoantibodies and muscle pathology.
    Autoimmun Rev 2017 May 4. Epub 2017 May 4.
    Department of Neuromuscular Research, National Institute of Neuroscience, National Center of Neurology and Psychiatry, 4-1-1 Ogawa-Higashi, Kodaira, Tokyo 187-8502, Japan; Department of Genome Medicine Development, Medical Genome Center, National Center of Neurology and Psychiatry, 4-1-1 Ogawa-Higashi, Kodaira, Tokyo 187-8581, Japan.
    Inflammatory myopathies are a heterogeneous group of immune-mediated diseases that involve skeletal muscle as well as many other organs. The classification of inflammatory myopathies has been based on clinical diagnoses, pathological diagnoses, and autoantibodies, independently. The clinical phenotypes of inflammatory myopathies are characterized by various autoantibodies that are originally detected by RNA or protein immunoprecipitation. Read More

    Histopathologic Spectrum of Connective Tissue Diseases Commonly Affecting the Skin.
    Surg Pathol Clin 2017 Jun 14;10(2):477-503. Epub 2017 Mar 14.
    Department of Pathology, Brigham and Women's Hospital, Harvard Medical School, Amory-3, 75 Francis Street, Boston, MA 02115, USA.
    Connective tissue disorders (CTDs), also known as collagen vascular diseases, are a heterogeneous group of diseases with a common pathogenic mechanism: autoimmunity. Precise classification of CTDs requires clinical, serologic, and pathologic correlation and may be difficult because of overlapping clinical and histologic features. The main contribution of histopathology in the diagnosis of these disorders is to confirm, rule out, or alert clinicians to the possibility of CTD as a disease category, rather than producing definitive diagnoses of specific entities. 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

    Four Cases of Pneumatosis Cystoides Intestinalis Complicated by Connective Tissue Diseases.
    Intern Med 2017 1;56(9):1101-1106. Epub 2017 May 1.
    Department of Gastroenterology and Rheumatology, Division of Medicine, Fukushima Medical University School of Medicine, Japan.
    Pneumatosis cystoides intestinalis (PCI) is a rare disease that involves the presence of gas in the intestinal wall. Connective tissue disease (CTD) is a major cause of secondary PCI. In addition to the nature of CTDs, the use of prednisolone and some immunosuppressants, and the presence of complicating diseases such as diabetes mellitus, constipation and pulmonary diseases are involved in the development of PCI. Read More

    Paraneoplastic Dermatomyositis.
    J Assoc Physicians India 2017 Feb;65(2):89-90
    Postgraduate, Department of General Medicine, Coimbatore Medical College Hospital, Coimbatore, Tamil Nadu.
    Dermatomyositis and its association with malignancy is well known. Though many tumors like breast, ovary, lung carcinomas are reported to be associated with it, cervical carcinoma is a very rare one. Tumors in these patients are usually unmasked by abnormal findings in the medical history and physical examination. Read More

    Comparison of long-term prognosis and relapse of dermatomyositis complicated with interstitial pneumonia according to autoantibodies: anti-aminoacyl tRNA synthetase antibodies versus anti-melanoma differentiation-associated gene 5 antibody.
    Rheumatol Int 2017 Apr 27. Epub 2017 Apr 27.
    Department of Internal Medicine IV, Osaka Medical College, Osaka, Japan.
    The aim of this study was to investigate long-term prognosis and relapse of dermatomyositis complicated with interstitial pneumonia (DMIP) according to anti-aminoacyl tRNA synthetase (ARS) antibodies and anti-melanoma differentiation-associated gene 5 (MDA5) antibody. This retrospective study comprised 36 patients with DMIP who were divided into the anti-ARS antibody-positive group (ARS+) (n = 12), anti MDA5 antibody-positive group (MDA5+) (n = 11), double-negative group (ARS-/MDA5-) (n = 11), and double-positive group (ARS+/MDA5+) (n = 1). Clinical features, treatment, prognoses, and relapses during the 2 years after initiation of treatment were compared between three groups excluding ARS+/MDA5+ group. Read More

    Retinal Manifestations of Juvenile Dermatomyositis: Case Report of Bilateral Diffuse Chorioretinopathy with Paracentral Acute Middle Maculopathy and Review of the Literature.
    Ocul Immunol Inflamm 2017 Apr 27:1-5. Epub 2017 Apr 27.
    a Department of Ophthalmology and Visual Sciences, John A. Moran Eye Center , University of Utah , Salt Lake City , Utah , USA.
    Purpose: To review a case of bilateral diffuse chorioretinopathy as a presenting sign of juvenile dermatomyositis (JDM) and review the literature regarding retinal manifestations associated with this disease.

