Nailfold capillary density is importantly associated over time with muscle and skin disease activity in juvenile dermatomyositis
Heinrike Schmeling,Samantha Stephens,Cristina Goia,Cedric Manlhiot,Rayfel Schneider,Sanjeev Luthra,Elizabeth Stringer,Brian M. Feldman +7 more
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TLDR
NCD is a marker of skin and muscle disease activity, and is an important measure of disease activity changes from visit to visit, and may be useful when making treatment decisions.Abstract:
Objectives. To investigate the longitudinal association of nailfold capillary density (NCD; as a potential marker of activity) with various other clinical measures of disease activity and to evaluate baseline NCD as a predictor of disease outcome in children with JDM. Methods. Data from 809 clinic visits from 92 JDM patients were prospectively collected at each clinic visit over a time period of 5.5 years. The number of capillaries per millimetre at the distal nailfold was scored using a stereomicroscope. Disease activity was determined using the Childhood Myositis Assessment Scale (CMAS) and a modification of the validated disease activity score (DAS), which included three skin (SDAS) and three muscle (MDAS) criteria. An inception cohort subgroup (n = 28) with a baseline visit at diagnosis was analysed separately. Results. Both DAS subscores, MDAS (b =0.04437, P < 0.0001) and SDAS (b =0.1589, P < 0.0001), as well as the CMAS (b = 0.02165, P < 0.0001) were significantly associated with loss of end row nailfold capillary over time (multiple regression mixed-model analysis). All patients in the inception subcohort showed a reduced baseline NCD (diagnostic sensitivity = 100%) that improved as the disease improved, but this did not predict longer term outcome or course of disease. Conclusion. NCD is a marker of skin and muscle disease activity, and is an important measure of disease activity changes from visit to visit. Determination of capillary density may be useful when making treatment decisions. A decrease in NCD may be considered for inclusion in the diagnostic criteria due to its high sensitivity.read more
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Journal ArticleDOI
Measures of adult and juvenile dermatomyositis, polymyositis, and inclusion body myositis.
Lisa G. Rider,Victoria P. Werth,Adam M. Huber,Helene Alexanderson,Anand Prahalad Rao,Nicolino Ruperto,Laura Herbelin,Richard J. Barohn,David A. Isenberg,Frederick W. Miller +9 more
TL;DR: In this article, the authors focus on the assessment of myositis patients using a set of core set measures, including disease activity, quality of life, and disease damage, which can be used as outcomes for therapeutic trials.
Journal ArticleDOI
Consensus-based recommendations for the management of juvenile dermatomyositis
Felicitas Bellutti Enders,Brigitte Bader-Meunier,Eileen Baildam,Tamás Constantin,Pavla Dolezalova,Brian M. Feldman,Pekka Lahdenne,Bo Magnusson,Kiran Nistala,Seza Ozen,Clarissa Pilkington,Angelo Ravelli,Ricardo Russo,Yosef Uziel,Marco van Brussel,Janjaap van der Net,Sebastiaan J. Vastert,Lucy R. Wedderburn,Nicolaas Wulffraat,Liza J McCann,Annet van Royen-Kerkhof +20 more
TL;DR: Recommendations for the diagnosis and treatment of JDM have been formulated by an evidence-informed consensus process to produce a standard of care for patients with JDM throughout Europe.
Journal ArticleDOI
Idiopathic inflammatory myopathies.
TL;DR: This review focuses on DM, PM, and NM and examines current and promising therapies and the overlap syndrome, which refers to the association of PM, DM, or NM with connective tissue disease, such as scleroderma or systemic lupus erythematosus.
Journal ArticleDOI
Vasculopathy-related clinical and pathological features are associated with severe juvenile dermatomyositis
Cyril Gitiaux,Marie De Antonio,Jessie Aouizerate,Romain K. Gherardi,Thomas Guilbert,Christine Barnerias,Christine Bodemer,Karine Brochard-Payet,Pierre Quartier,Lucile Musset,Bénédicte Chazaud,Isabelle Desguerre,Brigitte Bader-Meunier +12 more
TL;DR: It is found that Childhood Myositis Assessment Scale <34 with either GI involvement or muscle endomysial fibrosis at disease onset were the best predictors of poor response to treatment.
Journal ArticleDOI
State of the art on nailfold capillaroscopy in dermatomyositis and polymyositis
TL;DR: An overview of the main nailfold capillaroscopy changes described in dermatomyositis and polymyositis confirms that NFC is a safe and noninvasive tool able to help the clinician in the diagnosis of DM and PM and to better characterize the phase of disease activity of these patients.
References
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Journal ArticleDOI
Polymyositis and dermatomyositis (first of two parts)
Anthony Bohan,James B. Peter +1 more
TL;DR: (First of Two Parts)
Journal ArticleDOI
Polymyositis and dermatomyositis (second of two parts).
Anthony Bohan,James B. Peter +1 more
Book
Muscles, Testing and Function
TL;DR: Muscles,Testing and function, Muscles, testing and function , کتابخانه دیجیتالی دانشگاه علوم پزشدکی و شهید بهشتی.
Book
Polymyositis and Dermatomyositis
TL;DR: Early initiation of therapy is essential, since both polymyositis and dermatomyositis respond to immunotherapeutic agents and new immunomodulatory agents currently being tested in controlled trials may prove promising for difficult cases.
Journal ArticleDOI
Juvenile dermatomyositis and other idiopathic inflammatory myopathies of childhood
TL;DR: Activation of dendritic cells with upregulation of genes induced by type-1 interferon (alpha) in muscle and peripheral blood seems to be central to disease pathogenesis and treatment often includes combinations of corticosteroids, methotrexate, and other immunosuppressive agents.
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