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    Assessment of anti-MDA5 antibody as a diagnostic biomarker in patients with dermatomyositis-associated interstitial lung disease or rapidly progressive interstitial lung disease.
    Oncotarget 2017 Jul 6. Epub 2017 Jul 6.
    Department of Rheumatology and Clinical Immunology, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, Key Laboratory of Rheumatology and Clinical Immunology, Ministry of Education, Beijing, China.
    Anti-melanoma differentiation-associated protein 5 (MDA5) antibody have been found in dermatomyositis (DM)-associated interstitial lung disease (DM-ILD) and DM-associated rapidly progressive ILD (DM-RPILD). Due to the conflicting results regarding the association between anti-MDA5 antibody and DM-ILD or DM-RPILD and the diagnostic value of this antibody for DM-ILD and DM-RPILD, we performed this meta-analysis. A systematic search was performed to identify studies published to January 14, 2017. Read More

    Dermatomyositis Induced by Hepatitis B Virus-related Hepatocellular Carcinoma: A Case Report and Review of the Literature.
    Intern Med 2017 15;56(14):1831-1837. Epub 2017 Jul 15.
    Department of Education, China Medical University Hospital, Taiwan.
    Dermatomyositis or polymyositis as a paraneoplastic syndrome of hepatocellular carcinoma (HCC) is an uncommon event. Few cases have been reported in the literature. We herein report the case of a 55-year-old man with chronic hepatitis B and alcoholism who presented with skin rash. Read More

    An efficacy analysis of whole-body magnetic resonance imaging in the diagnosis and follow-up of polymyositis and dermatomyositis.
    PLoS One 2017 17;12(7):e0181069. Epub 2017 Jul 17.
    Department of Rheumatology, China-Japan Friendship Hospital, Beijing, China.
    Objectives: To evaluate the value of whole-body magnetic resonance imaging (WBMRI) in diagnosing muscular and extra muscular lesions in patients with polymyositis (PM) and dermatomyositis (DM).

    Methods: A retrospective analysis of WBMRI data from PM/DM patients who met the Bohan and Peter diagnostic criteria was performed. X2 test was used to compare the rate of positive diagnosis of newly diagnosed patients using WBMRI, serum creatine kinase test, and EMG. Read More

    HLA-DRB1 Alleles as Genetic Risk Factors for the Development of Anti-MDA5 Antibodies in Patients with Dermatomyositis.
    J Rheumatol 2017 Jul 15. Epub 2017 Jul 15.
    From the Department of Rheumatology and Immunology, and Department of Clinical Laboratory, the Affiliated Drum Tower Hospital, Nanjing University Medical School, Nanjing, China; Department of Allergy and Rheumatology, Nippon Medical School Graduate School of Medicine; Department of Molecular Pathogenesis, Medical Research Institute, Tokyo Medical and Dental University, Tokyo, Japan. Supported by the National Natural Science Foundation of China (to ZC, NO. 81302556). Key Project supported by Medical Science and Technology Development Foundation, Nanjing Department of Health (to ZC, NO. ZKX16039). Z. Chen, MD, PhD, Department of Rheumatology and Immunology, the Affiliated Drum Tower Hospital, Nanjing University Medical School; Y. Wang, MD, Department of Rheumatology and Immunology, the Affiliated Drum Tower Hospital, Nanjing University Medical School; M. Kuwana, MD, PhD, Department of Allergy and Rheumatology, Nippon Medical School Graduate School of Medicine; X. Xu, MD, PhD, Department of Rheumatology and Immunology, the Affiliated Drum Tower Hospital, Nanjing University Medical School; W. Hu, MD, Department of Clinical Laboratory, the Affiliated Drum Tower Hospital, Nanjing University Medical School; X. Feng, MD, PhD, Department of Rheumatology and Immunology, the Affiliated Drum Tower Hospital, Nanjing University Medical School; H. Wang, MD, Department of Rheumatology and Immunology, the Affiliated Drum Tower Hospital, Nanjing University Medical School; A. Kimura, MD, PhD, Department of Molecular Pathogenesis, Medical Research Institute, Tokyo Medical and Dental University; L. Sun, MD, PhD, Department of Rheumatology and Immunology, the Affiliated Drum Tower Hospital, Nanjing University Medical School. Address correspondence to Dr. A. Kimura, Department of Molecular Pathogenesis, Medical Research Institute, Tokyo Medical and Dental University, 1-5-45 Yushima, Bunkyo-ku, Tokyo 113-8510, Japan. E-mail: or Dr. L. Sun, Department of Rheumatology and Immunology, Drum Tower Clinical College of Nanjing Medical University, 321 Zhongshan Road, Nanjing, 210008, China. E-mail: Accepted for publication May 25, 2017.
    Objective: Patients with polymyositis/dermatomyositis (PM/DM) who express anti-melanoma differentiation associated protein 5 (anti-MDA5) antibodies frequently present with interstitial lung disease (ILD). The aim of this study was to investigate the association of HLA-DRB1 with anti-MDA5 expression in PM/DM.

