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Dermatomyositis

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https://www.readbyqxmd.com/read/28225510/effective-use-of-calcineurin-inhibitor-in-combination-therapy-for-interstitial-lung-disease-in-patients-with-dermatomyositis-and-polymyositis
#1
Yasuhiro Shimojima, Wataru Ishii, Masayuki Matsuda, Dai Kishida, Shu-Ichi Ikeda
OBJECTIVE: The current study demonstrated the potential use of calcineurin inhibitor (CNI) in combination therapy for interstitial lung disease (ILD) complicated with dermatomyositis (DM) and polymyositis (PM). METHODS: Thirty DM/PM patients with ILD were enrolled in this study. Continuous intravenous administration of cyclosporine A (IV-CsA) was simultaneously started with corticosteroid in patients presenting more than two respiratory distress factors as follows: <70 mmHg of PaO2, percentage of vital capacity <70%, and/or exertional dyspnea...
March 2017: Journal of Clinical Rheumatology: Practical Reports on Rheumatic & Musculoskeletal Diseases
https://www.readbyqxmd.com/read/28224635/infections-and-vaccinations-as-possible-triggers-of-inflammatory-myopathies
#2
Vidya Limaye, Caroline Smith, Barbara Koszyca, Peter Blumbergs, Sophia Otto
INTRODUCTION: The role of vaccinations and infections in triggering idiopathic inflammatory myopathies (IIM) has not been confirmed. METHODS: Among patients with histologically-confirmed myositis, infections or vaccinations administered prior to myositis onset were determined. The characteristics of this group were compared with controls (myositis patients without prior infection or vaccination). RESULTS: The frequency of IIM with a prior vaccination was 20 of 206, (9...
February 22, 2017: Muscle & Nerve
https://www.readbyqxmd.com/read/28224486/human-herpes-virus-6-encephalitis-causing-severe-anterograde-amnesia-associated-with-rituximab-azathioprine-and-prednisolone-combination-therapy-for-dermatomyositis
#3
Thomas Baumer, Charlie Fry, Sebastian Luppe, Harsha Gunawardena, Kasia Sieradzan
Human herpes virus-6 (HHV-6) reactivation is a well-recognised complication following haematological stem cell transplantation, but it is novel in the context of combination immunomodulatory therapy for autoimmune disease. We report a case of severe anterograde amnesia caused by HHV-6 encephalitis in a young female patient on rituximab, azathioprine and prednisolone for dermatomyositis (DM). The use of targeted biologic treatments for systemic autoimmune connective tissue diseases (CTDs) is increasing, particularly when refractory to conventional management...
February 21, 2017: Journal of Neurovirology
https://www.readbyqxmd.com/read/28210638/an-unusual-suspect-causing-hypoxemic-respiratory-failure
#4
Masooma Aqeel, Bjorn Batdorf, Horatiu Olteanu, Jayshil J Patel
Introduction: Antisynthetase syndrome (ASS) is characterized by the presence of anti-Jo-1 antibodies in conjunction with clinical findings of fever, polymyositis-dermatomyositis, and interstitial lung disease (ILD). Inflammatory myopathies carry a high risk of malignancy, but this association is less well outlined in ASS. We present the case of a patient with ASS who developed non-Hodgkin's lymphoma with acute hypoxemic respiratory failure. Case Presentation: A 44-year-old female with ASS presented with acute hypoxemic respiratory failure...
January 2017: Journal of Investigative Medicine High Impact Case Reports
https://www.readbyqxmd.com/read/28210382/paraneoplastic-dermatomyositis-with-cutaneous-and-myopathic-disease-responsive-to-adrenocorticotropic-hormone-therapy
#5
Marisa Wolff, Christopher Mancuso, Karan Lal, Damian Dicostanzo, Charles Gropper
Dermatomyositis is a myopathic or amyopathic autoimmune connective tissue disease that presents with classic dermatologic findings ranging from: poikilodermatous photosensitivity (shawl sign), eyelid edema and violaceous-pigmentation (heliotrope sign), lichenoid eruptions on the knuckles and elbows (Gottron's sign), periungual telangiectasias, and ragged cuticles (Samitz sign). Up to 30 percent of adult-onset cases of dermatomyositis may represent a paraneoplastic syndrome warranting a thorough work-up for malignancy...