    Methods: Review of case record and literature regarding retinal manifestations related to juvenile dermatomyositis.

    Results: A 13-year-old girl presented with bilateral diffuse chorioretinopathy as the presenting sign of juvenile dermatomyositis. Read More

    Squamous cell carcinoma of the lung associated with anti-Jo1 antisynthetase syndrome: a case report and review of the literature.
    Rheumatol Int 2017 Apr 26. Epub 2017 Apr 26.
    Department of Rheumatology, Reims University Hospitals, 45 rue Cognacq-Jay, 51092, Reims cedex, France.
    Antisynthetase syndrome is a heterogeneous idiopathic inflammatory myopathy. Anti-Jo1 is the most common antibody found in this condition. Dermatomyositis is known to be associated with malignancy, but the association between antisynthetase syndrome and malignancy is not clearly established. Read More

    Environmental factors associated with disease flare in juvenile and adult dermatomyositis.
    Rheumatology (Oxford) 2017 Apr 21. Epub 2017 Apr 21.
    Division of Rheumatology, George Washington University School of Medicine and Health Sciences, Washington, DC.
    Objective.: The aim was to assess environmental factors associated with disease flare in juvenile and adult dermatomyositis (DM).

    Methods. Read More

    Amyopathic Dermatomyositis Associated with Histopathological Findings of Organizing Pneumonia and Pulmonary Vasculitis.
    Balkan Med J 2017 Apr 13. Epub 2017 Apr 13.
    Background: Clinically amyopathic dermatomyositis (CADM) is a distinct subgroup of dermatomyositis characterized by unique dermatologic manifestations without muscle involvement. CADM is frequently associated with interstitial lung disease (ILD), which usually has a rapidly progressive, fatal clinical course. Although CADM-related ILD (CADM-ILD) is well-described, data on the histopathology of CADM-ILD are limited. Read More

    Direct hemoperfusion with polymyxin B-immobilized fibre treatment for acute exacerbation of interstitial pneumonia.
    Respirology 2017 Apr 25. Epub 2017 Apr 25.
    Department of Respiratory Medicine, Tokyo Medical and Dental University, Tokyo, Japan.
    Background And Objective: Acute exacerbation of idiopathic pulmonary fibrosis (AE-IPF) is recognized as an important cause of mortality. AE has also been reported in patients with other interstitial lung diseases such as idiopathic non-specific interstitial pneumonia (NSIP) and interstitial pneumonia associated with collagen vascular disease (CVD). Current therapies such as high-dose corticosteroid with immunosuppressive agents have provided little benefit for AE. 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

    Efficacy and safety of leflunomide as an adjuvant drug in refractory dermatomyositis with primarily cutaneous activity.
    Clin Exp Rheumatol 2017 Apr 18. Epub 2017 Apr 18.
    Hospital das Clinicas HCFMUSP, Faculdade de Medicina, Universidade de Sao Paulo, Brazil.
    Objectives: To evaluate leflunomide as an adjuvant drug in refractory dermatomyositis (DM) with primarily cutaneous activity.

    Methods: A retrospective, single-centre, cohort study including 18 adult patients with DM (classical or clinically amyopathic DM) and cutaneous activity from 2001 to 2016 was conducted. Patients were dependent on glucocorticoid and refractory to at least two full-dose immunosuppressants/immunomodulators or presented previous adverse events with immunobiological drugs. Read More

    Evaluation of the Reliability of the Cutaneous Dermatomyositis Disease Area and Severity Index (CDASI) and the Cutaneous Assessment Tool Binary Method (CAT-BM) in Juvenile Dermatomyositis Among Pediatric Dermatologists, Rheumatologists, and Neurologists.
    Br J Dermatol 2017 Apr 19. Epub 2017 Apr 19.
    Department of Dermatology, Perelman School of Medicine at the University of Pennsylvania, Philadelphia, PA.
    Background: The Cutaneous Dermatomyositis Disease Area and Severity Index (CDASI) and Cutaneous Assessment Tool-Binary Method (CAT-BM) have been shown to be reliable and valid outcome measures to assess cutaneous disease in adult dermatomyositis (DM) and juvenile DM (JDM), respectively.