    Methods: The frequency of DRB1 alleles was compared among 70 patients with PM, 104 patients with DM, and 400 healthy controls in a Han Chinese population. Read More

    Clinical Utility of YKL-40 in Polymyositis/dermatomyositis-associated Interstitial Lung Disease.
    J Rheumatol 2017 Jul 15. Epub 2017 Jul 15.
    From the Second Division, Department of Internal Medicine, Hamamatsu University School of Medicine; Department of Clinical Pharmacology and Therapeutics, Hamamatsu University School of Medicine, Hamamatsu; Department of Rheumatology and Clinical Immunology, Graduate School of Medicine, Kyoto University, Kyoto; Department of IVD Development, Medical and Biological Laboratories Co. Ltd., Ina, Japan. Supported by a grant from the Japan Society for the Promotion of Science (JP16K19449 to H.H.). H. Hozumi, MD, PhD, Research Associate, Second Division, Department of Internal Medicine, Hamamatsu University School of Medicine; T. Fujisawa, MD, PhD, Research Associate, Second Division, Department of Internal Medicine, Hamamatsu University School of Medicine; N. Enomoto, MD, PhD, Lecturer, Second Division, Department of Internal Medicine, Hamamatsu University School of Medicine; R. Nakashima, MD, PhD, Research Associate, Department of Rheumatology and Clinical Immunology, Graduate School of Medicine, Kyoto University; Y. Enomoto, MD, Second Division, Department of Internal Medicine, Hamamatsu University School of Medicine; Y. Suzuki, MD, PhD, Research Associate, Second Division, Department of Internal Medicine, Hamamatsu University School of Medicine; M. Kono, MD, PhD, Research Associate, Second Division, Department of Internal Medicine, Hamamatsu University School of Medicine; M. Karayama, MD, PhD, Lecturer, Second Division, Department of Internal Medicine, Hamamatsu University School of Medicine; K. Furuhashi, MD, PhD, Research Associate, Second Division, Department of Internal Medicine, Hamamatsu University School of Medicine; A. Murakami, PhD, Department of IVD Development, Medical and Biological Laboratories Co. Ltd.; N. Inui, MD, PhD, Assistant Professor, Department of Clinical Pharmacology and Therapeutics, Hamamatsu University School of Medicine; Y. Nakamura, MD, PhD, Lecturer, Second Division, Department of Internal Medicine, Hamamatsu University School of Medicine; T. Mimori, MD, PhD, Professor, Department of Rheumatology and Clinical Immunology, Graduate School of Medicine, Kyoto University; T. Suda, MD, PhD, Professor, Second Division, Department of Internal Medicine, Hamamatsu University School of Medicine. Address correspondence to Dr. H. Hozumi, Second Division, Department of Internal Medicine, Hamamatsu University School of Medicine, 1-20-1 Handayama Higashiku, Hamamatsu 431-3192, Japan. E-mail: for publication May 13, 2017.
    Objective: Interstitial lung disease (ILD) is involved in polymyositis/dermatomyositis (PM/DM), a disease associated with poor prognoses. Chitinase-3-like-1 protein (YKL-40) has pleiotropic biological activities involved in inflammation, cell proliferation, and tissue remodeling; however, the clinical application of YKL-40 remains limited. We investigated the clinical significance of YKL-40 in PM/DM-ILD. Read More

