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    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 Apr 18. 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 Apr 13. 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

    Original article: Varicella vaccination elicits a humoral and cellular response in children with rheumatic diseases using immune suppressive treatment.
    Vaccine 2017 Apr 12. 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

    Race, Income, and Disease Outcomes in Juvenile Dermatomyositis.
    J Pediatr 2017 May 3;184:38-44.e1. Epub 2017 Mar 3.
    Division of Rheumatology, University of California at San Francisco, Benioff Children's Hospital, San Francisco, CA.
    Objective: To determine the relationships among race, income, and disease outcomes in children with juvenile dermatomyositis (JDM).

    Study Design: Data from 438 subjects with JDM enrolled in the Childhood Arthritis and Rheumatology Research Alliance (CARRA) Legacy Registry were analyzed. Demographic data included age, sex, race, annual family income, and insurance status. Read More

    Accidental hydroxychloroquine overdose resulting in neurotoxic vestibulopathy.
    BMJ Case Rep 2017 Apr 12;2017. Epub 2017 Apr 12.
    Department of Dermatology, University of Pennsylvania, Philadelphia, Pennsylvania, USA
    Hydroxychloroquine is an oral antimalarial medication commonly used off-label for a variety of rheumatological conditions, including systemic lupus erythematosus, rheumatoid arthritis, Sjögren's syndrome and dermatomyositis. We present a case of a 64-year-old woman who presented with acute onset headache, bilateral tinnitus, and left-sided facial numbness and tingling in the setting of accidentally overdosing on hydroxychloroquine. By the next morning, the patient began to experience worsening in the tingling sensation and it eventually spread to her left arm, thigh and distal extremities. Read More

    Muscle MRI at the time of questionable disease flares in Juvenile Dermatomyositis (JDM).
    Pediatr Rheumatol Online J 2017 Apr 12;15(1):25. Epub 2017 Apr 12.
    Nationwide Children's Hospital, 700 Children's Dr, Columbus, OH, 43205, USA.
    Background: The course of JDM has improved substantially over the last 70 years with early and aggressive treatments. Yet it remains difficult to detect disease flares as symptoms may be mild; signs of rash and muscle weakness vary widely and are often equivocal; laboratory tests of muscle enzyme levels are often normal; electromyography and muscle biopsy are invasive. Alternative tools are needed to help decide if more aggressive treatment is needed. Read More

    Lichenoid and interface dermatoses.
    Semin Diagn Pathol 2017 Mar 22. Epub 2017 Mar 22.
    Department of Radiation Oncology, The Ohio State University, Columbus, OH, United States.
    The terms 'lichenoid' and 'interface' dermatitis are often used interchangeably to describe an inflammatory pattern characterized histologically by damage to the basal keratinocytes in the epidermis. The mechanism of cell damage of such cells is now best understood as apoptosis, or programmed cell death. This inflammatory pattern of dermatoses, is also accompanied frequently by a band of lymphocytes and histiocytes in the superficial dermis, that often obscures the dermal-epidermal junction, hence the term 'lichenoid'. Read More

    Coexisting Juvenile Dermatomyositis and Sickle Cell Disease: Maintaining a High Degree of Suspicion.
    J Pediatr 2017 Apr 7. Epub 2017 Apr 7.
    Department of Pediatrics, Nemours/A.I. duPont Hospital for Children, Wilmington, DE; Nemours Center for Cancer and Blood Disorders, Wilmington, DE.
    Juvenile dermatomyositis is an idiopathic inflammatory myopathy of childhood not previously described in a patient with sickle cell disease. We present a case of an 11-year-old girl with sickle cell disease who was diagnosed subsequently with juvenile dermatomyositis, and highlight the diagnostic and therapeutic challenges of these concurrent chronic diseases. Read More

    Subcutaneous Emphysema, Pneumomediastinum and Pneumothorax in a Patient with Dermatomyositis.
    Iran J Otorhinolaryngol 2017 Mar;29(91):113-116
    Department of Otorhinolaryngology-Head & Neck Surgery, Mashhad University of Medical Sciences, Mashhad, Iran.
    Introduction: Spontaneous pneumomediastinum, pneumothorax, and subcutaneous emphysema are rare, but serious complications of inflammatory myopathies and occur more commonly in DM than PM. complications of dermatomyositis (DM) and polymyositis (PM), both of which can be fatal.

