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    Validation of a Brazilian Portuguese version of the transition readiness assessment questionnaire (TRAQ) in a population of transitional youth with chronic rheumatologic disorders.
    J Pediatr (Rio J) 2018 Feb 16. Epub 2018 Feb 16.
    University of Southern California, Keck School of Medicine, Children's Hospital Los Angeles, Division of Rheumatology, Los Angeles, United States.
    Objective: To translate and validate the Brazilian Portuguese version of the Transition Readiness Assessment Questionnaire in a population of adolescents and young adults with chronic rheumatologic disorders. This questionnaire evaluates the patient's readiness for making the transition from the pediatric health service to adult care.

    Methods: The four-phase methodology for the translation and validation of generic questionnaires was followed, including translation, back-translation, pilot testing and clinical validation of the final tool. Read More

    Clinical characteristics and factors associated with disability and impaired quality of life in children with juvenile systemic sclerosis.
    Arthritis Care Res (Hoboken) 2018 Feb 18. Epub 2018 Feb 18.
    Center for Immunity and Immunotherapies, Seattle Children's Research Institute, Seattle, WA.
    Objective: To investigate clinical manifestations of juvenile systemic sclerosis (jSSc), including disease characteristics and patient quality of life, through the multinational Childhood Arthritis and Rheumatology Research Alliance (CARRA) Legacy Registry.

    Methods: Subjects with jSSc were prospectively enrolled between 2010 and 2013. Diagnosis of jSSc was determined by the enrolling pediatric rheumatologist, with disease onset required prior to age 18. Read More

    The clinical phenotype of patients positive for antibodies to myositis and myositis-related disorders.
    Clin Rheumatol 2018 Feb 16. Epub 2018 Feb 16.
    Department of Medicine A, Sheba Medical Center, 5262000, Tel Hashomer, Israel.
    Inflammatory myopathies are a clinically diverse group of diseases, in which the detection of particular autoantibodies may facilitate diagnosis, treatment, and prognosis. The aim of this report is to summarize our experience with specific autoantibody testing in patients with inflammatory myopathies. Data were collected over the last decade in the Autoimmune Center of the Sheba Medical Center, a tertiary referral hospital. Read More

    A patient case highlighting the myriad of cutaneous adverse effects of prolonged use of hydroxyurea.
    Dermatol Online J 2017 Nov 15;23(11). Epub 2017 Nov 15.
    Division of Dermatology, University of Kansas Medical Center, Kansas City, Kansas.
    Background: Hydroxyurea is an antimetabolite primarily used to treat myeloproliferative disorders, and chronic treatment is associated with many cutaneous adverse effects ranging in severity from ichthyosis to aggressive nonmelanoma skin cancer.

    Case Presentation: We report a 67-year-oldman with a history of polycythemia vera who was referred for management of progressively worsening dorsal hand lesions. The patient presented withhyperpigmentation, ichthyosis, plantar keratoderma, dermatomyositis-like eruptions, two squamous cell carcinomas, and actinic keratoses. Read More

    Survival of adults with systemic autoimmune rheumatic diseases and pulmonary arterial hypertension after lung transplantation.
    Rheumatology (Oxford) 2018 Feb 13. Epub 2018 Feb 13.
    Department of Medicine, Columbia University Medical Center, New York, NY.
    Objectives: Pulmonary arterial hypertension (PAH) is a major cause of morbidity and mortality in adults with systemic autoimmune rheumatic diseases (ARDs). The aim of this study was to determine whether adults with ARDs and PAH on right-sided heart catheterization (ARD-PAH) have increased mortality following lung transplantation compared with those with PAH not due to an ARD.

    Methods: We conducted a retrospective cohort study of 93 adults with ARD-PAH and 222 adults with PAH who underwent lung transplantation in the USA between 4 May 2005 and 9 March 2015 using data from the United Network for Organ Sharing. Read More

    Livedo Racemosa, Reticulated Ulcerations, Panniculitis and Violaceous Plaques in a 46-year-old Woman.
    Indian Dermatol Online J 2018 Jan-Feb;9(1):47-49
    Department of Dermatology, Complejo Hospitalario de Navarra, Pamplona, Spain.
    Clinically amyopathic dermatomyositis (CADM) is a subset of dermatomyositis (DM) that has conventional cutaneous manifestations of DM, but paradoxically, little or no muscle involvement. In 2005, a novel antibody was described in association with CADM - anti-melanoma differentiation-associated gene 5 (anti-MDA5). Patients with this serologic marker have a characteristic mucocutaneous phenotype consisting of skin ulceration among other signs. Read More

