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    1 OF 194

    [Dermatologic toxicities of immune checkpoint inhibitors].
    Ann Dermatol Venereol 2018 Apr 17. Epub 2018 Apr 17.
    Oncodermatologie, institut universitaire du cancer Toulouse Oncopole, 1, avenue Irène-Joliot-Curie, 31059 Toulouse cedex 9, France.
    The development of immune checkpoint inhibitors (monoclonal antibodies targeting PD-1/PD-L1 or CTLA-4) represents a significant advance in the treatment of multiple cancers. Given their particular mechanism of action, which involves triggering CD4+/CD8+ T-cell activation and proliferation, they are associated with a specific safety profile. Their adverse events are primarily immune-related, and can affect practically all organs. Read More

    Comparing the importance of quality measurement themes in juvenile idiopathic inflammatory myositis between patients and families and healthcare professionals.
    Pediatr Rheumatol Online J 2018 Apr 19;16(1):28. Epub 2018 Apr 19.
    Pediatric Rheumatology, Benioff Children's Hospital and University of San Francisco Medical Center, 1975 4th Street, San Francisco, CA, 94158, USA.
    Background: A standardized set of quality measures for juvenile idiopathic inflammatory myopathies (JIIM) is not in use. Discordance has been shown between the importance ascribed to quality measures between patients and families and physicians. The objective of this study was to assess and compare the importance of various aspects of high quality care to patients with JIIM and their families with healthcare providers, to aid in future development of comprehensive quality measures. Read More

    Musculoskeletal Manifestations of Non-RA Connective Tissue Diseases: Scleroderma, Systemic Lupus Erythematosus, Still's Disease, Dermatomyositis/Polymyositis, Sjögren's Syndrome, and Mixed Connective Tissue Disease.
    Semin Musculoskelet Radiol 2018 Apr 19;22(2):166-179. Epub 2018 Apr 19.
    Department of Musculoskeletal Radiology, University Hospital of Lille, Lille Cedex, France.
    The most common systemic rheumatologic conditions are connective tissue diseases (including rheumatoid arthritis [RA]) followed by spondyloarthropathy. With the advent of biotherapies and imaging biomarkers, development in the imaging of RA and spondyloarthropathies has received substantial attention in the literature. This article details the various musculoskeletal imaging features of the other connective tissue diseases such as scleroderma and progressive systemic sclerosis, systemic lupus erythematosus, Still's disease, dermatomyositis and polymyositis, Sjögren's syndrome, and mixed connective tissue disease. Read More

    Imaging Features of the Juvenile Inflammatory Arthropathies.
    Semin Musculoskelet Radiol 2018 Apr 19;22(2):147-165. Epub 2018 Apr 19.
    Department of Paediatric Imaging, Hospital Jeanne de Flandre, CHU Lille, France.
    We discuss the imaging of several juvenile inflammatory arthropathies including juvenile idiopathic arthritis, juvenile systemic lupus erythematosus, juvenile scleroderma, juvenile dermatomyositis, and chronic recurrent multifocal osteomyelitis. Juvenile idiopathic arthritis is the most common autoimmune chronic systemic disease of connective tissue in children. The remaining systemic juvenile connective tissue diseases are rare. Read More

    Current diagnosis and treatment of polymyositis and dermatomyositis.
    Mod Rheumatol 2018 Apr 18:1-24. Epub 2018 Apr 18.
    a Department of Rheumatology , Graduate School of Medical and Dental Sciences, Tokyo Medical and Dental University (TMDU) , Tokyo , Japan.
    Idiopathic inflammatory myopathies (IIMs) are heterogeneous disorders that affect the skeletal muscles. Polymyositis, dermatomyositis, and inclusion body myositis are major IIM subsets. Immune-mediated necrotizing myopathy became recognized as a potentially new IIM subset. Read More