January 2017: Journal of Clinical and Aesthetic Dermatology
https://www.readbyqxmd.com/read/28210273/isolated-ro52-antibodies-as-immunological-marker-of-a-mild-phenotype-of-undifferentiated-connective-tissue-diseases
#6
Ana Alonso-Larruga, Sagrario Bustabad, José Antonio Navarro-Gonzálvez, Beatriz Rodríguez-Lozano, Andrés Franco, Yvelise Barrios
The term undifferentiated connective tissue disease (UCTD) is used to describe undiagnosed patients that do not fulfill classification criteria for definite connective tissue disease (Systemic Lupus, Systemic Sclerosis, Sjögren Syndrome, and Dermatomyositis/Polymyositis). It is important to find serological markers as predictors of the evolution or severity of these diseases. The objective of this retrospective study was to investigate if there was a milder subgroup of UCTD with a special clinical profile consisting only in the presence of anti-Ro52 autoantibodies...
2017: International Journal of Rheumatology
https://www.readbyqxmd.com/read/28208969/acute-presentation-of-juvenile-dermatomyositis-with-subclinical-cardiac-involvement-a-rare-case
#7
Rhythm Khera, Shrayash Khare, Shailendra Kumar Singh
Cardiac involvements are common in patients with Dermatomyositis, most of which are not severe enough to present definite or readily observable symptoms. However, Cardiovascular (CVS) manifestations constitute a major cause of death in these patients. The most frequently reported clinically evident of CVS manifestations in-patient of dermatomyositis are Congestive Heart Failure (CHF), conduction aberrations, that may predispose to complete heart block and coronary artery disease. The principal pathophysiological mechanisms that may produce these cardiac manifestations involve coronary artery disease as well as small vessels vasculitis of the myocardium...
December 2016: Journal of Clinical and Diagnostic Research: JCDR
https://www.readbyqxmd.com/read/28203172/recurrent-multiple-squamous-cell-carcinomas-on-the-scalp-in-a-patient-with-juvenile-dermatomyositis
#8
Yota Sato, Taku Fujimura, Yumi Kambayashi, Akira Tsukada, Takanori Hidaka, Kayo Tanita, Takahiro Haga, Akira Hashimoto, Setsuya Aiba
Both long-term administration of immunosuppressive agents and chronic inflammatory conditions, such as autoimmune disease, could be risk factors for the development of cutaneous squamous cell carcinoma (cSCC). In this report, we present a case of recurrent multiple cSCC on the scalp in a patient with juvenile dermatomyositis who had been administered cyclosporine and Predonine since she was a 1-year-old infant. Interestingly, immunohistochemical staining revealed IL-17-producing cells adjacent to IL-17R-expressing atypical keratinocytes...
January 2017: Case Reports in Oncology
https://www.readbyqxmd.com/read/28202739/anti-hmgcr-autoantibodies-in-juvenile-idiopathic-inflammatory-myopathies-identify-a-rare-but-clinically-important-subset-of-patients
#9
Sarah L Tansley, Zoe E Betteridge, Stefania Simou, Thomas S Jacques, Clarissa Pilkington, Mark Wood, Kishore Warrier, Lucy R Wedderburn, Neil J McHugh
OBJECTIVE: We aimed to establish the prevalence and clinical associations of anti-HMG-CoA-reductase (anti-HMGCR) in a large UK cohort with juvenile myositis. METHODS: There were 381 patients investigated for anti-HMGCR using ELISA. RESULTS: Anti-HMGCR autoantibodies were detected in 4 patients (1%). These children had no or minimal rash and significant muscle disease. Muscle biopsies were considered distinctive, with widespread variation in fiber size, necrotic fibers, and chronic inflammatory cell infiltrates; all had prolonged elevation of creatine kinase and all ultimately received biologic therapies...
February 15, 2017: Journal of Rheumatology
https://www.readbyqxmd.com/read/28190982/dermatomyositis-leading-to-necrotizing-vasculitis-a-perfect-response-to-applied-therapy
#10
Mahmood Akbaryan, Farideh Darabi, Zahra Soltani
Dermatomyositis is an idiopathic inflammatory myopathy that cause skin and muscle complications. The ethiology is not understood well yet. Released cytokines including interferon and interleukins are suggested to make inflammatory responses in the skin or muscle. Muscle weakness and skin lesions including heliotrope rash, shawl sign and Gottron's papules are the most common symptoms. A biopsy (muscle or skin) is always the most reliable method for diagnosis. Corticosteroids in association with immunosuppressive agents are used as standard treatment...