    Case Report: A 20-year-old woman was admitted with neck pain, dyspnea, cough, and fever. Read More

    A case of anti-aminoacyl tRNA synthetase (ARS) antibody-positive polymyositis (PM)/dermatomyositis (DM)-associated interstitial pneumonia (IP) successfully controlled with bosentan therapy.
    Respir Med Case Rep 2017 29;21:62-65. Epub 2017 Mar 29.
    Department of Pulmonary Medicine and Oncology, Graduate School of Medicine, Nippon Medical School, Tokyo, Japan.
    A 72-year-old woman was admitted to our hospital and was diagnosed with interstitial pneumonia (IP) associated with amyopathic dermatomyositis (ADM). The patient experienced three acute IP exacerbations in the 7 years that followed, which were each treated and resolved with steroid pulse therapy. The patient was closely examined for respiratory failure with right heart catheterization (RHC), which demonstrated that she had a mean pulmonary artery pressure (mPAP) of 34 mmHg. Read More

    "Hiker's feet": a novel cutaneous finding in the inflammatory myopathies.
    Clin Rheumatol 2017 Apr 7. Epub 2017 Apr 7.
    Department of Medicine; Division of Rheumatology, Johns Hopkins University School of Medicine, Baltimore, MD, USA.
    Mechanic's hands is a well-characterized manifestation of select idiopathic inflammatory myopathy (IIM) syndromes. Less well characterized is the hyperkeratosis of the toes and plantar surface of the feet that can also accompany these disorders. We aim to describe common pedal signs in the context of IIM, and suggest that it may be another key feature in the presentation of these syndromes. Read More

    Pregnancy in polymyositis or dermatomyositis: retrospective results from a tertiary centre in China.
    Rheumatology (Oxford) 2017 Apr 5. Epub 2017 Apr 5.
    Department of Rheumatology, Peking Union Medical College Hospital, Peking Union Medical College & Chinese Academy of Medical Science, Key Laboratory of Rheumatology and Clinical Immunology, Ministry of Education, Beijing.
    Objective: . To examine if patients with PM/DM are at higher risk of complicated pregnancies.

    Methods: . Read More

    The epidemiology of dermatomyositis in northwestern Thrace region in Turkey: epidemiology of dermatomyositis in Turkey.
    Rheumatol Int 2017 Apr 6. Epub 2017 Apr 6.
    Medicine, Department of Rheumatology, Trakya University Medical Faculty, 22030, Edirne, Turkey.
    Dermatomyositis (DM) is a rare disease that may affect the skeletal muscles and the skin. Literature data on its incidence and prevalence are limited. There are no data on its incidence or prevalence in Turkey. Read More

    2016 American College of Rheumatology/European League Against Rheumatism criteria for minimal, moderate, and major clinical response in adult dermatomyositis and polymyositis: An International Myositis Assessment and Clinical Studies Group/Paediatric Rheumatology International Trials Organisation Collaborative Initiative.
    Ann Rheum Dis 2017 May;76(5):792-801
    Charles University, Prague, Czech Republic.
    To develop response criteria for adult dermatomyositis (DM) and polymyositis (PM). Expert surveys, logistic regression, and conjoint analysis were used to develop 287 definitions using core set measures. Myositis experts rated greater improvement among multiple pairwise scenarios in conjoint analysis surveys, where different levels of improvement in 2 core set measures were presented. Read More

    2016 American College of Rheumatology/European League Against Rheumatism Criteria for Minimal, Moderate, and Major Clinical Response in Juvenile Dermatomyositis: An International Myositis Assessment and Clinical Studies Group/Paediatric Rheumatology International Trials Organisation Collaborative Initiative.
    Ann Rheum Dis 2017 May;76(5):782-791
    Istituto Giannina Gaslini, Pediatria II - Reumatologia, PRINTO, Genoa, Italy.
    To develop response criteria for juvenile dermatomyositis (DM). We analysed the performance of 312 definitions that used core set measures from either the International Myositis Assessment and Clinical Studies Group (IMACS) or the Paediatric Rheumatology International Trials Organisation (PRINTO) and were derived from natural history data and a conjoint analysis survey. They were further validated using data from the PRINTO trial of prednisone alone compared to prednisone with methotrexate or cyclosporine and the Rituximab in Myositis (RIM) trial. Read More