    MDA-5 associated rapidly progressive interstitial lung disease with recurrent Pneumothoraces: a case report.
    BMC Pulm Med 2018 Apr 17;18(1):59. Epub 2018 Apr 17.
    Rheumatology Department, Cork University Hospital, Wilton, Cork, Ireland.
    Background: Clinically hypomyopathic dermatomyositis is a rare disease that is important to recognize, investigate and treat early as it is associated with poor prognosis. In a proportion of patients, myositis specific antibodies could be negative, but with high clinical suspicion, myositis associated antibodies should be ordered. Anti-MDA-5 antibodies was reported in literature to be associated with severe and rapidly progressive interstitial lung disease, with few case reports of pneumothorax and/or pneumomediastinum. Read More

    Dermatomyositis Associated With a Skull Base Chondrosarcoma.
    J Clin Rheumatol 2018 Apr 17. Epub 2018 Apr 17.
    Division of Rheumatology Department of Internal Medicine Stanford University School of Medicine Stanford, CA Department of Dermatology Massachusetts General Hospital Harvard Medical School Boston, MA Division of Rheumatology Department of Medicine Massachusetts General Hospital Harvard Medical School Boston, MA

    Association of Serum Soluble CD163 with Polymyositis and Dermatomyositis, Especially in Anti-MDA5 Antibody-positive Cases.
    J Rheumatol 2018 Apr 15. Epub 2018 Apr 15.
    From the Institute of Rheumatology, Tokyo Women's Medical University; Department of Allergy and Rheumatology, Nippon Medical School Graduate School of Medicine, Tokyo, Japan. Supported in part by the Japanese Ministry of Education, Culture, Sports, Science, and Technology Grants-in-Aid for Scientific Research (KAKENHI Grant Number 17K16215). This study was also supported in part by the Nagao Takeshi Nanbyo Foundation. The funders of the study had no role in the study design, conduct of the study, data collection, or data interpretation or preparation of the report. MK holds a patent on an anti-MDA5 antibody measuring kit. H. Kawasumi, MD, Institute of Rheumatology, Tokyo Women's Medical University; Y. Katsumata, MD, PhD, Institute of Rheumatology, Tokyo Women's Medical University; N. Nishino, MD, Institute of Rheumatology, Tokyo Women's Medical University; S. Hirahara, MD, Institute of Rheumatology, Tokyo Women's Medical University; Y. Kawaguchi, MD, PhD, Institute of Rheumatology, Tokyo Women's Medical University; M. Kuwana, MD, PhD, Department of Allergy and Rheumatology, Nippon Medical School Graduate School of Medicine; H. Yamanaka, MD, PhD, Institute of Rheumatology, Tokyo Women's Medical University. Address correspondence to Dr. Y. Katsumata, Institute of Rheumatology, Tokyo Women's Medical University, 10-22 Kawada-cho, Shinjuku-ku, Tokyo 162-0054, Japan. E-mail: Accepted for publication January 19, 2018.
    Objective: We elucidated the association of serum soluble CD163 (sCD163) with rapidly progressive interstitial lung disease (RP-ILD), autoantibody profiles, and serum ferritin in patients with polymyositis (PM), classic dermatomyositis (DM), and clinical amyopathic dermatomyositis (CADM).

    Methods: Serum sCD163 levels were retrospectively measured by ELISA in patients with PM, classic DM, and CADM, as well as in healthy controls (HC). Repeat sera samples were obtained posttreatment from available patients. Read More

    Isolated Acute Appendicitis Caused by Aspergillus in a Patient Who Underwent Lung Transplantation: A Case Report.
    Transplant Proc 2018 Apr 11. Epub 2018 Apr 11.
    Research Institute for Convergence of Biomedical Science and Technology, Pusan National University Yangsan Hospital, Yangsan, Republic of Korea; Department of Thoracic and Cardiovascular Surgery, Pusan National University Yangsan Hospital, Yangsan, Republic of Korea.
    Invasive aspergillosis is an important cause of morbidity and mortality in patients who have undergone lung transplantation. Aspergillus infections usually involve the respiratory tract, with vascular invasion and subsequent dissemination. However, acute appendicitis associated with localized aspergillosis is rare, especially among patients who have undergone prophylaxis with voriconazole. Read More