    Dermatology and Immunoglobulin Therapy: Who to Treat and How to Administer Immunoglobulins.
    Actas Dermosifiliogr 2018 Feb 8. Epub 2018 Feb 8.
    Unidad de Gestión Clínica de Dermatología y Venereología, Complejo Hospitalario Universitario de Granada, Granada, España.
    Intravenous immunoglobulin (IVIG) replacement therapy has been used in immune deficiency diseases for more than 50 years. The indications for this treatment have evolved, however, and IVIG therapy is now used in various diseases in which the immune system plays a prominent role. IVIG therapy has carved out a niche in dermatology for the treatment of such conditions as dermatomyositis, autoimmune bullous diseases, and toxic epidermal necrolysis. Read More

    Increased Levels of Soluble Programmed Death Ligand 1 Associate with Malignancy in Patients with Dermatomyositis.
    J Rheumatol 2018 Feb 1. Epub 2018 Feb 1.
    From the Department of Rheumatology, China-Japan Friendship Hospital; Graduate School of Peking Union Medical College; Beijing Key Lab for Immune-Mediated Inflammatory Diseases, Beijing, China. Supported by the National Natural Science Foundation of China (81701615, 91542121, 81571603) and the Capital Foundation of Medical Developments (No. 2016-2-4063). H. Chen, MD Candidate, Department of Rheumatology, China-Japan Friendship Hospital, and Graduate School of Peking Union Medical College, and Beijing Key Lab for Immune-Mediated Inflammatory Diseases; Q. Peng, PhD, Department of Rheumatology, China-Japan Friendship Hospital; H. Yang, MD Candidate, Department of Rheumatology, China-Japan Friendship Hospital; L. Yin, MD, Department of Rheumatology, China-Japan Friendship Hospital; J. Shi, MD Candidate, Department of Rheumatology, China-Japan Friendship Hospital; Y. Zhang, MD Candidate, Department of Rheumatology, China-Japan Friendship Hospital; G. Wang, MD, PhD, Department of Rheumatology, China-Japan Friendship Hospital, and Graduate School of Peking Union Medical College, and Beijing Key Lab for Immune-Mediated Inflammatory Diseases. Address correspondence to Dr. G. Wang, MD, Department of Rheumatology, China-Japan Friendship Hospital, Beijing 100029, China. E-mail: Accepted for publication November 7, 2017.
    Objective: To investigate the levels of soluble programmed death ligand 1 (sPD-L1) and evaluate its association with malignancy in patients with dermatomyositis (DM).

    Methods: Levels of sPD-L1 were measured in serum from 88 DM patients without malignancies (sDM), 40 with cancer-related DM (CRDM), and 30 healthy controls (HC) using ELISA. The CRDM subjects were divided into new-onset cancers (nCRDM) and stable cancers (sCRDM). Read More