    Inflammatory Myopathies with Cutaneous Involvement: from Diagnosis to Therapy.
    Folia Med (Plovdiv) 2017 Mar;59(1):7-13
    Department of Dermatology and Venereology, Medical University of Sofia, 1 St Georgi Sofiiski St., 1431 Sofia.
    The group of idiopathic inflammatory myopathies (IIM) include various disorders of skeletal muscles with or without skin involvement. The most common types are dermatomyositis (DM), polymyositis (PM), inclusion body myositis (IBM) and necrotizing autoimmune myopathy (NAM). Dermatomyositis subdivides into various clinical forms such as juvenile, amyopathic or paraneoplastic dermatomyositis, scleromyositis, overlap or anti-synthetase syndromes, etc. Read More

    2016 American College of Rheumatology/European League Against Rheumatism Criteria for Minimal, Moderate, and Major Clinical Response in Adult Dermatomyositis and Polymyositis: An International Myositis Assessment and Clinical Studies Group/Paediatric Rheumatology International Trials Organisation Collaborative Initiative.
    Arthritis Rheumatol 2017 Apr 6. Epub 2017 Apr 6.
    Charles University, Prague, Czech Republic.
    Objective: To develop response criteria for adult dermatomyositis (DM) and polymyositis (PM).

    Methods: Expert surveys, logistic regression, and conjoint analysis were used to develop 287 definitions using core set measures. Myositis experts rated greater improvement among multiple pairwise scenarios in conjoint analysis surveys, where different levels of improvement in 2 core set measures were presented. Read More

    2016 American College of Rheumatology/European League Against Rheumatism Criteria for Minimal, Moderate, and Major Clinical Response in Juvenile Dermatomyositis: An International Myositis Assessment and Clinical Studies Group/Paediatric Rheumatology International Trials Organisation Collaborative Initiative.
    Arthritis Rheumatol 2017 Apr 6. Epub 2017 Apr 6.
    Istituto Giannina Gaslini, Pediatria II - Reumatologia, PRINTO, Genoa, Italy.
    Objective: To develop response criteria for juvenile dermatomyositis (DM).

    Methods: We analyzed the performance of 312 definitions that used core set measures from either the International Myositis Assessment and Clinical Studies Group (IMACS) or the Paediatric Rheumatology International Trials Organisation (PRINTO) and were derived from natural history data and a conjoint analysis survey. They were further validated using data from the PRINTO trial of prednisone alone compared to prednisone with methotrexate or cyclosporine and the Rituximab in Myositis (RIM) trial. Read More

    Paraneoplastic Neurological Disorder in Nasopharyngeal Carcinoma.
    Malays J Med Sci 2017 Mar 24;24(1):113-116. Epub 2017 Feb 24.
    Department of Otorhinolaryngology, Head & Neck Surgery, UKM Medical Centre, Jalan Yaacob Latif, 56000 Cheras, Kuala Lumpur, Malaysia.
    Paraneoplastic neurological disorder (PND) is a condition due to immune cross-reactivity between the tumour cells and the normal tissue, whereby the "onconeural" antibodies attack the normal host nervous system. It can present within weeks to months before or after the diagnosis of malignancies. Nasopharyngeal carcinoma is associated with paraneoplastic syndrome, for example, dermatomyositis, and rarely with a neurological disorder. Read More

    Increased risk of comorbid rheumatic disorders in vitiligo patients: A nationwide population-based study.
    J Dermatol 2017 Mar 31. Epub 2017 Mar 31.
    Department of Dermatology, Veterans Health Service Medical Center, Seoul, Korea.
    Vitiligo is a common acquired depigmentation disorder. Previous studies have shown that vitiligo is associated with a variety of autoimmune disorders. However, a large-scale epidemiological study focused on comorbid rheumatic disorders has not been undertaken. Read More

