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Dermatomyositis

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https://www.readbyqxmd.com/read/28934971/assessment-classification-and-treatment-of-calcinosis-as-a-complication-of-juvenile-dermatomyositis-a-survey-of-pediatric-rheumatologists-by-the-childhood-arthritis-and-rheumatology-research-alliance-carra
#1
A B Orandi, K W Baszis, V R Dharnidharka, A M Huber, M F Hoeltzel
BACKGROUND: There is no standardized approach to the management of JDM-associated calcinosis and its phenotypes. Current knowledge of treatment outcomes is confined to small series and case reports. We describe physician perspectives toward diagnostic approach, classification and treatment directly targeting calcinosis, independent of overall JDM therapy. METHODS: An electronic survey of 22 questions was organized into sections regarding individual practices of assessment, classification and treatment of calcinosis, including perceived successes of therapies...
September 21, 2017: Pediatric Rheumatology Online Journal
https://www.readbyqxmd.com/read/28912913/paraneoplastic-dermatomyositis-syndrome-presenting-as-dysphagia
#2
Emmanuel Ofori, Daryl Ramai, Mel Ona, Madhavi Reddy
Dermatomyositis (DM) is a rare autoimmune condition which predominantly affects females in the fifth and sixth decades of life. DM presents acutely or progressively as painless proximal skeletal muscle weakness and can be associated with a heliotropic rash around the eyes, and Gottron's papules on extensor surfaces of the hands. While the pathophysiology of DM is still unclear, abnormal T- and B-cell immune activity has been reported. DM has been associated with malignancy and has been shown to appear before, concurrently with, or after diagnosis of cancer...
August 2017: Gastroenterology Research
https://www.readbyqxmd.com/read/28911766/juvenile-dermatomyositis-key-roles-of-muscle-magnetic-resonance-imaging-and-early-aggressive-treatment
#3
O Corral-Magaña, A F Bauzá-Alonso, M M Escudero-Góngora, L Lacruz, A Martín-Santiago
Juvenile dermatomyositis is a rare systemic connective tissue disease with onset during childhood. It presents clinically with proximal muscle weakness and characteristic skin involvement. Diagnosis is based on the Bohan and Peter criteria, though many authors are now substituting biopsy with muscle magnetic resonance imaging (MRI) for both diagnosis and follow-up. Without intensive early treatment, complications such as calcinosis cutis and lipodystrophy can develop in the chronic phases of the disease. Early recognition is therefore key to management...
September 11, 2017: Actas Dermo-sifiliográficas
https://www.readbyqxmd.com/read/28895299/a-case-of-lipoatrophic-diabetes-induced-by-juvenile-dermatomyositis
#4
Yusuke Baba, Hiyori Kaneko, Minoru Takemoto, Kana Ide, Yuki Haga, Tatsuo Kanda, Shinsuke Akita, Yoshitaka Kubota, Masayuki Kuroda, Yutaka Kitagawa, Koutaro Yokote
Lipodystrophy is a rare condition that is often accompanied by one or more metabolic diseases. Here we report a case of lipoatrophic diabetes induced by juvenile dermatomyositis. Although pioglitazone was not effective for lowering blood glucose levels, our observation suggested that it improved liver function slightly. The effectiveness of metreleptin for lowering blood glucose levels could not be determined, as we administered it in short period. Liver biopsy revealed burned-out nonalcoholic steatohepatitis...
September 12, 2017: Journal of Diabetes Investigation
https://www.readbyqxmd.com/read/28895209/focal-hyperkeratosis-overlying-the-palmar-faces-of-interphalangeal-and-metacarpophalangeal-joints-revealing-a-juvenile-dermatomyositis
#5
C Abasq-Thomas, V Devauchelle, S Fraitag, N Jay, G Gourier, A Le Duc Pennec, E Brenaut, L Misery
Dermatomyositis (DM) is an idiopathic inflammatory myopathy characterized by an inflammatory infiltrate primarily affecting the skeletal muscle and skin [1]. It is the commonest of the idiopathic inflammatory myopathies of childhood, comprising 85% of cases. It has an annual incidence estimated to range between 1.9 and 4.1 per million children [2, 3]. This article is protected by copyright. All rights reserved.