    Classification of myositis.
    Nat Rev Rheumatol 2018 May 12;14(5):269-278. Epub 2018 Apr 12.
    Department of Dermatology, Perelman School of Medicine, University of Pennsylvania, Philadelphia, PA, USA.
    The idiopathic inflammatory myopathies (IIMs; also known as myositis) are a heterogeneous group of disorders in which a common feature is chronic inflammation of skeletal muscle, leading to muscle weakness. Other organs are frequently affected in IIMs, such as the skin, joints, lungs, gastrointestinal tract and heart, contributing to morbidity and mortality. Currently, IIMs are most often subclassified into polymyositis, dermatomyositis and inclusion body myositis, but this subclassification has limitations as these subgroups often have overlapping clinical and histopathological features, and outcomes vary within the subgroups; additionally, subgroups without considerable myopathy are not included. Read More

    Update on outcome assessment in myositis.
    Nat Rev Rheumatol 2018 May 12;14(5):303-318. Epub 2018 Apr 12.
    Istituto Giannina Gaslini, Clinica Pediatria e Reumatologia, PRINTO, Genoa, Italy.
    The adult and juvenile myositis syndromes, commonly referred to collectively as idiopathic inflammatory myopathies (IIMs), are systemic autoimmune diseases with the hallmarks of muscle weakness and inflammation. Validated, well-standardized measures to assess disease activity, known as core set measures, were developed by international networks of myositis researchers for use in clinical trials. Composite response criteria using weighted changes in the core set measures of disease activity were developed and validated for adult and juvenile patients with dermatomyositis and adult patients with polymyositis, with different thresholds for minimal, moderate and major improvement in adults and juveniles. Read More

    Successful treatment of plasma exchange for rapidly progressive interstitial lung disease with anti-MDA5 antibody-positive dermatomyositis: A case report.
    Medicine (Baltimore) 2018 Apr;97(15):e0436
    Department of Rheumatology, Unit of Advanced Preventive Medical Sciences, Graduate School of Bio-medical Sciences, Nagasaki University.
    Rationale: As the initial treatment of rapidly progressive interstitial lung disease (RPILD) with antimelanoma differentiation-associated gene 5 antibody (anti-MDA5 Ab)-positive dermatomyositis (DM) patients, a combination of corticosteroids, cyclophosphamide, and calcineurin inhibitor is recommended. However, some of these patients have poor prognoses despite such intensive treatment. Other more effective treatments are desired. Read More

    Panniculitis associated with amyopathic dermatomyositis.
    An Bras Dermatol 2018 Jan-Feb;93(1):119-121
    Department of Pathology, Faculdade de Medicina de Jundiaí (FMJ) - Jundiaí (SP), Brasil.
    Panniculitis is a rare clinical finding in dermatomyositis, with less than 30 cases reported and there is only one case associated with the amyopathic subtype described in the literature. The present report describes a 49-year-old female patient that one year after being diagnosed with amyopathic dermatomyositis, presented indurated, painful, erythematous to violaceous nodules located on the upper limbs, thighs and gluteal region. Skin biopsy revealed lobular panniculitis with a lymphocytic infiltrate. Read More

    Hyaluronic acid in dermatomyositis and polymyositis: relationship with disease and cutaneous lesions.
    An Bras Dermatol 2018 Jan-Feb;93(1):72-75
    Discipline of Rheumatology, Faculdade de Medicina, Universidade de Sao Paulo (USP) - Sao Paulo (SP), Brazil.
    Background: There are scarce studies in the literature about hyaluronic acid in systemic autoimmune myopathies.

    Objectives: To analyze the serum level of hyaluronic acid in patients with dermatomyositis and polymyositis.