    Submaximal Exercise Capacity in Juvenile Dermatomyositis after Longterm Disease: The Contribution of Muscle, Lung, and Heart Involvement.
    J Rheumatol 2017 Apr 1. Epub 2017 Apr 1.
    From the Department of Rheumatology, the Department of Cardiology, the Department of Radiology and Nuclear Medicine, and the Department of Respiratory Medicine, Oslo University Hospital, Rikshospitalet; Institute of Clinical Medicine, Faculty of Medicine, University of Oslo; Institute for Experimental Medical Research, Oslo University Hospital and University of Oslo; Norwegian National Advisory Unit on Rheumatic Diseases in Children and Adolescents, Oslo University Hospital, Rikshospitalet, Oslo, Norway. Supported by the Norwegian Extra Foundation for Health and Rehabilitation (2013/FOM5610) through the Norwegian Rheumatism Association. K.S. Berntsen, MD, Department of Rheumatology, Oslo University Hospital, Rikshospitalet; A. Tollisen, RN, MSc, Department of Rheumatology, Oslo University Hospital, Rikshospitalet; T. Schwartz, MD, PhD, Department of Cardiology, Oslo University Hospital, and Institute for Experimental Medical Research, Oslo University Hospital and University of Oslo; E. Kirkhus, MD, PhD, Department of Radiology and Nuclear Medicine, Oslo University Hospital; T.M. Aaløkken, MD, PhD, Department of Radiology and Nuclear Medicine, Oslo University Hospital; M.B. Lund, MD, PhD, Department of Respiratory Medicine, Oslo University Hospital, Rikshospitalet; B. Flatø, MD, PhD, Department of Rheumatology, Oslo University Hospital, Rikshospitalet, and Institute of Clinical Medicine, Faculty of Medicine, University of Oslo; I. Sjaastad, MD, PhD, Department of Cardiology, Oslo University Hospital, and Institute for Experimental Medical Research, Oslo University Hospital and University of Oslo; H. Sanner, MD, PhD, Department of Rheumatology, and Norwegian National Advisory Unit on Rheumatic Diseases in Children and Adolescents, Oslo University Hospital, Rikshospitalet. Address correspondence to Dr. K.S. Berntsen, Oslo University Hospital, Rikshospitalet, Department of Rheumatology, P.O. Box 4950, 0424 Oslo, Norway. E-mail: Accepted for publication February 14, 2017.
    Objective: To compare submaximal exercise capacity in patients with juvenile dermatomyositis (JDM) with controls, and analyze contributions of muscle, heart, and lung impairment in patients.

    Methods: Fifty-nine patients with JDM, with a mean 16.9 years after symptom onset, and 59 sex- and age-matched controls completed a 6-min walk test (6MWT) and a timed up and go (TUG) test. Read More

    Subclinical Cardiac Dysfunction in Polymyositis and Dermatomyositis: A Speckle-tracking Case-control Study.
    J Rheumatol 2017 Apr 1. Epub 2017 Apr 1.
    From the Cardiology and Arrhythmology Clinic, and the Clinical Medicine, Clinical and Molecular Sciences Department, Marche Polytechnic University, University Hospital "Ospedali Riuniti," Ancona, Italy. F. Guerra, MD, Cardiology and Arrhythmology Clinic, Marche Polytechnic University, University Hospital Ospedali Riuniti; C. Gelardi, MD, Clinical Medicine, Clinical and Molecular Sciences Department, Marche Polytechnic University, University Hospital Ospedali Riuniti; A. Capucci, MD, Professor, Cardiology and Arrhythmology Clinic, Marche Polytechnic University, University Hospital Ospedali Riuniti; A. Gabrielli, MD, Professor, Clinical Medicine, Clinical and Molecular Sciences Department, Marche Polytechnic University, University Hospital Ospedali Riuniti; M.G. Danieli, MD, PhD, Associate Professor, Clinical Medicine, Clinical and Molecular Sciences Department, Marche Polytechnic University, University Hospital Ospedali Riuniti. Address correspondence to Prof. M.G. Danieli, Clinica Medica, Dipartimento di Scienze Cliniche e Molecolari, Via Tronto 10, Torrette di Ancona, 60126 Italy. E-mail: Accepted for publication February 14, 2017.
    Objective: Subclinical heart disease occurs in up to 50% of patients with idiopathic inflammatory myopathies (IIM) and is difficult to detect through conventional imaging. We investigated the usefulness of global longitudinal strain (GLS) measurement to detect a subclinical systolic ventricular dysfunction in patients with IIM.

    Methods: We enrolled 28 patients with IIM and 28 matched controls in a 1:1 fashion. Read More

    Trichilemmal cystis in metastatic melanoma: a case report.
    Exp Oncol 2017 03;39(1):86-87
    Department of Dermatology, Department of Critical Care Medicine and Surgery, University of Florence, Firenze 50121, 50132, Italy.
    The malignant melanoma is a neoplasm associated with a wide variety of cutaneous paraneoplastic syndromes, as dermatomyositis, systemic sclerosis, paraneoplastic pemphigus. We describe a case of four multiple trichilemmal cystis arising on frontal region in the same patient with brain metastasis and unknown primary melanoma and discuss their relationship. Read More