    Objective: This study compared the CDASI and CAT-BM for use by pediatric dermatologists, pediatric rheumatologists, and pediatric neurologists in patients with JDM.

    Methods: Five pediatric dermatologists, five pediatric rheumatologists, and five pediatric neurologists each evaluated 14 patients with JDM using the CDASI, CAT-BM, and skin Physician Global Assessment (PGA) scales. Read More

    Incidence of autoimmune diseases in patients with scabies: a nationwide population-based study in Taiwan.
    Rheumatol Int 2017 Apr 18. Epub 2017 Apr 18.
    Graduate Institute of Life Sciences, National Defense Medical Center, No. 161, Sec. 6, Minquan E. Road, Neihu District, Taipei, 114, Taiwan.
    Scabies is a commonly occurring infectious immune-mediated inflammatory skin disease. Immune-mediated inflammatory processes are also observed in autoimmune diseases. There have been very few previous studies; however, that have investigated the possible association between scabies and autoimmune diseases. Read More

    Detection of interferon alpha protein reveals differential levels and cellular sources in disease.
    J Exp Med 2017 May 18;214(5):1547-1555. Epub 2017 Apr 18.
    Immunobiology of Dendritic Cells, Institut Pasteur, 75015 Paris, France.
    Type I interferons (IFNs) are essential mediators of antiviral responses. These cytokines have been implicated in the pathogenesis of autoimmunity, most notably systemic lupus erythematosus (SLE), diabetes mellitus, and dermatomyositis, as well as monogenic type I interferonopathies. Despite a fundamental role in health and disease, the direct quantification of type I IFNs has been challenging. 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

    Cytokine profiles of amyopathic dermatomyositis with interstitial lung diseases treated with mycophenolate.
    Respirol Case Rep 2017 Jul 11;5(4):e00235. Epub 2017 Apr 11.
    Division of Respiratory MedicineNiigata University Medical and Dental HospitalNiigataJapan.
    A 59-year-old Japanese man diagnosed with interstitial lung disease associated with amyopathic dermatomyositis with anti-melanoma differentiation-associated gene 5 (MDA-5) antibodies was treated with intravenous methyl prednisolone (PSL) 1000 mg, oral PSL 1 mg/kg, and oral cyclosporin 200 mg daily. His respiratory condition worsened after treatment with two times of intravenous cyclophosphamide and another steroid pulse therapy as well as PSL and cyclosporin. Addition of mycophenolate mofetil (MMF), 1. Read More

    Varicella vaccination elicits a humoral and cellular response in children with rheumatic diseases using immune suppressive treatment.
    Vaccine 2017 May 12;35(21):2818-2822. Epub 2017 Apr 12.
    Laboratory for Translational Immunology, Paediatric Immunology, University Medical Center Utrecht, Utrecht, The Netherlands; Department of Paediatric Rheumatology, Wilhelmina Children's Hospital, University Medical Center Utrecht, Utrecht, The Netherlands.
    Objective: To assess humoral and cellular responses to live-attenuated varicella zoster virus (VZV) vaccination of patients with juvenile idiopathic arthritis (JIA), juvenile dermatomyositis (JDM) or juvenile scleroderma (JScle) compared to those of healthy controls (HC).

    Methods: Before, 4-6weeks and one year after VZV vaccination, blood samples of patients and HC were collected. VZV-specific antibody concentrations were measured by ELISA and multiplex immune-assay. Read More

    The epigenetic mechanism for discordance of autoimmunity in monozygotic twins.
    J Autoimmun 2017 Apr 12. Epub 2017 Apr 12.
    Department of Dermatology, Second Xiangya Hospital, Central South University, Hunan Key Laboratory of Medical Epigenomics, Changsha, Hunan, China. Electronic address:
    Monozygotic twins share an identical DNA sequence but are not truly "identical". In fact, when it comes to health and disease, they may often display some level of phenotypic discordance. The cause of this discordance is often unknown. Read More

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