December 2016: International Journal of Biomedical Science: IJBS
https://www.readbyqxmd.com/read/28185705/cardiovascular-magnetic-resonance-imaging-pattern-at-the-time-of-diagnosis-of-treatment-na%C3%A3-ve-patients-with-connective-tissue-diseases
#11
Sophie Mavrogeni, George Markousis-Mavrogenis, Loukia Koutsogeorgopoulou, Theodoros Dimitroulas, Konstantinos Bratis, George D Kitas, Petros Sfikakis, Maria Tektonidou, Georgia Karabela, Efthymios Stavropoulos, Gikas Katsifis, Kyriaki A Boki, Anastasia Kitsiou, Vasiliki Filaditaki, Elias Gialafos, Sotiris Plastiras, Vasiliki Vartela, Genovefa Kolovou
BACKGROUND-AIM: Cardiac involvement at diagnosis of connective tissue disease (CTD) has been described by echocardiography. We hypothesized that cardio-vascular magnetic resonance (CMR) detects occult lesions at CTD diagnosis. PATIENTS-METHODS: CMR was performed early after diagnosis in 78 treatment-naïve CTDs (aged 43±11, 59F/19M) without cardiac involvement [5 Takayasu arteritis (TA), 4 Churg Strauss syndrome (CSS), 5 Wegener granulomatosis (WG), 16 systemic lupus erythematosus (SLE), 12 rheumatoid arthritis (RA), 8 mixed connective tissue diseases (MCTD), 12 ankylosing spondylitis (AS), 3 polymyalgia rheumatica (PMR), 8 systemic sclerosis (SSc) and 5 dermatomyositis (DM)]...
February 1, 2017: International Journal of Cardiology
https://www.readbyqxmd.com/read/28183315/pyruvate-kinase-m2-and-the-mitochondrial-atpase-inhibitory-factor-1-provide-novel-biomarkers-of-dermatomyositis-a-metabolic-link-to-oncogenesis
#12
Fulvio Santacatterina, María Sánchez-Aragó, Marc Catalán-García, Glòria Garrabou, Cristina Nuñez de Arenas, Josep M Grau, Francesc Cardellach, José M Cuezva
BACKGROUND: Metabolic alterations play a role in the development of inflammatory myopathies (IMs). Herein, we have investigated through a multiplex assay whether proteins of energy metabolism could provide biomarkers of IMs. METHODS: A cohort of thirty-two muscle biopsies and forty plasma samples comprising polymyositis (PM), dermatomyositis (DM) and sporadic inclusion body myositis (sIBM) and control donors was interrogated with monoclonal antibodies against proteins of energy metabolism using reverse phase protein microarrays (RPPA)...
February 10, 2017: Journal of Translational Medicine
https://www.readbyqxmd.com/read/28181122/association-between-rheumatic-diseases-and-cancer-results-from-a-clinical-practice-cohort-study
#13
Mattia Bellan, Enrico Boggio, Daniele Sola, Antonello Gibbin, Alessandro Gualerzi, Serena Favretto, Giulia Guaschino, Ramona Bonometti, Roberta Pedrazzoli, Mario Pirisi, Pier Paolo Sainaghi
The association between cancer and immune-mediated rheumatic conditions is controversial, especially as far as polymyalgia rheumatica (PMR) is concerned. Furthermore, no clinical feature has been shown to be suggestive of a paraneoplastic rheumatic syndrome. With the present study, we aim to address both these issues. The study population comprised N = 1750 patients, including N = 100 with PMR, who attended our tertiary immuno-rheumatology clinic between January 1, 2005 and November 30, 2012. A rheumatic disease was deemed paraneoplastic if cancer had been diagnosed in the 2 years preceding or following its onset...
February 8, 2017: Internal and Emergency Medicine
https://www.readbyqxmd.com/read/28174638/juvenile-dermatomyositis-in-a-4-year-old-kenyan-girl
#14
Marlous L Grijsen, Deborah Mchaile, Inge Geut, Raimos Olomi, Maitseo Nwako, Luis Requena, William P Howlett, Daudi R Mavura, Marieke C J Dekker
To our knowledge, this is the first case report of juvenile dermatomyositis (JDM) in Tanzania. It demonstrates that the characteristic cutaneous findings of JDM may easily be overlooked, especially on dark skin, and the difficulty of clinical management in resource-constrained settings.
February 2017: Clinical Case Reports
https://www.readbyqxmd.com/read/28168623/a-report-of-two-cases-of-solid-facial-edema-in-acne
#15
Sarah Kuhn-Régnier, Joanna Mangana, Katrin Kerl, Jivko Kamarachev, Lars E French, Antonio Cozzio, Alexander A Navarini
INTRODUCTION: Solid facial edema (SFE) is a rare complication of acne vulgaris. To examine the clinical features of acne patients with solid facial edema, and to give an overview on the outcome of previous topical and systemic treatments in the cases so far published. METHODS: We report two cases from Switzerland, both young men with initially papulopustular acne resistant to topical retinoids. RESULTS: Both cases responded to oral isotretinoin, in one case combined with oral steroids...