    Muscle Strength and Muscle Endurance During the First Year of Treatment of Polymyositis and Dermatomyositis: A Prospective Study.
    J Rheumatol 2018 Feb 1. Epub 2018 Feb 1.
    From the Department of Care Science and Society, Division of Physiotherapy, Karolinska Institutet, Huddinge; Functional Area Occupational Therapy and Physiotherapy, Allied Health Professionals Function, and the Rheumatology Clinic, Karolinska University Hospital, Stockholm; Department of Learning, Informatics, Management and Ethics, Karolinska Institutet, Stockholm; Rheumatology Unit, Department of Medicine, Solna, Karolinska Institutet, Karolinska University Hospital, Stockholm, Sweden. Supported by the Centre for Care Science at Karolinska Institutet, Swedish Rheumatism Association, the Swedish Research Council, Funds of the Karolinska Institutet, Börje Dahlin Foundation, King Gustaf V 80-year Foundation, the "Regional Agreement on medical training and clinical research (ALF) between Stockholm City Council and Karolinska Institutet," and the European Science Foundation. H. Alexanderson, PhD, RPT, Department of Care Science and Society, Division of Physiotherapy, Karolinska Institutet, and Functional Area Occupational Therapy and Physiotherapy, Allied Health Professionals Function, Karolinska University Hospital; M. Regardt, PhD, ROT, Functional Area Occupational Therapy and Physiotherapy, Allied Health Professionals Function, Karolinska University Hospital, Department of Learning, Informatics, Management and Ethics, Karolinska Institutet; C. Ottosson, RN, Rheumatology Clinic, Karolinska University Hospital; L. Alemo Munters, PhD, RPT, Functional Area Occupational Therapy and Physiotherapy, Allied Health Professionals Function, Karolinska University Hospital; M. Dastmalchi, PhD, MD, Rheumatology Clinic, Karolinska University Hospital, and Rheumatology Unit, Department of Medicine, Solna, Karolinska Institutet, Karolinska University Hospital; L. Dani, MD, Rheumatology Clinic, Karolinska University Hospital, and Rheumatology Unit, Department of Medicine, Solna, Karolinska Institutet, Karolinska University Hospital; I.E. Lundberg, PhD, MD, Rheumatology Clinic, Karolinska University Hospital, and Rheumatology Unit, Department of Medicine, Solna, Karolinska Institutet, Karolinska University Hospital. Address correspondence to Dr. H. Alexanderson, Karolinska University Hospital, Solna, D2:01, SE-171 76 Stockholm, Sweden. E-mail: Accepted for publication November 3, 2017.
    Objective: To investigate muscle impairment (isometric and dynamic) and disease activity during the first year after diagnosis of polymyositis (PM) and dermatomyositis (DM), and to study the relationship between muscle impairment, patient-reported health, and disease activity.

    Methods: Seventy-two patients enrolled in the Swedish Myositis Register, 2003-2010, were followed prospectively. The Manual Muscle test (MMT-8; isometric muscle strength), the Functional Index of myositis test (FI-2; dynamic, repetitive muscle function), and disease activity (6-item core set) were retrieved at the time of diagnosis, and after 6 and 12 months. Read More

    Rapidly progressive interstitial lung disease due to anti-MDA5 antibodies without skin involvement: a case report and literature review.
    Rheumatol Int 2018 Feb 7. Epub 2018 Feb 7.
    Pathology Department, Hospital Son Llàtzer, Carretera de Manacor Km4, 07198, Palma, Baleares, Spain.
    Anti-MDA5 antibodies have been strongly associated with rapidly progressive interstitial lung disease (RP-ILD) in dermatomyositis (DM) patients, especially in the clinically amyopathic subset (CADM). We present a case of anti-MDA5 antibody-associated RP-ILD in a patient with arthritis but with no other clinical signs suggestive of DM or CADM successfully treated with a combination of cyclophosphamide, cyclosporine and corticoids. A review of the literature was also done. Read More

    Increased risk of venous thromboembolism associated with polymyositis and dermatomyositis: a meta-analysis.
    Ther Clin Risk Manag 2018 23;14:157-165. Epub 2018 Jan 23.
    School of Nursing, Weifang Medical University, Weifang, People's Republic of China.
    Objective: Polymyositis and dermatomyositis (PM/DM) have been implicated in the development of venous thromboembolism (VTE). Previous studies investigating the association between PM/DM and VTE risk had yielded inconsistent findings. The aim of this study was to precisely estimate this association by meta-analysis of all available publications. Read More

    Ground-glass opacity heralding invasive lung adenocarcinoma with prodromal dermatomyositis: a case report.
    J Cardiothorac Surg 2018 Feb 7;13(1):20. Epub 2018 Feb 7.
    Division of Thoracic Surgery, Stanford University School of Medicine, Falk Building, 300 Pasteur Drive, Stanford, 94305, USA.
    Background: Dermatomyositis, an inflammatory myopathy with cutaneous involvement, is associated with malignancy and often manifests paraneoplastically. While co-occurrence with small cell carcinoma is well attested, primary lung adenocarcinoma, which may present as focal ground-glass opacification on computed tomography of the thorax, is less frequently coincident.