    Methods: Cross-sectional study, single-center, that evaluated hyaluronic acid in 18 dermatomyositis and 15 polymyositis (Bohan and Peter criteria), newly diagnosed, with clinical and laboratory activity, with no previous drug treatment. Read More

    Ajulemic acid: potential treatment for chronic inflammation.
    Pharmacol Res Perspect 2018 Apr;6(2):e00394
    Department of Biochemistry & Molecular Pharmacology, University of Massachusetts Medical School, Worcester, MA, USA.
    Ajulemic acid (AJA, CT-3, IP-751, JBT-101, anabasum) is a first-in-class, synthetic, orally active, cannabinoid-derived drug that preferentially binds to the CB2 receptor and is nonpsychoactive. In preclinical studies, and in Phase 1 and 2 clinical trials, AJA showed a favorable safety, tolerability, and pharmacokinetic profile. It also demonstrated significant efficacy in preclinical models of inflammation and fibrosis. Read More

    Dermatomyositis Clinical and Pathological Phenotypes Associated with Myositis-Specific Autoantibodies.
    Curr Rheumatol Rep 2018 Apr 10;20(5):28. Epub 2018 Apr 10.
    Department of Dermatology, Stanford University School of Medicine, 450 Broadway Street, Redwood City, CA, 94063, USA.
    Purpose Of Review: Dermatomyositis is an idiopathic inflammatory myopathy with a variety of systemic and cutaneous manifestations. The myositis-specific autoantibodies (MSAs) are associated with phenotypic features and provide a tool for sub-classification of dermatomyositis patients. This review focuses on recent work characterizing the clinical features that accompany the MSAs in dermatomyositis. Read More

    Innovative Research Design to Meet the Challenges of Clinical Trials for Juvenile Dermatomyositis.
    Curr Rheumatol Rep 2018 Apr 10;20(5):29. Epub 2018 Apr 10.
    Università degli Studi di Genova, Genoa, Italy.
    Purpose Of Review: This paper aims to provide a summary of the recent therapeutic advances and the latest research on outcome measures for clinical trials in juvenile dermatomyositis (JDM).

    Recent Findings: Recent randomized controlled trials (RCTs) have demonstrated the superiority of the combination of prednisone with methotrexate over other conventional therapies and the potential effectiveness of rituximab in refractory cases. A multinational project has led to develop new criteria for the definition of minimal, moderate, and major improvement in future JDM clinical trials. Read More

    Potential of Krebs von den Lungen-6 as a predictor of relapse in interstitial pneumonia with anti-aminoacyl tRNA synthetase antibodies-positive dermatomyositis.
    Clin Respir J 2018 Apr 6. Epub 2018 Apr 6.
    Department of Internal Medicine (IV), Osaka Medical College, Osaka, Japan.
    Objective: To identify a predictor of relapse in interstitial pneumonia (IP) in patients with anti-aminoacyl tRNA synthetase antibodies-positive dermatomyositis (ARS-DMIP).

    Methods: This retrospective cohort study comprised 27 ARS-DMIP patients. We compared clinical and laboratory findings between the relapse and non-relapse groups during 2 years after treatment initiation to find predictors of relapse in IP. Read More

    Autoantibodies Associated With Connective Tissue Diseases: What Meaning for Clinicians?
    Front Immunol 2018 26;9:541. Epub 2018 Mar 26.
    Department of Internal Medicine, Infectious Diseases, and Clinical Immunology, Reims Teaching Hospitals, Robert Debré Hospital, Reims, France.
    Connective tissue diseases (CTDs) such as systemic lupus erythematosus, systemic sclerosis, myositis, Sjögren's syndrome, and rheumatoid arthritis are systemic diseases which are often associated with a challenge in diagnosis. Autoantibodies (AAbs) can be detected in these diseases and help clinicians in their diagnosis. Actually, pathophysiology of these diseases is associated with the presence of antinuclear antibodies. Read More