    Methods for analyzing observational longitudinal prognosis studies for rheumatic diseases: a review & worked example using a clinic-based cohort of juvenile dermatomyositis patients.
    Pediatr Rheumatol Online J 2017 Mar 29;15(1):18. Epub 2017 Mar 29.
    Institute of Health Policy Management and Evaluation, University of Toronto, Toronto, Canada.
    Most outcome studies of rheumatic diseases report outcomes ascertained on a single occasion. While single assessments are sufficient for terminal or irreversible outcomes, they may not be sufficiently informative if outcomes change or fluctuate over time. Consequently, longitudinal studies that measure non-terminal outcomes repeatedly afford a better understanding of disease evolution. Read More

    Hydroxychloroquine-induced hyperpigmentation in systemic diseases: prevalence, clinical features and risk factors: a cross-sectional study of 41 cases.
    Lupus 2017 Jan 1:961203317700486. Epub 2017 Jan 1.
    1 Department of Internal Medicine, Hedi Chaker Hospital, Sfax, Tunisia.
    Introduction Hydroxychloroquine is an antimalarial agent widely prescribed in internal medicine, rheumatology and dermatology. Its use can be complicated by various side effects including skin pigmentation. Objectives The aim of the study is to review epidemiological, clinical features and risk factors of hydroxychloroquine-induced pigmentation. Read More

    Value of whole-body magnetic resonance imaging for screening multifocal osteonecrosis in patients with polymyositis/dermatomyositis.
    Br J Radiol 2017 Mar 29:20160780. Epub 2017 Mar 29.
    2 Department of Rheumatology, China-Japan Friendship Hospital, Beijing, China.
    Objectives: To assess the value of coronal short-tau inversion recovery whole-body magnetic resonance imaging (STIR-WBMRI) for screening osteonecrosis in patients with polymyositis (PM)/dermatomyositis (DM).

    Methods: The imaging and medical records of 129 PM/DM patients who met the Bohan and Peter diagnostic criteria were retrospectively analyzed. STIR-WBMRI was performed in all patients. Read More

    TWEAK/Fn14 activation contributes to the pathogenesis of bullous pemphigoid.
    J Invest Dermatol 2017 Mar 25. Epub 2017 Mar 25.
    Department of Dermatology, The Second Affiliated Hospital, School of Medicine, Xi'an Jiaotong University, Xi'an, China. Electronic address:
    Tumor necrosis factor-like weak inducer of apoptosis (TWEAK) participates in various cellular effects by engaging its receptor of fibroblast growth factor inducible 14 (Fn14). Increased levels of soluble TWEAK are associated with systemic autoimmunity in patients with lupus erythematosus, rheumatoid arthritis or dermatomyositis. However, the role of TWEAK in bullous pemphigoid (BP) remains unknown. Read More

    DEPTOR-mTOR Signaling Is Critical for Lipid Metabolism and Inflammation Homeostasis of Lymphocytes in Human PBMC Culture.
    J Immunol Res 2017 27;2017:5252840. Epub 2017 Feb 27.
    Department of General Practice, West China Hospital of Sichuan University, Chengdu 610041, China.
    Abnormal immune response of the body against substances and tissues causes autoimmune diseases, such as polymyositis, dermatomyositis, and rheumatoid arthritis. Irregular lipid metabolism and inflammation may be a significant cause of autoimmune diseases. Although much progress has been made, mechanisms of initiation and proceeding of metabolic and inflammatory regulation in autoimmune disease have not been well-defined. Read More

    Intravenous immunoglobulin contributes to control anti-melanoma differentiation-associated protein 5 (MDA5) antibody-associated dermatomyositis with palmar violaceous macules/papules.
    Br J Dermatol 2017 Mar 27. Epub 2017 Mar 27.
    Department of Dermatology, Faculty of Medicine, University of Tsukuba, Ibaraki, Japan.
    Autoantibodies to melanoma differentiation-associated protein 5 (MDA5) are associated with a subset of dermatomyositis (DM) patients who have rapidly progressive interstitial lung disease (RP-ILD) with poor prognosis. Intensive immunosuppressive therapy is initiated before irreversible lung damage can occur; however, there are few lines of evidence for the treatment of RP-ILD. Here, we report 3 cases of anti-MDA5 antibody-associated DM with RP-ILD in which the patients were treated with combined modality therapy including high-dose prednisolone, tacrolimus, intravenous cyclophosphamide, and immunoglobulins (IVIG). Read More