    Case Presentation: We report the case of a 72-year-old female patient with dermatomyositis - treated with a combination of prednisone, methotrexate, and intravenous immunoglobulin - and an indolent, subsolid, non-hypermetabolic pulmonary lesion, which was determined to be invasive primary lung adenocarcinoma. Read More

    ADAM-17 is expressed in the inflammatory myopathy and is involved with interstitial lung disease.
    Clin Rheumatol 2018 Feb 6. Epub 2018 Feb 6.
    Division of Rheumatology, Department of Medicine, Showa University School of Medicine, 1-5-8 Hatanodai, Shinagawa-ku, Tokyo, 142-8666, Japan.
    The "A disintegrin and metalloprotease" (ADAM) family is thought to play an important role in tissue destruction and inflammatory reactions. ADAM-17 was first described as the protease responsible for tumor necrosis factor (TNF)-α shedding. Here, we have shown the expression of ADAM-17 in inflammatory myopathy and demonstrated the role of inflammation in interstitial lung diseases (ILD). Read More

    [Myositis-specific antibodies associated with juvenile dermatomyositis].
    Z Rheumatol 2018 Feb 6. Epub 2018 Feb 6.
    Asklepios Klinik Sankt Augustin, Arnold-Janssen-Str. 29, 53757, Sankt Augustin, Deutschland.
    Background: Juvenile dermatomyositis (JDM) is a rare autoimmune disease associated with typical skin changes and muscle weakness. Within the framework of the diagnostics, myositis-associated (MAA) and myositis-specific antibodies (MSA) can be detected. These are important for the assessment of the course of the disease and the prognosis. Read More

    Horizontal and vertical sections of scalp biopsy specimens from dermatomyositis patients with scalp involvement.
    J Am Acad Dermatol 2018 Jan 30. Epub 2018 Jan 30.
    Department of Dermatology and Cutaneous Surgery, University of Miami L Miller School of Medicine, Miami, FL, USA. Electronic address:
    Background: The histologic findings of scalp involvement in dermatomyositis are not well characterized due to lack of large series.

    Objective: To systematize the histologic features of scalp involvement in dermatomyositis on horizontal and vertical sections.

    Materials And Methods: A descriptive, prospective, cross-sectional study recruited 31 patients with pathologically and serologically confirmed dermatomyositis in Mexico City from June 2014 to June 2015. Read More

    Coexistence of anti-Jo1 and anti-signal recognition particle antibodies in a polymyositis patient.
    Reumatol Clin 2018 Jan 25. Epub 2018 Jan 25.
    Departamento de Bioquímica, Hospital Universitario Virgen Macarena, Sevilla, España.
    Idiopathic inflammatory myopathies are a heterogeneous group of potentially treatable myopathies. They are classified, on the basis of clinical and histopathological features, into four subtypes: dermatomyositis, polymyositis, necrotizing autoimmune myositis and inclusion-body myositis. Myositis-associated antibodies and myositis-specific autoantibodies are frequently found in patients with idiopathic inflammatory myopathies, and are useful in the diagnosis and classification. Read More

    [A Case of Breast Cancer Associated with Dermatitis That Was Difficult to Differentiate from Dermatomyositis].
    Gan To Kagaku Ryoho 2017 Nov;44(12):1930-1932
    Dept. of Surgical Oncology, Osaka City University Graduate School of Medicine.
    A 46-year-old woman visited our hospital with a chief complaint of a mass in the right breast. Breast ultrasonography revealed a hypoechoic area with an indistinct border on the right breast, and right axillary lymph node swelling. A core needle biopsy revealed invasive ductal carcinoma, and the diagnosis was right breast cancer, cT2N2M0, Stage III A, HER2-enriched type. Read More

    Analysis of anti-melanoma differentiation-associated gene 5 antibody in Hong Kong Chinese patients with idiopathic inflammatory myopathies: diagnostic utility and clinical correlations.
    Int J Rheum Dis 2018 Jan 30. Epub 2018 Jan 30.
    Department of Medicine and Geriatrics, Kwong Wah Hospital, Kowloon, Hong Kong.
    Aim: To compare the prevalence of the anti-melanoma differentiation-associated gene 5 antibody (anti-MDA5 Ab) in Hong Kong Chinese patients with dermatomyositis (DM) and polymyositis (PM); in addition, to examine the association of anti-MDA5 Ab and the clinical characteristics of these patients.