    [Dermatomyositis and Autoantibodies].
    Brain Nerve 2018 Apr;70(4):427-438
    Department of Dermatology, Faculty of Medicine, University of Tsukuba.
    Recent studies have identified novel dermatomyositis-specific autoantibodies and revealed that disease-specific autoantibodies become positive at a high rate in this disease. Moreover, these autoantibodies have been demonstrated to show a strong correlation with distinct clinical manifestations and complications such as interstitial lung disease and malignancy. Thus, these autoantibodies are now recognized as useful tools to classify this varied disease into more homogeneous subsets. Read More

    Gastrointestinal Tract Vasculopathy: Clinicopathology and Description of a Possible "New Entity" With Protean Features.
    Am J Surg Pathol 2018 Apr 5. Epub 2018 Apr 5.
    Department of Pathology, Stanford University School of Medicine, Stanford, CA.
    Noninfectious gastrointestinal (GI) vasculopathic disorders are rare and are often overlooked in histopathologic examination or when forming differential diagnoses due to their rarity. However, involvement of the GI tract may lead to serious complications, including ischemia and perforation. Since awareness of the types of vasculopathy that may involve the GI tract is central to arriving at a correct diagnosis, we reviewed our institutional experience with GI tract vasculopathy in order to enhance diagnostic accuracy of these rare lesions. Read More

    Interleukin-23 as a therapeutic target for inflammatory myopathy.
    Sci Rep 2018 Apr 3;8(1):5498. Epub 2018 Apr 3.
    Department of Rheumatology, Graduate School of Medical and Dental Sciences, Tokyo Medical and Dental University (TMDU), Tokyo, Japan.
    Current treatments of polymyositis and dermatomyositis (PM/DM) depend on non-specific immunosuppressants. This study was performed to elucidate the role of interleukin (IL)-23, as their possible therapeutic target. As was reported earlier in PM/DM patients, serum IL-23 levels were elevated in mice with C protein induced-myositis (CIM), a murine model of PM. Read More

    Advances in biomarkers for dermatomyositis.
    Clin Chim Acta 2018 Mar 31;482:172-177. Epub 2018 Mar 31.
    Department of Clinical Laboratory,The Second Affiliated Hospital of Nanchang University, Jiangxi Province Key Laboratory of Laboratory Medicine, Nanchang 330006, Jiangxi, China. Electronic address:
    Dermatomyositis (DM) and polymyositis (PM) are heterogeneous complex autoimmune diseases involving muscle damage. Patients with DM and PM display a wide spectrum of clinical manifestations and serological biomarkers, which may mislead and delay the proper diagnosis. Therefore, specific biomarkers or indicators for diagnosing DM and PM and monitoring disease activity are essential. Read More

    Potential association of LMNA-associated generalized lipodystrophy with juvenile dermatomyositis.
    Clin Diabetes Endocrinol 2018 27;4. Epub 2018 Mar 27.
    2Metabolism Endocrinology and Diabetes Division, Department of Internal Medicine, University of Michigan and Brehm Center for Diabetes, 1000 Wall Street, Room 5313, Ann Arbor, MI MI48105 USA.
    Background: Juvenile dermatomyositis (JDM) is an auto-immune muscle disease which presents with skin manifestations and muscle weakness. At least 10% of the patients with JDM present with acquired lipodystrophy. Laminopathies are caused by mutations in the lamin genes and cover a wide spectrum of diseases including muscular dystrophies and lipodystrophy. Read More

    [Idiopathic inflammatory myopathies].
    Vnitr Lek 2018 ;64(2):155-163
    Idiopathic inflammatory myopathies form a heterogeneous group of acquired inflammatory diseases afflicting striated muscles. The disease is frequently accompanied by systemic and organ involvement. Dermatomyositis, polymyositis, cancer associated myositis, immune mediated necrotizing myopathy, myositis in overlap syndromes, juvenile myositis and inclusion body myositis are the most frequently encountered subtypes. Read More