    Anti-TIF1-gamma antibodies are not associated with other paraneoplastic rheumatic syndromes than dermatomyositis.
    Arthritis Care Res (Hoboken) 2017 Jul 13. Epub 2017 Jul 13.
    Rheumatology unit, Department of Medicine, Karolinska Institutet, Solna, Karolinska University Hospital, and Center for Molecular Medicine, Stockholm, Sweden.
    Objectives: An association between cancer and dermatomyositis (DM) is well recognized. The high frequency of malignancies detected close to DM diagnosis suggests that DM can be a paraneoplastic syndrome. Recently anti-TIF1-gamma has been discovered to be associated with cancer and DM. Read More

    NXP-2 Positive Dermatomyositis: A Unique Clinical Presentation.
    Case Rep Rheumatol 2017 13;2017:4817275. Epub 2017 Jun 13.
    Division of Rheumatology, John Hopkins University School of Medicine, 733 N Broadway, Baltimore, MD 21205, USA.
    Dermatomyositis (DM), a myopathy associated with inflammation and muscle weakness, has historically been difficult to diagnose. Recently, nuclear matrix protein (NXP-2) antibodies have been described as a myositis-specific antibody that may aid in the diagnostic evaluation. We present the case of a 21-year-old, previously healthy, African American male with DM. Read More

    Cardiac findings in children with juvenile Dermatomyositis at disease presentation.
    Pediatr Rheumatol Online J 2017 Jul 11;15(1):54. Epub 2017 Jul 11.
    Department of Pediatrics, Faculty of Medicine, and the Institute of Health Policy, Management & Evaluation, the Dalla Lana School of Public Health,, University of Toronto, Division of Rheumatology, The Hospital for Sick Children, Toronto, Canada.
    Background: Juvenile Dermatomyositis (JDM) is a pediatric vasculopathy characterized primarily by skin and muscle involvement. Cardiac findings have been reported in children with JDM but have rarely been investigated in detail.

    Methods: We aimed to describe the relevant clinical and laboratory cardiac findings of a cohort of patients with JDM, followed at one centre, at disease diagnosis. Read More

    Pharmacological management of dermatomyositis.
    Expert Rev Clin Pharmacol 2017 Jul 17:1-10. Epub 2017 Jul 17.
    a Division of Clinical Immunology, Faculty of Medicine , University of Debrecen , Debrecen , Hungary.
    Introduction: Dermatomyositis is a rare heterogeneous systemic autoimmune disease with multiple organ involvement which can result in significant disability and mortality. Despite the lack of placebo-controlled trials, glucocorticoids are considered to be the mainstay of initial management. Treatment strategies are mainly based on uncontrolled studies, evidence based guidelines for treatments do not exist. Read More

    Autoamputation and Polyneuropathy in Mixed Connective Tissue Disorder: A Case Report.
    Cureus 2017 Jun 5;9(6):e1313. Epub 2017 Jun 5.
    Department of Medicine, Jinnah Postgraduate Medical Center Karachi Pakistan.
    Mixed connective tissue disorder (MCTD) is a multisystem disease with overlapping features of other autoimmune diseases, such as systemic lupus erythematosus (SLE), myositis, rheumatoid arthritis, and scleroderma. MCTD presents with a distinctive antibody in serum known as U1-ribonucleoprotein (RNP). MCTD is quite rare as compared to other connective tissue disorders like SLE, systemic sclerosis, dermatomyositis, and polymyositis. Read More

    Prevalence of anti-NT5C1A antibodies in Japanese patients with autoimmune rheumatic diseases in comparison with other patient cohorts.
    Clin Chim Acta 2017 Jul 4;472:1-4. Epub 2017 Jul 4.
    Department of Dermatology, Nagoya University Graduate School of Medicine, Nagoya, Japan.
    Background: Sporadic inclusion body myositis (sIBM) is usually classified as an idiopathic inflammatory myopathies. Although the diagnosis of sIBM is sometimes challenging, recent studies have shown that the autoantibodies against cytosolic 5'-nucleotidase 1A (NT5C1A) are the possible diagnostic biomarker for sIBM. Few reports have shown the frequencies of anti-NT5C1A antibodies in systemic autoimmune rheumatic diseases (SARDs) using large cohorts of SARDs. Read More

