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Juvenile dermatomyositis

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https://www.readbyqxmd.com/read/29037322/improvement-of-nailfold-capillary-microangiopathy-after-immunosuppressant-therapy-in-a-child-with-clinically-amyopathic-juvenile-dermatomyositis
#1
Lúcia Maria Arruda Campos, Adriana M E Sallum, Cintia Z Camargo, Luís Eduardo C Andrade, Cristiane Kayser
No abstract text is available yet for this article.
September 2017: Revista Brasileira de Reumatologia
https://www.readbyqxmd.com/read/29037319/devic-s-disease-in-an-adolescent-girl-with-juvenile-dermatomyositis
#2
Melissa Mariti Fraga, Enedina Maria Lobato de Oliveira, Claudio Arnaldo Len, Maria Fernanda Campos, Maria Teresa Terreri
No abstract text is available yet for this article.
September 2017: Revista Brasileira de Reumatologia
https://www.readbyqxmd.com/read/29029283/trajectories-of-cardiorespiratory-fitness-in-patients-with-juvenile-dermatomyositis
#3
Kiki J Blom, Tim Takken, Barbara C H Huijgen, Judith Wienke, Annet van Royen-Kerkhof, Marco van Brussel
Objectives: Previous research demonstrated decreased cardiorespiratory fitness (CRF) in patients with JDM during active disease and remission. However, longitudinal data regarding trajectories of CRF are currently lacking. The objective of this study was to determine trajectories of CRF in patients with both monocyclic and chronic JDM, and to identify potential predictors of these trajectories. Methods: Thirty-six patients with JDM [median age (interquartile range) at diagnosis: 8...
September 27, 2017: Rheumatology
https://www.readbyqxmd.com/read/28977549/2016-acr-eular-adult-dermatomyositis-and-polymyositis-and-juvenile-dermatomyositis-response-criteria-methodological-aspects
#4
Lisa G Rider, Nicolino Ruperto, Angela Pistorio, Brian Erman, Nastaran Bayat, Peter A Lachenbruch, Howard Rockette, Brian M Feldman, Adam M Huber, Paul Hansen, Chester V Oddis, Ingrid E Lundberg, Anthony A Amato, Hector Chinoy, Robert G Cooper, Lorinda Chung, Katalin Danko, David Fiorentino, Ignacio García-De la Torre, Ann M Reed, Yeong Wook Song, Rolando Cimaz, Rubén J Cuttica, Clarissa A Pilkington, Alberto Martini, Janjaap van der Net, Susan Maillard, Frederick W Miller, Jiri Vencovsky, Rohit Aggarwal
Objective: The objective was to describe the methodology used to develop new response criteria for adult DM/PM and JDM. Methods: Patient profiles from prospective natural history data and clinical trials were rated by myositis specialists to develop consensus gold-standard ratings of minimal, moderate and major improvement. Experts completed a survey regarding clinically meaningful improvement in the core set measures (CSM) and a conjoint-analysis survey (using 1000Minds software) to derive relative weights of CSM and candidate definitions...
August 2, 2017: Rheumatology
https://www.readbyqxmd.com/read/28957568/presentations-and-outcomes-of-juvenile-dermatomyositis-patients-admitted-to-intensive-care-units
#5
Alix Besançon, Karine Brochard, Laurent Dupic, Cyril Gitiaux, Marianne Delville, Saoussen Krid, Pierre Quartier, Elsa Saire, Rémi Salomon, Cécile Talbotec, Christine Bodemer, Brigitte Bader-Meunier
No abstract text is available yet for this article.
October 1, 2017: Rheumatology
https://www.readbyqxmd.com/read/28957546/life-threatening-systemic-capillary-leak-syndrome-in-juvenile-dermatomyositis
#6
Alessandra Meneghel, Giorgia Martini, Carolina Birolo, Alvise Tosoni, Andrea Pettenazzo, Francesco Zulian
No abstract text is available yet for this article.
October 1, 2017: Rheumatology
https://www.readbyqxmd.com/read/28954874/fibrous-arthropathy-associated-with-morphea-a-new-cause-of-diffuse-acquired-joint-contractures
#7
Etienne Merlin, Sylvain Breton, Sylvie Fraitag, Jean-Louis Stéphan, Carine Wouters, Christine Bodemer, Brigitte Bader-Meunier
Etiologies for childhood-onset diffuse joint contractures encompass a large group of inherited disorders and acquired diseases, in particular a subtype of juvenile idiopathic arthritis called "dry polyarthritis," dermatomyositis, and systemic sclerosis. We report on 2 boys, aged 5 and 8 years, who developed acquired symmetric painless joint contractures preceding the development of superficial plaques of morphea by 7 to 13 months. There was no other clinical involvement, biological inflammation, or autoantibodies...