    Triggers of inflammatory myopathy: insights into pathogenesis.
    Discov Med 2018 Feb;25(136):75-83
    Department of Medicine Division of Rheumatology, Johns Hopkins University School of Medicine, Baltimore, MD 21287, USA.
    The inflammatory myopathies, which include dermatomyositis, polymyositis, and the immune-mediated necrotizing myopathies, are a heterogeneous group of autoimmune diseases that manifest with muscle, skin, or lung damage. Collectively, these autoimmune diseases result from loss of tolerance to a select group of self-antigens, although the precise mechanism through which this occurs is not known. Infection, malignancy, and certain medications including statins and the immune checkpoint inhibitors used in cancer therapy have been identified as potential immunologic triggers of the inflammatory myopathies. Read More

    Calcinosis of the mandible in dermatomyositis.
    Arthritis Rheumatol 2018 Mar 26. Epub 2018 Mar 26.
    Department of Medicine, Division of Rheumatology, Johns Hopkins University School of Medicine, 5200 Eastern Avenue, MFL Building, Center Tower, Suite 4100 Baltimore, MD 21224, USA.
    A 52-year-old woman presented to the Rheumatology clinic complaining of new tender nodules along the inferior border of her jaw. She has an 8-year history of amyopathic dermatomyositis with anti-MDA5 antibodies complicated by interstitial lung disease. Her current treatments include tacrolimus, prednisone and hydroxychloroquine. Read More

    Venous thromboembolic events in idiopathic inflammatory myopathy - occurrence and relation to disease onset.
    Arthritis Care Res (Hoboken) 2018 Mar 26. Epub 2018 Mar 26.
    Department of Medicine Solna, Rheumatology Unit, Karolinska Institutet and Rheumatology Clinic Karolinska University Hospital, Stockholm, Sweden.
    Objectives: To assess the incidence of venous thromboembolism (VTE) in patients with idiopathic inflammatory myopathies (IIM), and compare the incidence in IIM to the incidence in the general population. To identify patient categories at high risk and investigate the development of risk in relation to IIM diagnosis.

    Methods: Using nationwide registers we identified a cohort of 440 individuals with newly diagnosed IIM and 4459 individuals from the general population. Read More

    Erythroderma and extensive poikiloderma - a rare initial presentation of dermatomyositis: a case report.
    J Med Case Rep 2018 Mar 24;12(1):83. Epub 2018 Mar 24.
    National Hospital, Colombo, Sri Lanka.
    Background: Dermatomyositis is a humoral-mediated inflammatory myopathy with symmetrical proximal muscle weakness and dermatological manifestations such as Gottron's papules, heliotrope rash, periungual abnormalities, and flagellate erythema. Erythroderma is a severe and potentially life-threatening dermatological condition with diffuse erythema and scaling involving more than 90% of the skin surface area. Poikiloderma vasculare atrophicans refers to mottled hyperpigmentation and hypopigmentation of the skin with in-between telangiectases and areas of atrophy and is considered a variant of mycosis fungoides. Read More

    hyperinfection in an unconscious diabetic patient with dermatomyositis.
    Indian J Pathol Microbiol 2018 Jan-Mar;61(1):109-112
    Center for Research of Endemic Parasites of Iran, Tehran University of Medical Sciences; Department of Medical Parasitology and Mycology, School of Public Health, Tehran University of Medical Sciences, Tehran, Iran.
    A case of Strongyloides stercoralis hyperinfection in a patient with dermatomyositis and diabetes mellitus is herein reported. The case was a 60-year-old female admitted due to watery diarrhea and unconsciousness. She had a 10-year history of chronic immunosuppressive therapy including methotrexate and prednisolone for dermatomyositis. Read More