    Phenotypic characteristics and outcome of juvenile dermatomyositis in Arab children.
    Rheumatol Int 2017 Jul 6. Epub 2017 Jul 6.
    Consultant and Section Head, Rheumatology, Department of Pediatrics, King Faisal Specialist Hospital and Research Center, Po Box 3354, Riyadh, 11211, Saudi Arabia.
    This study describes the disease characteristics and outcome of Arab children with juvenile dermatomyositis (JDM) and compares the findings with other ethnicities. We retrospectively reviewed the hospital registries of the participating hospitals for children with JDM seen between 1990 and 2016 in three Arab countries. All patients fulfilled Bohan and Peter criteria for JDM, diagnosed before 14 years of age and were of Arab ethnicity. Read More

    A simple method for removing low-density granulocytes to purify T lymphocytes from peripheral blood mononuclear cells.
    J Zhejiang Univ Sci B 2017 Jul;18(7):605-614
    Department of Rheumatology, China-Japan Friendship Hospital, Beijing 100029, China.
    Objective: Low-density granulocytes (LDGs) can form neutrophil extracellular traps (NETs) spontaneously and excessively. When peripheral blood mononuclear cells (PBMCs) are used for studying T lymphocytes, LDGs contained in the PBMCs may decrease the threshold of activating T lymphocytes by forming NETs. This study focused on the profiles of LDGs in common autoimmune diseases and methods for removing LDGs from PBMCs. Read More

    Autoantibodies to Su/Argonaute 2 in Japanese patients with inflammatory myopathy.
    Clin Chim Acta 2017 Aug 30;471:304-307. Epub 2017 Jun 30.
    Department of Dermatology, Nagoya University Graduate School of Medicine, Showa-ku, Nagoya, Japan.
    Background: Anti-Su antibodies are found in 5-20% of cases of various systemic autoimmune rheumatic diseases and in 5-10% of dermatomyositis (DM)/polymyositis (PM) patients. In 2006, the 100kDa Su antigen was identified as argonaute2 (Ago2), and it was found to play a major role in RNA interference. However, immunoprecipitation (IP) remains the main method for detecting anti-Su and the clinical significance of the antibodies is uncertain. Read More

    Review of Primary Cutaneous Mucinoses in Nonlupus Connective Tissue Diseases.
    J Cutan Med Surg 2017 Jul 1:1203475417719053. Epub 2017 Jul 1.
    1 Division of Dermatology, Cumming School of Medicine, Calgary, AB, Canada.
    Lichen myxedematosus is an idiopathic, cutaneous mucinosis with 2 clinicopathologic subsets. There is the generalised papular and sclerodermoid form, more properly termed scleromyxedema, and the localised papular form. We report the first case, to our knowledge, of lichen myxedematosus in association with rheumatoid arthritis as well as a case in association with dermatomyositis. Read More

    Is it cardiac involvement mimicking acute myocardial infarction in idiopathic inflammatory myopathy?
    Exp Ther Med 2017 Jul 11;14(1):349-354. Epub 2017 May 11.
    Department of Rheumatology and Immunology, Xiangya Hospital, Central South University, Changsha, Hunan 410008, P.R. China.
    Idiopathic inflammatory myopathy (IIM) comprises a group of rare systemic diseases characterized by progressive weakness of the symmetrical proximal limb muscles, elevated muscle enzymes, inflammation or necrosis on muscle biopsy. IIM may impair the function of multiple organs, particularly the heart. However, it rarely manifests as acute myocardial infarction (AMI) at initial presentation. Read More

    State of the art on nailfold capillaroscopy in dermatomyositis and polymyositis.
    Semin Arthritis Rheum 2017 Jun 28. Epub 2017 Jun 28.
    Division of Musculoskeletal and Rheumatic Disorders, Instituto Nacional de Rehabilitación, Mexico City, Mexico. Electronic address:
    Objectives: To provide an overview of the main nailfold capillaroscopy (NFC) changes described in dermatomyositis (DM) and polymyositis (PM) and to discuss the current evidence supporting its clinical relevance and applications in daily practice.