September 12, 2017: Journal of the European Academy of Dermatology and Venereology: JEADV
https://www.readbyqxmd.com/read/28893408/inflammatory-myopathy-associated-with-antimitochondrial-antibodies-a-distinct-phenotype-with-cardiac-involvement
#6
Jemima Albayda, Aamna Khan, Livia Casciola-Rosen, Andrea M Corse, Julie J Paik, Lisa Christopher-Stine
OBJECTIVE: In the context of clinical evaluations performed on our prospective myositis cohort, we noted a striking association of severe cardiac disease in myositis patients with antimitochondrial antibodies. We sought to review all cases of antimitochondrial antibody (AMA) associated myositis in our cohort to describe the clinical features of this disease subset. METHODS: We identified 7 patients with confirmed antimitochondrial antibodies who presented as an inflammatory myopathy...
June 13, 2017: Seminars in Arthritis and Rheumatism
https://www.readbyqxmd.com/read/28879187/clinical-and-dermoscopic-features-of-the-scalp-in-31-patients-with-dermatomyositis
#7
Julio C Jasso-Olivares, Antonella Tosti, Mariya Miteva, Judith Domínguez-Cherit, José M Díaz-González
BACKGROUND: Scalp involvement is not directly evaluated in patients with dermatomyositis (DM). Therefore, the exact frequency of scalp dermatomyositis (SDM) and its clinical and trichoscopic characteristics have been poorly described. OBJECTIVE: The aim of this study was to determine the frequency and clinical and dermoscopic features of SDM in patients diagnosed with DM. METHODS: We performed a descriptive prospective, cross-sectional observational study that included all patients diagnosed with DM at a Mexican academic institute over the course of a year...
August 2017: Skin Appendage Disorders
https://www.readbyqxmd.com/read/28879041/pulmonary-arterial-hypertension-a-novelty-in-idiopathic-inflammatory-myopathies-insights-and-first-experiences-with-vasoactive-therapy
#8
Kavish J Bhansing, Anton Vonk-Noordegraaf, Frank Pt Oosterveer, Piet Lcm van Riel, Madelon C Vonk
To characterise the different types of pulmonary hypertension (PH) among idiopathic inflammatory myopathy (IIM). A retrospective case series with assessment of PH by right heart catheterisation, extent of interstitial lung disease (ILD) and outcome of vasoactive therapy.The group of patients with IIM with PH (n=9) showed a median age at PH diagnosis of 62 years (IQR 48-71 years; eight women), seven diagnosed with polymyositis and two with dermatomyositis; median disease duration of 5.7 years and five patients with a positive anti-Jo1 antibody...
2017: RMD Open
https://www.readbyqxmd.com/read/28872085/-manifestations-of-the-connective-tissue-associated-interstitial-lung-disease-under-high-resolution-computed-tomography
#9
Hailan Li, Zeng Xiong, Jinkang Liu, Yisha Li, Bin Zhou
To analyze the features of the connective tissue associated interstitial lung disease (CTD-ILD) by high resolution computed tomography (HRCT).
 Methods: A total of 127 patients with CTD-ILD, who were diagnosed by clinic laboratory examination and pathology in Xiangya Hospital of Central South University form September 2013 to September 2015, were enrolled for this study. Their lung features of HRCT imaging were retrospectively analyzed.
 Results: The classifications for 127 patients were as follows: 36 cases of rheumatoid arthritis (28...
August 28, 2017: Zhong Nan da Xue Xue Bao. Yi Xue Ban, Journal of Central South University. Medical Sciences
https://www.readbyqxmd.com/read/28871670/-early-detection-of-endothelial-dysfunction-in-children-with-autoimmune-diseases-by-a-novel-noninvasive-technique
#10
Liron Borenstein-Levin, Riva Brik, Giora Pillar, Yonatan Butbul Aviel
INTRODUCTION: Atherosclerosis is emerging as one of the most important causes of morbidity and mortality among patients with different rheumatologic disease. Endothelial dysfunction may be an early sign of atherosclerosis. OBJECTIVES: To evaluate the occurrence of endothelial dysfunction in children with autoimmune diseases, including juvenile idiopathic arthritis (JIA), systemic lupus erythematosus (SLE) and dermatomyositis, using a novel noninvasive technique...
July 2017: Harefuah
https://www.readbyqxmd.com/read/28871260/dermatomyositis-and-immune-mediated-necrotizing-myopathies-a-window-on-autoimmunity-and-cancer
#11
REVIEW
Audrey Aussy, Olivier Boyer, Nadège Cordel
Autoimmune myopathies (myositides) are strongly associated with malignancy. The link between myositis and cancer, originally noticed by Bohan and Peter in their classification in 1975 (1), has been evidenced by large population-based cohort studies and a recent meta-analysis. The numerous reports of cases in which the clinical course of myositis reflects that of cancer and the short delay between myositis and cancer onset support the notion that myositis may be an authentic paraneoplastic disorder. Thus, cancer-associated myositis raises the question of cancer as a cause rather than a consequence of autoimmunity...