    New Myositis Classification Criteria-What We Have Learned Since Bohan and Peter.
    Curr Rheumatol Rep 2018 Mar 17;20(4):18. Epub 2018 Mar 17.
    Rheumatology Unit, Department of Medicine, Solna, Karolinska Institutet and Karolinska University Hospital, Rheumatology Unit, Karolinska University Hospital, SE-171 76, Stockholm, Sweden.
    Purpose Of Review: Idiopathic inflammatory myopathy (IIM) classification criteria have been a subject of debate for many decades. Despite several limitations, the Bohan and Peter criteria are still widely used. The aim of this review is to discuss the evolution of IIM classification criteria. Read More

    The prevalence and clinical significance of anti-PUF60 antibodies in patients with idiopathic inflammatory myopathy.
    Clin Rheumatol 2018 Mar 15. Epub 2018 Mar 15.
    Department of Rheumatology, Beijing Key Lab for Immune-Mediated Inflammatory Diseases, China-Japan Friendship Hospital, Yinghua East Road, Chaoyang District, Beijing, 100029, China.
    Autoantibodies against poly-U-binding factor 60 kDa protein (PUF60) have been reported in Caucasian dermatomyositis (DM) patients. However, their clinical significance in idiopathic inflammatory myopathy (IIM) remains to be fully clarified. Our objective was to analyze the prevalence and clinical significance of anti-PUF60 antibodies in a large cohort of Chinese IIM patients. Read More

    Bench to bedside review of myositis autoantibodies.
    Clin Mol Allergy 2018 7;16. Epub 2018 Mar 7.
    Experimental and Clinical Medicine Department, University of Florence, Largo Brambilla 3, 50134 Florence, Italy.
    Idiopathic inflammatory myopathies represent a heterogeneous group of autoimmune diseases with systemic involvement. Even though numerous specific autoantibodies have been recognized, they have not been included, with the only exception of anti-Jo-1, into the 2017 Classification Criteria, thus perpetuating a clinical-serologic gap. The lack of homogeneous grouping based on the antibody profile deeply impacts the diagnostic approach, therapeutic choices and prognostic stratification of these patients. Read More

    Myositis-specific autoantibodies in Japanese patients with juvenile idiopathic inflammatory myopathies.
    Mod Rheumatol 2018 Apr 9:1-6. Epub 2018 Apr 9.
    a Department of Pediatrics, Faculty of Medicine and Graduate School of Medicine , Hokkaido University , Sapporo , Japan.
    Objectives: The aim of our study is to clarify the association of myositis-specific autoantibodies (MSAs) with clinical and laboratory features in Japanese patients with juvenile idiopathic inflammatory myopathies (JIIMs).

    Methods: We retrospectively analyzed the frequency of MSAs and their association with clinical or laboratory findings in 25 Japanese patients with JIIMs in Hokkaido district.

    Results: Eighteen of the 25 patients (72%) were positive for MSAs; seven with anti-melanoma differentiation associated gene (MDA) 5 (28%), five with anti-transcriptional intermediary factor (TIF)-1γ (20%), four with anti-MJ/nuclear matrix protein (NXP)-2 (16%), two with anti-Jo-1 (8%), one with anti- HMG-CoA reductase, one with anti-signal recognition peptide (SRP) antibodies (4% each), including co-existence and transition of MSAs in one patient each. Read More

    Monoclonal gammopathy in rheumatic diseases.
    Clin Rheumatol 2018 Mar 13. Epub 2018 Mar 13.
    Department of Rheumatology and Immunology, Peking University People's Hospital, Beijing, China.
    To analyze the clinical spectrum, laboratory characteristics, and outcomes of monoclonal gammopathy (MG) in patients with rheumatic diseases. Screening for the presence of MG was performed in 872 inpatients with rheumatic diseases from January 2010 to July 2017. A total of 41 patients were enrolled. Read More

    Breast cancer with dermatomyositis as initial presentation.
    Med J Malaysia 2018 Feb;73(1):44-45
    Penang General Hospital, Department of General Surgery, Penang, Malaysia.
    Patients with breast cancer normally present with breast lump or abnormal mammogram. Dermatomyositis is rarely the first presentation. We present a case of a 63-year-old woman who had generalised dermatitis, progressive fatigue and muscle weakness. Read More