    Methods: Twenty consecutive existing patients with DM being followed up at the Rheumatology Clinic of Kwong Wah Hospital, Hong Kong were recruited. Twenty patients with PM were recruited from the same clinic as the controls. Read More

    Cardiovascular and cerebrovascular comorbidities of juvenile dermatomyositis in US children: an analysis of the National Inpatient Sample.
    Rheumatology (Oxford) 2018 Jan 24. Epub 2018 Jan 24.
    Division of Rheumatology, Departments of Pediatrics and Medical Social Sciences, Northwestern University Feinberg School of Medicine, Chicago, IL, USA.
    Objective: JDM is associated with multiple potential risk factors for cardiovascular disease, including reduced heart rate variability, systolic/diastolic cardiac dysfunction, abnormal brachial artery reactivity and metabolic syndrome. However, little is known about cardiovascular risk in JDM. We sought to examine the association between JDM and cardiovascular risk factors and disease in US children. Read More

    Dermatomyositis: clinicopathological correlations.
    G Ital Dermatol Venereol 2018 Jan 24. Epub 2018 Jan 24.
    Dermatology Unit, Papa Giovanni XXIII Hospital, Bergamo, Italy.
    Dermatomyositis (DM) is a chronic autoimmune disease affecting primarily the skeletal muscle and the skin. Although the diagnosis is usually based upon clinical and laboratory findings, histopathology is helpful for confirmation purposes and is mandatory in amiopathic DM where there is only skin involvement. Histologically, the typical cases are characterized by subtle vacuolar interface alterations with epidermal atrophy, mild capillary ectasia and sparse perivascular lymphocytic infiltrate; these findings are similar to those seen in other connective tissue diseases, especially lupus erythematosus. Read More

    Extensive skin ulcers in a child with juvenile dermatomyositis.
    BMJ Case Rep 2018 Jan 23;2018. Epub 2018 Jan 23.
    Department of Pediatrics, Postgraduate Institute of Medical Education and Research, Chandigarh, India.
    Juvenile dermatomyositis (JDM) is a multisystemic disorder. Vasculitic ulcers in JDM have been reported to involve axilla, elbow or extensor surfaces of other joints. We report a young boy with JDM who presented with extensive cutaneous ulcers involving scrotum, prepuce, gluteal region, neck, bilateral axilla, periumbilical area and bilateral elbows and popliteal fossa. Read More

    Role of the chemokine receptors CXCR3, CXCR4 and CCR7 in the intramuscular recruitment of plasmacytoid dendritic cells in dermatomyositis.
    J Neuroimmunol 2018 Jan 9. Epub 2018 Jan 9.
    Department of Neurology and Neuromuscular Center, Qilu Hospital of Shandong University, Jinan, PR China. Electronic address:
    To explore the possible mechanism implicated in the recruitment of plasmacytoid dendritic cells (pDCs), we investigated the expression of the chemokine receptors CXCR3, CXCR4, and CCR7 on intramuscular and circulating pDCs from patients with dermatomyositis (DM). Using immunohistochemistry, preferential expression of CXCR3, CXCR4 and CCR7 was identified in the perivascular inflammatory infiltrates within the perimysium in DM muscle. Western-blot analysis showed marked up-regulation of expression of CXCR3, CXCR4 and CCR7 in muscle homogenate from patients with DM compared with that in non-diseased controls. Read More

    Anti-NT5C1A autoantibodies are associated with more severe disease in patients with juvenile myositis.
    Ann Rheum Dis 2018 Jan 23. Epub 2018 Jan 23.
    Muscle Disease Unit, Laboratory of Muscle Stem Cells and Gene Regulation, National Institute of Arthritis and Musculoskeletal and Skin Diseases, National Institutes of Health (NIH), Bethesda, Maryland, USA.
    Objectives: Autoantibodies recognising cytosolic 5'-nucleotidase 1A (NT5C1A) are found in adult patients with myositis and other autoimmune diseases. They are especially prevalent in adults with inclusion body myositis (IBM), in which they are associated with more severe weakness and higher mortality. This study was undertaken to define the prevalence and clinical features associated with anti-NT5C1A autoantibodies in juvenile myositis. Read More

    Multiple target autoantigens on endothelial cells identified in juvenile dermatomyositis using proteomics.
    Rheumatology (Oxford) 2018 Jan 18. Epub 2018 Jan 18.
    Department of Pediatrics and Genetics, Genomics, & Bioinformatics Program, Jacobs School of Medicine and Biomedical Sciences University at Buffalo, Buffalo, NY, USA.
    Objective: Although generally classified within the group of inflammatory myopathies, JDM displays many pathological features of vasculitis. Previous work has shown that AECA are abundant in other forms of vasculitis. We therefore investigated whether such antibodies might also be detected in JDM. Read More

    Immune myopathies with perimysial pathology: Clinical and laboratory features.
    Neurol Neuroimmunol Neuroinflamm 2018 Mar 17;5(2):e434. Epub 2018 Jan 17.
    Department of Neurology, Washington University School of Medicine, St. Louis, MO.
    Objective: Immune myopathies with perimysial pathology (IMPP) have a combination of damage to perimysial connective tissue and muscle fiber necrosis, more prominent near the perimysium. We studied the clinical and laboratory correlates of patients with pathologically defined IMPP.