2017: Frontiers in Immunology
https://www.readbyqxmd.com/read/28870032/-neutrophil-extracellular-traps-induced-endothelial-cell-damage-in-the-pathogenesis-of-dermatomyositis-associated-interstitial-lung-disease
#12
Y Zhang, B Yan, Y Liu
Objective: To explore the role of neutrophil extracellular traps (NETs)-induced endothelial cell damage in the pathogenesis of dermatomyositis (DM)-associated interstitial lung disease(ILD). Methods: Serum free DNA and krebs von den lungen-6 (KL-6) level were tested in healthy controls, dermatomyositis patients with or without interstitial lung disease (DM-ILD and DM-NILD). Subjects' peripheral blood neutrophils were stimulated with phorbol 12-myristate 13-acetate (PMA), then human umbilical vein endothelial cells (HUVECs) were co-cultured with NETs...
September 1, 2017: Zhonghua Nei Ke za Zhi [Chinese Journal of Internal Medicine]
https://www.readbyqxmd.com/read/28869080/altered-chemokine-receptor-expression-in-the-peripheral-blood-lymphocytes-in-polymyositis-and-dermatomyositis
#13
Ziwei Zhu, Chunshu Yang, Jianing Wang, Qianhui Feng, Qi Chen, Pingting Yang
OBJECTIVE: To examine the expression of chemokine receptors in different peripheral blood T-cell subsets in patients with polymyositis (PM) and dermatomyositis (DM). METHODS: We used flow cytometry to measure the frequencies of chemokinereceptors CXCR3 and CCR4 expression in the CD4(+) or CD8(+) lymphocytes. Enzyme linked immunosorbent assays were also used to measure the concentrations of C-X-C motif chemokine 10 (CXCL10), thymus and activation regulated chemokine (TARC) and macrophage derived chemokine (MDC)...
August 28, 2017: Cytokine
https://www.readbyqxmd.com/read/28866745/mortality-and-prognostic-factors-in-idiopathic-inflammatory-myositis-a-retrospective-analysis-of-a-large-multicenter-cohort-of-spain
#14
Laura Nuño-Nuño, Beatriz Esther Joven, Patricia E Carreira, Valentina Maldonado-Romero, Carmen Larena-Grijalba, Irene Llorente Cubas, Eva Gloria Tomero, María Carmen Barbadillo-Mateos, Paloma García De la Peña Lefebvre, Lucía Ruiz-Gutiérrez, Juan Carlos López-Robledillo, Henry Moruno-Cruz, Ana Pérez, Tatiana Cobo-Ibáñez, Raquel Almodóvar González, Leticia Lojo, María Jesús García De Yébenes, Francisco Javier López-Longo
The present study was undertaken to assess mortality, causes of death, and associated prognostic factors in a large cohort of patients diagnosed with idiopathic inflammatory myositis (IIM) from Spain. A retrospective longitudinal study was carried out in 467 consecutive patients with IIM, identified from 12 medical centers. Patients were classified as primary polymyositis, primary dermatomyositis (DM), overlap myositis, cancer-associated myositis (CAM), and juvenile idiopathic inflammatory myopathies. A total of 113 deaths occurred (24%) after a median follow-up time of 9...
September 2, 2017: Rheumatology International
https://www.readbyqxmd.com/read/28864939/intravenous-immunoglobulins-for-treatment-of-connective-tissue-diseases-in-dermatology
#15
REVIEW
Lyubomir A Dourmishev, Dimitrina V Guleva, Ljubka G Miteva
BACKGROUND: Connective tissue diseases are a heterogeneous group of autoimmune disorders affecting not only skin, but also various organs and systems. First-line treatment of connective tissue diseases is systemic steroids as monotherapy or combined with immunosuppressive drugs. Since intravenous immunoglobulins (IVIGs) have been found to be effective for various autoimmune dermatoses, their indications have expanded tremendously. OBJECTIVE: The aim this review article is to highlight the indications, effectiveness, and side effects of high doses immunoglobulins for treatment of patients with connective tissue diseases...