    Improved quantification of a commercial enzyme-linked immunosorbent assay kit for measuring anti-MDA5 antibody.
    Mod Rheumatol 2018 Apr 9:1-6. Epub 2018 Apr 9.
    a Department of Allergy and Rheumatology , Nippon Medical School Graduate School of Medicine , Tokyo , Japan.
    Objectives: To compare the quantitative performance for measuring anti-MDA5 antibody titer of two enzyme-linked immunosorbent assay (ELISA) systems: an in-house ELISA and the commercial MESACUP anti-MDA5 test.

    Methods: Anti-MDA5 antibody titer was measured in sera from 70 patients with dermatomyositis using an in-house ELISA and the MESACUP anti-MDA5 test side-by-side. For the commercial ELISA kit, serum samples diluted 1:101 were used according to the manufacturer's protocol, but serial dilutions of sera were also examined to identify the optimal serum dilution for quantification. Read More

    A Case of Advanced Lung Adenocarcinoma with Nvolumab-associated Dermatomyositis.
    Intern Med 2018 Mar 9. Epub 2018 Mar 9.
    Department of Pulmonary Medicine, Jichi Medical University, Saitama Medical Center, Japan.
    We herein report a 42-year-old man with advanced lung adenocarcinoma and nivolumab-associated dermatomyositis. Nivolumab, an anticancer drug that is classified as an immune checkpoint inhibitor, often induces immune-related adverse events (irAEs). However, there have so far been no reports regarding nivolumab-associated dermatomyositis. Read More

    Anti-PL-7 Antisynthetase Syndrome with Eosinophilic Pleural Effusion.
    Intern Med 2018 Mar 9. Epub 2018 Mar 9.
    Department of Pulmonary Medicine, Seirei Hamamatsu General Hospital, Japan.
    A 68-year-old woman was admitted to our hospital with fever and pleural effusion. Her thoracentesis showed eosinophilic pleural effusion (EPE) without any evidence of malignancy, infection, or trauma. Pleural biopsy revealed pleuritis and intercostal myositis. Read More

    Dermatomyositis panniculitis: a clinicopathological and immunohistochemical study of 18 cases.
    J Eur Acad Dermatol Venereol 2018 Mar 10. Epub 2018 Mar 10.
    Department of Dermatology, Fundación Jiménez Diaz, Universidad Autónoma, Madrid, Spain.
    Background: Panniculitis occurring in dermatomyositis is uncommon, with only a few cases described in the literature, most of them as case reports.

    Objective: This report describes the clinicopathological and immunohistochemical findings in a series of 18 patients with panniculitis associated with dermatomyositis.

    Methods: In each patient, we collected the clinical data of the cutaneous lesions as well as the characteristic clinical and laboratory findings. Read More

    Revisiting Pathological Classification Criteria for Adult Idiopathic Inflammatory Myopathies: In-Depth Analysis of Muscle Biopsies and Correlation Between Pathological Diagnosis and Clinical Manifestations.
    J Neuropathol Exp Neurol 2018 May;77(5):395-404
    Research Institute of Neuromuscular and Neurodegenerative Diseases and Department of Neurology, Qilu Hospital, Shandong University, Jinan, Shandong, China.
    The European Neuromuscular Centre (ENMC) pathological classification criteria of idiopathic inflammatory myopathies (IIMs) are debatable. The aim of this study was to explore their practicability and reproducibility. We conducted a retrospective analysis of 57 cases of IIMs excluding dermatomyositis (DM) and sporadic inclusion body myositis (sIBM) by in-depth analysis of muscle biopsies and comparisons of the clinical characteristics among polymyositis (PM), non-specific myositis (NSM) and necrotizing autoimmune myopathy (NAM). Read More

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