    Methods: All relevant literature in the field of NFC and DM and PM published in the last 30 years has been systematically reviewed. A systematic research was performed in the electronic databases PubMed and EMBASE. Read More

    [Amyopathic dermatomyositis (DM) with anti-MDA5 antibodies, associated with bullous pemphigoid, Sjögren syndrome and gastric MALT lymphoma].
    Ann Dermatol Venereol 2017 Jun 28. Epub 2017 Jun 28.
    Service de dermatologie, hôpital Saint-Vincent-de-Paul, université catholique de Lille, boulevard de Belfort, BP 387, 59020 Lille, France.
    Background: The inflammatory myopathies are a heterogeneous group of muscle diseases and comprise polymyositis, dermatomyositis (DM), myopathies associated with cancers, necrotising myositis and inclusion body myositis. DM occasionally exhibits few or no muscular signs: i.e. Read More

    Clinical and Economic Evaluation of Repository Corticotropin Injection: A Narrative Literature Review of Treatment Efficacy and Healthcare Resource Utilization for Seven Key Indications.
    Adv Ther 2017 Jun 28. Epub 2017 Jun 28.
    Mallinckrodt Pharmaceuticals Inc., Hampton, NJ, USA.
    Introduction: Repository corticotropin injection (RCI; H.P. Acthar(®) Gel; Mallinckrodt Pharmaceuticals Inc. Read More

    Dermatomyositis Associated with Myelofibrosis following Polycythemia Vera.
    Case Rep Hematol 2017 4;2017:9091612. Epub 2017 Jun 4.
    Department of Internal Medicine, West Virginia University Hospital, 1 Medical Center Dr., Morgantown, WV 26505, USA.
    Dermatomyositis (DM) is a unique inflammatory myopathy with clinical findings of proximal muscle weakness, characteristic rash, and elevated muscle enzymes. The association of DM and malignancy, most commonly adenocarcinoma, is well known. There have been few case reports of primary myelofibrosis associated with DM. Read More

    Elevated IL-4 and IFN-γ Levels in Muscle Tissue of Patients with Dermatomyositis.
    In Vivo 2017 Jul-Aug;31(4):657-660
    Department of Neuroscience, Aziz Sancar Institute for Experimental Medical Research, Istanbul University, Istanbul, Turkey
    Background/aim: To investigate the contribution of muscle tissue-derived cytokines in dermatomyositis (DM).

    Materials And Methods: Muscle homogenates were prepared from deltoid muscle biopsy specimens of 10 patients with DM and eight controls with no pathological signs of myopathy. Interleukin (IL)-4, interferon (IFN)-γ and IL-17 levels were evaluated by enzyme-linked immunosorbent assay (ELISA) and immunoblotting analysis. Read More

    Dermatomyositis with renal infarction: a case report and literature review.
    J Int Med Res 2017 Jan 1:300060517709673. Epub 2017 Jan 1.
    Department of Rheumatology and Immunology, Shanghai Changzheng Hospital, the Second Military Medical University, Shanghai, China.
    Renal infarction is a rare clinical entity that is not easily detected by low-sensitivity ultrasound. We herein report a case of dermatomyositis with renal infarction detected during corticosteroid therapy. The patient was followed up for 18 months. Read More

    Expression of the autoantigen TRIM33/TIF1γ in skin and muscle of patients with dermatomyositis is upregulated, together with markers of cellular stress.
    Clin Exp Dermatol 2017 Aug 22;42(6):659-662. Epub 2017 Jun 22.
    Department of Dermatology and Allergy, University Hospital, Bonn, Germany.
    Dermatomyositis (DM) is an autoimmune disorder associated with a dysregulation of immune homeostasis of both the innate and adaptive immune system. Earlier data suggested that these two arms of the immune system interconnect in DM. In the current study, we analysed the association of autoantigen expression [adaptive system components: Mi2, transcriptional intermediary factor (TIF)1γ, small ubiquitin-like modifier 1 activating enzyme subunit (SAE)1, melanoma differentiation-associated protein (MDA)5] with markers of cellular stress (innate system components: MxA, p53) in skin and muscle (immunohistology and gene expression data, respectively). Read More