September 1, 2017: Wiener Medizinische Wochenschrift
https://www.readbyqxmd.com/read/28864644/myositis-associated-interstitial-lung-disease-predictors-of-failure-of-conventional-treatment-and-response-to-tacrolimus-in-a-us-cohort
#16
Niharika Sharma, Michael S Putman, Rekha Vij, Mary E Strek, Anisha Dua
OBJECTIVE: Patients with myositis-associated interstitial lung disease (MA-ILD) are often refractory to conventional treatment, and predicting their response to therapy is challenging. Recent case reports and small series suggest that tacrolimus may be useful in refractory cases. METHODS: A retrospective cohort study of patients with MA-ILD comparing clinical characteristics between those who responded to or failed conventional treatment. In those who failed conventional treatment and received adjunctive tacrolimus, response to tacrolimus was measured by the improvement in myositis, ILD, and change in the dose of glucocorticoids...
September 1, 2017: Journal of Rheumatology
https://www.readbyqxmd.com/read/28864092/facts-and-controversies-in-mixed-connective-tissue-disease
#17
REVIEW
Julia Martínez-Barrio, Lara Valor, F Javier López-Longo
Mixed connective tissue disease (MCTD) is a systemic autoimmune rheumatic disease (SARD) characterised by the combination of clinical manifestations of systemic lupus erythematosus (SLE), cutaneous systemic sclerosis (SSc) and polymyositis-dermatomyositis, in the presence of elevated titers of anti-U1-RNP antibodies. Main symptoms of the disease are polyarthritis, hand oedema, Raynaud's phenomenon, sclerodactyly, myositis and oesophageal hypomobility. Although widely discussed, most authors today accept MCTD as an independent entity...
August 29, 2017: Medicina Clínica
https://www.readbyqxmd.com/read/28855174/the-euromyositis-registry-an-international-collaborative-tool-to-facilitate-myositis-research
#18
James B Lilleker, Jiri Vencovsky, Guochun Wang, Lucy R Wedderburn, Louise Pyndt Diederichsen, Jens Schmidt, Paula Oakley, Olivier Benveniste, Maria Giovanna Danieli, Katalin Danko, Nguyen Thi Phuong Thuy, Monica Vazquez-Del Mercado, Helena Andersson, Boel De Paepe, Jan L deBleecker, Britta Maurer, Liza J McCann, Nicolo Pipitone, Neil McHugh, Zoe E Betteridge, Paul New, Robert G Cooper, William E Ollier, Janine A Lamb, Niels Steen Krogh, Ingrid E Lundberg, Hector Chinoy
AIMS: The EuroMyositis Registry facilitates collaboration across the idiopathic inflammatory myopathy (IIM) research community. This inaugural report examines pooled Registry data. METHODS: Cross-sectional analysis of IIM cases from 11 countries was performed. Associations between clinical subtypes, extramuscular involvement, environmental exposures and medications were investigated. RESULTS: Of 3067 IIM cases, 69% were female. The most common IIM subtype was dermatomyositis (DM) (31%)...
August 30, 2017: Annals of the Rheumatic Diseases
https://www.readbyqxmd.com/read/28854220/automated-diagnosis-of-myositis-from-muscle-ultrasound-exploring-the-use-of-machine-learning-and-deep-learning-methods
#19
Philippe Burlina, Seth Billings, Neil Joshi, Jemima Albayda
OBJECTIVE: To evaluate the use of ultrasound coupled with machine learning (ML) and deep learning (DL) techniques for automated or semi-automated classification of myositis. METHODS: Eighty subjects comprised of 19 with inclusion body myositis (IBM), 14 with polymyositis (PM), 14 with dermatomyositis (DM), and 33 normal (N) subjects were included in this study, where 3214 muscle ultrasound images of 7 muscles (observed bilaterally) were acquired. We considered three problems of classification including (A) normal vs...
2017: PloS One
https://www.readbyqxmd.com/read/28846454/glis3-and-tyk2-single-nucleotide-polymorphisms-are-not-associated-with-dermatomyositis-polymyositis-in-chinese-han-population
#20
Liubing Li, Si Chen, Qian Wang, Chanyuan Wu, Xiaoting Wen, Funing Yang, Chenxi Liu, Fengchun Zhang, Yongzhe Li
AIM: Racial differences and genetic overlap have been shown to be responsible for the difference in susceptibility to dermatomyositis (DM)/polymyositis (PM) in a variety of populations. Single nucleotide polymorphisms (SNPs) in the GLI-similar 3 (GLIS3) and tyrosine kinase 2 (TYK2) genes have been associated with various autoimmune diseases. The aim of this study was to investigate whether SNPs in GLIS3 (rs7020673, rs10758593, and rs10814916) and TYK2 (rs280519, rs2304256, rs17000730, and rs280501) were associated with an increase in susceptibility to DM/PM in a Chinese Han population...
September 2017: Genetic Testing and Molecular Biomarkers
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