    Methods: This is a retrospective chart and pathology review of 57 consecutive patients with IMPP myopathology and, for comparison, 20 patients with dermatomyositis with vascular pathology (DM-VP). Read More

    Clinical Characteristics and Associated Systemic Diseases in Patients With Esophageal "Absent Contractility"-A Clinical Algorithm.
    J Clin Gastroenterol 2018 Jan 19. Epub 2018 Jan 19.
    Digestive Disease Institute, Cleveland Clinic.
    Goals: This study was carried out to assess the clinical characteristics and associated systemic diseases seen in patients diagnosed with absent contractility as per the Chicago Classification version 3.0, allowing us to propose a diagnostic algorithm for their etiologic testing.

    Background: The Chicago Classification version 3. Read More

    Efficacy and safety of cyclophosphamide treatment in severe juvenile dermatomyositis shown by marginal structural modelling.
    Arthritis Rheumatol 2018 Jan 17. Epub 2018 Jan 17.
    Rheumatology Unit, Great Ormond Street Hospital for Children, London, UK.
    Objective: In patients with severe or refractory juvenile dermatomyositis (JDM), second-line treatments may be required. Cyclophosphamide (CYC) is used to treat some connective tissue diseases, but evidence of efficacy in JDM is limited. This study aimed to describe clinical improvement in JDM patients treated with CYC and model efficacy of CYC compared to patients not treated with CYC. Read More

    Correlation between disease activity and serum ferritin in clinically amyopathic dermatomyositis with rapidly-progressive interstitial lung disease: a case report.
    BMC Res Notes 2018 Jan 16;11(1):34. Epub 2018 Jan 16.
    Department of Respiratory Medicine, Graduate School of Medicine, Osaka City University, 1-4-3, Asahi-machi, Abeno-ku, Osaka, 545-8585, Japan.
    Background: Clinically amyopathic dermatomyositis with anti-Melanoma Differentiation-Associated gene 5 (MDA5) antibody often presents with severe interstitial lung disease. Although serum ferritin level is known to reflect interstitial lung disease activity, there are few case reports describing this association.

    Case Presentation: A 58-year-old man was referred to our outpatient clinic with a 3-week history of cough and respiratory distress. Read More

    The Childhood Arthritis & Rheumatology Research Alliance Consensus Treatment Plans: Towards Comparative Effectiveness in the Pediatric Rheumatic Diseases.
    Arthritis Rheumatol 2018 Jan 15. Epub 2018 Jan 15.
    Pediatric Rheumatology, Hackensack University Medical Center, Hackensack, United States.
    The pediatric rheumatic diseases are a heterogeneous group of rare diseases, posing a number of challenges for the use of traditional clinical and translational research approaches. Innovative comparative effectiveness approaches are needed to efficiently study treatment approaches and disease outcomes. The Childhood Arthritis and Rheumatology Research Alliance (CARRA) developed the consensus treatment plan (CTP) approach as a comparative effectiveness tool for research in pediatric rheumatology. Read More

    Splicing variant ofaugments MDA5 signalling and the risk of clinically amyopathic dermatomyositis.
    Ann Rheum Dis 2018 Jan 13. Epub 2018 Jan 13.
    Laboratory for Autoimmune Diseases, RIKEN Center for Integrative Medical Sciences, Yokohama, Japan.
    Objectives: Idiopathic inflammatory myopathies (IIMs) are a heterogeneous group of rare autoimmune diseases in which both genetic and environmental factors play important roles. To identify genetic factors of IIM including polymyositis, dermatomyositis (DM) and clinically amyopathic DM (CADM), we performed the first genome-wide association study for IIM in an Asian population.