    Gottron Papules Show Histopathologic Features of Localized Lymphedema.
    Am J Dermatopathol 2017 Jul;39(7):518-523
    *Department of Cellular Pathology, Hospital El Bierzo, Ponferrada, Spain; and †Department of Pathology, Southern California Kaiser Permanente, Los Angeles Medical Center (LAMC), Kaiser Permanente, Los Angeles, CA.
    Although Gottron papules are a relevant feature of dermatomyositis, they are reported in very few papers in the literature, and the largest series on the subject includes 11 cases. We intend to study the main histopathologic features of Gottron papules in a series of 12 biopsies. We evaluated hematoxylin-eosin stains on all cases. Read More

    Evaluation of cancer-associated myositis and scleroderma autoantibodies in breast cancer patients without rheumatic disease.
    Clin Exp Rheumatol 2017 Jun 19. Epub 2017 Jun 19.
    Department of Epidemiology, Johns Hopkins Bloomberg School of Public Health, Baltimore; and Division of Medical Oncology, Department of Medicine, Johns Hopkins University School of Medicine Baltimore MD, USA.
    Objectives: Systemic sclerosis (scleroderma) and dermatomyositis are two prototypic autoimmune diseases that are strongly associated with malignancy. While specific autoantibodies in these diseases are markers of an increased risk of cancer at scleroderma and dermatomyositis onset, it is not known whether these autoantibodies are biomarkers of cancer risk in patients without rheumatic disease.

    Methods: In a matched case-control study of women without rheumatic disease, identified from a familial breast cancer cohort, 50 breast cancer cases and 50 controls were assayed for 3 autoantibodies that are known markers of cancer-associated scleroderma and dermatomyositis: anti-RNA polymerase III, anti-NXP2, and anti-TIF1γ. Read More

    IFN-β-induced reactive oxygen species and mitochondrial damage contribute to muscle impairment and inflammation maintenance in dermatomyositis.
    Acta Neuropathol 2017 Jun 16. Epub 2017 Jun 16.
    Institut de Physiologie EA 3072, Service de Physiologie et d'Explorations Fonctionnelles, Hôpitaux Universitaires de Strasbourg, Strasbourg, France.
    Dermatomyositis (DM) is an autoimmune disease associated with enhanced type I interferon (IFN) signalling in skeletal muscle, but the mechanisms underlying muscle dysfunction and inflammation perpetuation remain unknown. Transcriptomic analysis of early untreated DM muscles revealed that the main cluster of down-regulated genes was mitochondria-related. Histochemical, electron microscopy, and in situ oxygraphy analysis showed mitochondrial abnormalities, including increased reactive oxygen species (ROS) production and decreased respiration, which was correlated with low exercise capacities and a type I IFN signature. Read More

    Rituximab versus cyclophosphamide for the treatment of connective tissue disease-associated interstitial lung disease (RECITAL): study protocol for a randomised controlled trial.
    Trials 2017 Jun 15;18(1):275. Epub 2017 Jun 15.
    NIHR Biomedical Research Unit, Royal Brompton Hospital, Sydney Street, London, SW3 6NP, UK.
    Background: Interstitial lung disease (ILD) frequently complicates systemic autoimmune disorders resulting in considerable morbidity and mortality. The connective tissue diseases (CTDs) most frequently resulting in ILD include: systemic sclerosis, idiopathic inflammatory myositis (including dermatomyositis, polymyositis and anti-synthetase syndrome) and mixed connective tissue disease. Despite the development, over the last two decades, of a range of biological therapies which have resulted in significant improvements in the treatment of the systemic manifestations of CTD, the management of CTD-associated ILD has changed little. Read More

    Biologic therapies for refractory juvenile dermatomyositis: five years of experience of the Childhood Arthritis and Rheumatology Research Alliance in North America.
    Pediatr Rheumatol Online J 2017 Jun 13;15(1):50. Epub 2017 Jun 13.
    School of Medicine, University of Missouri, Columbia, MO, USA.
    Background: The prognosis of children with juvenile dermatomyositis (JDM) has improved remarkably since the 1960's with the use of corticosteroid and immunosuppressive therapy. Yet there remain a minority of children who have refractory disease. Since 2003 the sporadic use of biologics (genetically-engineered proteins that usually are derived from human genes) for inflammatory myositis has been reported. Read More