    Red blood cell distribution width and neutrophil to lymphocyte ratio are correlated with disease activity of dermatomyositis and polymyositis.
    J Clin Lab Anal 2017 Mar 27. Epub 2017 Mar 27.
    Department of Rheumatology, Wuxi Second People's Hospital of Nanjing Medical University, Wuxi, China.
    Background: Previous studies indicated that both red blood cell distribution width (RDW) and neutrophil to lymphocyte ratio (NLR) were useful indices in assessing the disease activity of autoimmune diseases. However, the evidence for the association between RDW, NLR and dermatomyositis (DM) and polymyositis (PM) is limited. The aim of this study is to investigate the association between the disease activity of PM/DM and both RDW and NLR. Read More

    Analysis of sexual function of patients with dermatomyositis and polymyositis through self-administered questionnaires: a cross-sectional study.
    Rev Bras Reumatol Engl Ed 2017 Mar - Apr;57(2):134-140. Epub 2016 Dec 5.
    Universidade de São Paulo, Faculdade de Medicina, Disciplina de Reumatologia, São Paulo, SP, Brazil. Electronic address:
    Introduction: To date, there are no descriptions in the literature on gynecologic and sexual function evaluation in female patients with dermatomyositis (DM) and polymyositis (PM).

    Objective: To assess sexual function in female patients with DM/PM.

    Patients And Methods: This is a monocentric, cross-sectional study in which 23 patients (16 DM and 7 PM), with ages between 18 and 40 years, were compared to 23 healthy women of the same age group. Read More

    Inflammatory Muscle Disease: A New Landscape.
    Joint Bone Spine 2017 Mar 22. Epub 2017 Mar 22.
    Service de Rhumatologie, Hôpitaux Universitaires de Strasbourg, 67000 Strasbourg, France; Centre de Référence des Maladies Autoimmunes Rares, Hôpitaux Universitaires de Strasbourg, 67000 Strasbourg, France; Fédération de Médecine Translationnelle de Stasbourg, Université de Strasbourg, France.
    Greater accuracy in clinical descriptions combined with advances in muscle histology and immunology have established that inflammatory muscle diseases (IMDs) resemble inflammatory joint diseases in that they constitute a highly heterogeneous group of conditions. The topographic distribution, severity, and tempo of onset vary widely, and the histological findings distinguish at least five different profiles, which may reflect different pathophysiological processes. Most IMDs are connective tissue diseases that can affect multiple organs, among which the most common targets are the skin, joints, and lungs. Read More

    A longitudinal cohort study of the anti-synthetase syndrome: increased severity of interstitial lung disease in black patients and patients with anti-PL7 and anti-PL12 autoantibodies.
    Rheumatology (Oxford) 2017 Mar 4. Epub 2017 Mar 4.
    Johns Hopkins University School of Medicine, Baltimore, MD, USA.
    Objective.: The aim was to study the prevalence, rate of appearance and severity of clinical features in patients with different anti-synthetase syndrome (ASyS) autoantibodies.

    Methods. Read More

    Autoantibodies in children with juvenile dermatomyositis: A single centre experience from North-West India.
    Rheumatol Int 2017 May 22;37(5):807-812. Epub 2017 Mar 22.
    Department of Pediatrics, Advanced Pediatrics Centre, Post Graduate Institute of Medical Education and Research (PGIMER), Chandigarh, 160012, India.
    The objective of this study is to determine autoantibody profile in children with juvenile dermatomyositis (JDM). Children who were diagnosed with JDM (either recently diagnosed during the study period or follow-up patients) were included in the study. Autoantibodies were detected with commercially available Immunodot kit. Read More

    Calcium dysregulation, functional calpainopathy, and endoplasmic reticulum stress in sporadic inclusion body myositis.
    Acta Neuropathol Commun 2017 03 22;5(1):24. Epub 2017 Mar 22.
    Department of Kinesiology, University of Maryland College Park, College Park, MD, USA.
    Sporadic inclusion body myositis (IBM) is the most common primary myopathy in the elderly, but its pathoetiology is still unclear. Perturbed myocellular calcium (Ca(2+)) homeostasis can exacerbate many of the factors proposed to mediate muscle degeneration in IBM, such as mitochondrial dysfunction, protein aggregation, and endoplasmic reticulum stress. Ca(2+) dysregulation may plausibly be initiated in IBM by immune-mediated membrane damage and/or abnormally accumulating proteins, but no studies to date have investigated Ca(2+) regulation in IBM patients. Read More

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