    Methods: We genotyped and tested 496 819 single nucleotide polymorphism for association using 576 patients with IIM and 6270 control subjects. Read More

    Dermatomyositis: Histopathologic findings of parakeratosis and dermal edema revisited.
    J Cutan Pathol 2018 Jan 13. Epub 2018 Jan 13.
    Department of Dermatology, Yale University Medical School, New Haven, Connecticut.
    The cutaneous manifestations of dermatomyositis range from classical in the case of heliotrope rash and Gottron papules to less common papulosquamous and edematous/vesiculobullous lesions; histopathologic descriptions are dominated by interface dermatitis. We present a case of dermatomyositis with a combination of common and rare skin findings, both clinically and histologically. Increased awareness of papulosquamous and edematous lesions of dermatomyositis can help direct patient care. Read More

    Early detection of myocardial involvement by Tmapping of cardiac MRI in idiopathic inflammatory myopathy.
    J Magn Reson Imaging 2018 Jan 12. Epub 2018 Jan 12.
    Cardiology Department, West China Hospital, Sichuan University, Chengdu, Sichuan Province, China.
    Background: Polymyositis (PM) and dermatomyositis (DM) are common types of idiopathic inflammatory myopathy (IIM), wherein patients are prone to adverse cardiovascular events.

    Purpose: To explore the value of cardiac magnetic resonance imaging (MRI) for detecting cardiac involvement in PM/DM patients using a Tmapping technique.

    Study Type: Prospective observational study. Read More

    The predictive prognostic factors for polymyositis/dermatomyositis-associated interstitial lung disease.
    Arthritis Res Ther 2018 Jan 11;20(1). Epub 2018 Jan 11.
    Department of Stem Cell and Immune Regulation, Yokohama City University Graduate School of Medicine, 3-9 Fukuura, Kanazawa-ku, Yokohama, 236-0004, Japan.
    Background: Interstitial lung disease (ILD) is the principal cause of death in polymyositis/dermatomyositis (PM/DM). Here we investigated prognostic factors for death and serious infection in PM/DM-ILD using the multicenter database.

    Methods: We retrospectively reviewed baseline demographic, clinical and laboratory findings, treatment regimens and outcomes in patients with PM/DM-ILD. Read More

    [Paraneoplasms of the skin].
    Internist (Berl) 2018 Feb;59(2):134-144
    Klinik und Poliklinik für Dermatologie und Allergologie, Klinikum der Universität München, Ludwig-Maximilians-Universität München, München, Deutschland.
    The cutaneous manifestations of malignancies include nonmalignant skin disorders that occur in association with malignancies (facultative paraneoplastic dermatoses) and skin disorders that are always associated with hematologic diseases or solid tumors (obligate paraneoplastic dermatoses). Paraneoplastic increase of growth factors or immunological reactions lead to a variety of inflammatory, hyperkeratotic or proliferative skin reactions. When paraneoplastic dermatoses develop before cancer is diagnosed, recognition of these skin diseases can accelerate both the diagnosis and treatment. Read More

    Circulating plasma microRNA profiling in patients with polymyositis/dermatomyositis before and after treatment: miRNA may be associated with polymyositis/dermatomyositis.
    Inflamm Regen 2018 8;38. Epub 2018 Jan 8.
    Institute for Environmental and Gender-Specific Medicine, Juntendo University Graduate School of Medicine, Chiba, Japan.
    Background: MicroRNAs (miRNAs) are involved in the regulation of key biological processes and have been implicated in various diseases, including autoimmune disorders. The pathogenesis of polymyositis (PM) and dermatomyositis (DM) is considered to be mediated by autoimmune reactions. To determine miRNA role in the development and progression of PM and DM, we performed plasma miRNA profiling in PM/DM patients before and after treatment. Read More

    Prevalence and predictors of asymptomatic vertebral fractures in inflammatory myositis.
    Int J Rheum Dis 2018 Jan 5. Epub 2018 Jan 5.
    Department of Clinical Immunology and Rheumatology, Sanjay Gandhi Postgraduate Institute of Medical Sciences, Lucknow, India.
    Aim: To assess the frequency and risk factors of asymptomatic vertebral fractures in inflammatory myositis.

    Patients And Methods: Dorsal and lumbar spine lateral radiographs were taken for adults with inflammatory myositis and scored using Genant's semi-quantitative technique. Demographic data, weight, height, postmenopausal status, duration of corticosteroid use, drug intake, co-morbidities and past history of fractures were recorded. Read More

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