    Modelling disease activity in juvenile dermatomyositis: A Bayesian approach.
    Stat Methods Med Res 2017 Jan 1:962280217713233. Epub 2017 Jan 1.
    5 Department of Statistical Science, University College London, UK.
    Juvenile dermatomyositis is the most common form of the juvenile idiopathic inflammatory myopathies characterised by muscle and skin inflammation, leading to symmetric proximal muscle weakness and cutaneous symptoms. It has a fluctuating course and varying prognosis. In a Bayesian framework, we develop a joint model for four longitudinal outcomes, which accounts for within individual variability as well as inter-individual variability. Read More

    Potential role of autophagy in T‑cell survival in polymyositis and dermatomyositis.
    Mol Med Rep 2017 Aug 6;16(2):1180-1188. Epub 2017 Jun 6.
    Department of Rheumatology, China‑Japan Friendship Hospital, Chaoyang, Beijing 100029, P.R. China.
    Peripheral blood T lymphocytopenia has previously been identified in polymyositis/dermatomyositis (PM/DM) patients. Therefore, the present study aimed to examine the potential role of autophagy in peripheral blood T cell survival in PM/DM patients. Transmission electron microscopy was used to detect the formation of autophagosomes of peripheral blood cluster of differentiation (CD)3+ T cells obtained from 24 patients with PM/DM and 21 healthy controls. Read More

    Quality of life correlates with muscle strength in patients with dermato- or polymyositis.
    Clin Rheumatol 2017 Jun 6. Epub 2017 Jun 6.
    Department of Rheumatology, Odense University Hospital, Sønder Boulevard 29, 5000, Odense, Denmark.
    The aim of this study was to compare health-related quality of life (HQoL) in adults with dermatomyositis (DM) or polymyositis (PM) with a healthy control group and to assess whether muscle strength was associated with HQoL in patients with DM or PM. A cross-sectional study was performed and included 75 patients with DM or PM and 48 healthy controls. HQoL was assessed by the Short Form 36 questionnaire (SF-36). Read More

    Spontaneous pneumomediastinum in a dermatomyositis patient with anti-melanoma differentiation-associated gene-5 antibody and interstitial lung disease despite an initial response to immunosuppressant.
    Int J Rheum Dis 2017 Jun 4. Epub 2017 Jun 4.
    Division of Rheumatology and Clinical Immunology, Queen Mary Hospital, Hong Kong, China.
    We report a 24-year-old man with anti-melanoma differentiation-associated gene-5 (MDA5) antibody-positive dermatomyositis (DM) and interstitial lung disease (ILD) who developed spontaneous pneumomediastinum. By comparing serial thoracic high-resolution computed tomography scans, we demonstrated the distinct time course showing a paradoxical occurrence of pneumomediastinum despite a radiological improvement of ILD. Our case shows that pneumomediastinum in DM can occur regardless of associated ILD and it is a serious complication that should be considered in DM patients presenting with pulmonary manifestations. Read More

    Purtscher-like retinopathy associated with dermatomyositis and hemophagocytic lymphohistiocytosis.
    Arch Soc Esp Oftalmol 2017 May 29. Epub 2017 May 29.
    CIBERER, Unidad de Patología Neuromuscular, Hospital Universitario y Politécnico La Fe, Valencia, España.
    Case Report: An 11 year-old girl with progressive muscle weakness due to dermatomyositis and hemophagocytic lymphohistiocytosis in which multiple cotton exudates, venous tortuosity and flame haemorrhages are detected in the funduscopic examination, leading to the diagnosis of Purtscher-like retinopathy.

    Discussion: Purstcher's retinopathy is a microvascular disorder with clinical signs of probable thrombotic origin. Its treatment is controversial, with systemic corticosteroids being the most widespread choice. Read More

    Dermatomyositis with Rapidly Progressive Interstitial Lung Disease Treated with Rituximab: A Report of 3 Cases in Japan.
    Intern Med 2017 1;56(11):1399-1403. Epub 2017 Jun 1.
    Division of Rheumatology, Teikyo University Chiba Medical Center, Japan.
    We performed a retrospective chart review of three patients with hypomyopathic dermatomyositis and rapidly progressive interstitial lung disease. The patients were Japanese women of 71, 69, and 65 years of age. Two patients were anti-melanoma differentiation-associated gene 5 (anti-MDA5) antibody-positive and 1 was anti-aminoacyl-tRNA synthetase (anti-ARS) antibody-positive. Read More

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