October 2017: Pediatrics
https://www.readbyqxmd.com/read/28941175/myogenic-progenitor-cells-exhibit-ifn-type-i-driven-pro-angiogenic-properties-and-molecular-signature-during-juvenile-dermatomyositis
#8
Cyril Gitiaux, Claire Latroche, Michèle Weiss-Gayet, Mathieu P Rodero, Darragh Duffy, Brigitte Bader Meunier, Christophe Glorion, Patrick Nusbaum, Christine Bodemer, Guy Mouchiroud, Jamel Chelly, Stéphane Germain, Isabelle Desguerre, Bénédicte Chazaud
OBJECTIVE: Juvenile DermatoMyositis (JDM) is an inflammatory pediatric myopathy characterized by focal capillary loss in muscle, followed by a progressive recovery upon adequate immunomodulating treatments, although some patients remain refractory to treatments. While the underlying mechanism of capillary depletion remains uncertain, recent studies have identified an upregulation of type I Interferon (IFN-I) expression specific to JDM. Given that myogenic precursor cells (MPCs) exert pro-angiogenic activity during normal skeletal muscle regeneration, we hypothesized that they may also modulate vascular remodeling /angiogenesis during JDM...
September 21, 2017: Arthritis & Rheumatology
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
#9
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/28911766/juvenile-dermatomyositis-key-roles-of-muscle-magnetic-resonance-imaging-and-early-aggressive-treatment
#10
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
#11
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
#12
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/28871670/-early-detection-of-endothelial-dysfunction-in-children-with-autoimmune-diseases-by-a-novel-noninvasive-technique
#13
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/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/28855174/the-euromyositis-registry-an-international-collaborative-tool-to-facilitate-myositis-research
#15
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/28805016/microvascular-abnormalities-assessed-by-nailfold-capillaroscopy-in-juvenile-dermatomyositis-after-medium-to-long-term-follow-up
#16
Zoltan Barth, Birgit N Witczak, Berit Flatø, Akos Koller, Ivar Sjaastad, Helga Sanner
OBJECTIVE: In juvenile dermatomyositis (JDM), microvascular abnormalities measured by nailfold capillaroscopy (NFC) are common early in disease course. We aimed to compare the presence of NFC abnormalities in patients with medium- to longstanding JDM with that of controls, and to explore associations between NFC abnormalities and disease activity and other disease characteristics. METHODS: Fifty-eight JDM patients clinically examined median 16.8 (2-38) years after disease onset were compared with matched controls...
August 13, 2017: Arthritis Care & Research
https://www.readbyqxmd.com/read/28794565/superimposed-segmental-manifestation-of-juvenile-amyopathic-dermatomyositis-in-a-9-year-old-boy
#17
Isil Bulur, Hilal Kaya Erdogan, Zeynep Nurhan Saracoglu, Rudolf Happle, Funda Canaz
Dermatomyositis is a well-known autoimmune disorder. On the other hand, juvenile amyopathic dermatomyositis (JADM) occurs rather rarely. Here, we report an unusual case in a 9-year-old Turkish boy showing a unilateral linear inflammatory skin lesion that was followed, after 16 months, by the appearance of bilateral disseminated features JADM.
July 2017: Indian Journal of Dermatology
https://www.readbyqxmd.com/read/28776361/first-report-of-anti-tif1%C3%AE-dermatomyositis-in-a-patient-with-myelodysplastic-syndrome
#18
B Palterer, G Vitiello, D Cammelli
Inflammatory myopathies as para-neoplastic phenomena were first described by Sterz in 1916. Recently, myositis specific autoantibodies were described in cancer-associated myositis. Anti-transcription intermediary factor 1 gamma (anti-TIF1γ) antibodies have been found in both young adults affected by juvenile dermatomyositis and in elderly patients with cancer-associated myositis. In this regard, we report herein the first case of anti-TIF1γ dermatomyositis secondary to a myelodysplastic syndrome.
August 3, 2017: Reumatismo
https://www.readbyqxmd.com/read/28774458/ultrasound-in-arthritis
#19
REVIEW
Iwona Sudoł-Szopińska, Claudia Schueller-Weidekamm, Athena Plagou, James Teh
Ultrasound is currently performed in everyday rheumatologic practice. It is used for early diagnosis, to monitor treatment results, and to diagnose remission. The spectrum of pathologies seen in arthritis with ultrasound includes early inflammatory features and associated complications. This article discusses the spectrum of ultrasound features of arthritides seen in rheumatoid arthritis and other connective tissue diseases in adults, such as Sjögren syndrome, lupus erythematosus, dermatomyositis, polymyositis, and juvenile idiopathic arthritis...
September 2017: Radiologic Clinics of North America
https://www.readbyqxmd.com/read/28763333/recent-clinical-trials-in-idiopathic-inflammatory-myopathies
#20
Valérie Leclair, Ingrid E Lundberg
PURPOSE OF REVIEW: Idiopathic inflammatory myopathies (IIMs) are complex multisystemic autoimmune diseases. Glucocorticoids remain the cornerstone of treatment in IIM, and the benefit of additional immunosuppressors is still debated. A limited number of controlled clinical trials have been available to support treatment guidelines, but in the last year, several clinical trials have been published. In this review, the highlights of recently published and on-going clinical trials in IIM will be summarized and discussed...
November 2017: Current Opinion in Rheumatology
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