February 6, 2017: Dermatology and Therapy
https://www.readbyqxmd.com/read/28164107/vascular-changes-and-perifascicular-muscle-fiber-damage-in-dermatomyositis-another-question-of-the-chicken-or-the-egg-that-is-on-our-mind
#16
COMMENT
Boel De Paepe
No abstract text is available yet for this article.
January 2017: Annals of Translational Medicine
https://www.readbyqxmd.com/read/28158183/beyond-the-mhc-a-canine-model-of-dermatomyositis-shows-a-complex-pattern-of-genetic-risk-involving-novel-loci
#17
Jacquelyn M Evans, Rooksana E Noorai, Kate L Tsai, Alison N Starr-Moss, Cody M Hill, Kendall J Anderson, Thomas R Famula, Leigh Anne Clark
Juvenile dermatomyositis (JDM) is a chronic inflammatory myopathy and vasculopathy driven by genetic and environmental influences. Here, we investigated the genetic underpinnings of an analogous, spontaneous disease of dogs also termed dermatomyositis (DMS). As in JDM, we observed a significant association with a haplotype of the major histocompatibility complex (MHC) (DLA-DRB1*002:01/-DQA1*009:01/-DQB1*001:01), particularly in homozygosity (P-val = 0.0001). However, the high incidence of the haplotype among healthy dogs indicated that additional genetic risk factors are likely involved in disease progression...
February 2017: PLoS Genetics
https://www.readbyqxmd.com/read/28154280/an-atypical-clinical-course-of-anti-mda5-antibody-positive-interstitial-lung-disease-in-a-patient-with-three-deteriorations-in-9-years
#18
Yuki Sato, Kojiro Otsuka, Koji Tamai, Yuichiro Ono, Yasuhito Hamaguchi, Keisuke Tomii
Anti-MDA5 antibody-positive patients with clinically amyopathic dermatomyositis (CADM) are at high risk of developing rapidly progressive interstitial lung disease (ILD), which is associated with a high mortality rate. Approximately half of the patients with ILD recover; however, the long-term clinical course of these patients has not been fully reported and is not completely understood. This report describes the atypical clinical course of an anti-MDA5 antibody-positive CADM patient who experienced three deteriorations of ILD in 9 years...
2017: Internal Medicine
https://www.readbyqxmd.com/read/28148694/myalgia-in-patients-with-dermatomyositis-and-polymyositis-is-attributable-to-fasciitis-rather-than-myositis-a-retrospective-study-of-32-patients-who-underwent-histopathological-examinations
#19
Kentaro Noda, Ken Yoshida, Taro Ukichi, Kazuhiro Furuya, Kenichiro Hirai, Isamu Kingetsu, Daitaro Kurosaka
OBJECTIVE: To determine the association between fasciitis and the clinical variables in patients with dermatomyositis (DM) and polymyositis (PM). METHODS: We retrospectively reviewed the medical records of 32 patients (24 DM, 8 PM) with newly diagnosed DM and PM and in whom fascia and muscle specimens were histopathologically examined. The relationship between fasciitis and the clinical variables was statistically analyzed. These included age, sex, myalgia, muscle weakness, creatine kinase (CK) and aldolase activities, anti-Jo1 antibody, interstitial lung disease, and malignancy...
February 1, 2017: Journal of Rheumatology
https://www.readbyqxmd.com/read/28143550/spectrum-of-paediatric-rheumatic-diseases-in-nigeria
#20
Babatunde Hakeem Olaosebikan, Olufemi Oladipo Adelowo, Barakat Adeola Animashaun, Richard Oluyinka Akintayo
BACKGROUND: Paediatric rheumatology service in Sub-Sahara African is virtually not available as there is a shortage of paediatric rheumatologists and other rheumatology health professionals. We aim to describe the clinical spectrum and the frequencies of paediatric rheumatic diseases (PRDs) in Lagos State University Teaching Hospital (LASUTH), Lagos, Nigeria. METHODS: This is a retrospective review of patients with PRDs seen over a five year period (March 2010 to February 2016) at the rheumatology clinic and children ward of LASUTH...
January 31, 2017: Pediatric Rheumatology Online Journal
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