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- Title
Vasculopathy-related clinical and pathological features are associated with severe juvenile dermatomyositis.
- Authors
Gitiaux, Cyril; De Antonio, Marie; Aouizerate, Jessie; Gherardi, Romain K.; Guilbert, Thomas; Barnerias, Christine; Bodemer, Christine; Brochard-Payet, Karine; Quartier, Pierre; Musset, Lucile; Chazaud, Bénédicte; Desguerre, Isabelle; Bader-Meunier, Brigitte
- Abstract
Objective. Outcome of JDM is highly heterogeneous. Our objective was to determine clinical and muscle biopsy features associated with poor outcome and response to treatment. Methods. Clinical data and muscle biopsy were obtained from a monocentric cohort of 29 patients. Clinical subgroups were defined by latent class model analysis of initial and follow-up parameters. Myopathological features were analysed using validated scores. Capillary loss was determined on reconstructions of transversal sections and assessed in the different age groups to take into account variations of muscle capillarization during post-natal development. Regression models were used to identify initial predictors of therapeutic response. Results. Two distinct homogeneous subgroups of patients were identified according to clinical severity and pathological findings. The smallest group of patients (7/29) presented with severe JDM. Compared with the other group (22/29), patients had more severe muscle weakness at disease onset, low remission rate at 12 months, frequent subcutaneous limb oedema or gastrointestinal (GI) involvement and higher myopathological scores (capillary dropout, perifascicular necrosis/regeneration, fibres with internal myonuclei and fibrosis subscores). Relevance of capillary dropout to JDM severity was substantiated by agebased analysis, confirming its major role in JDM pathophysiology. Most of these manifestations could be related to vasculopathy (limb oedema, GI involvement, capillary dropout). Furthermore, 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. Conclusion. Vasculopathy is prominent in severe JDM. Simple criteria can be used at initial evaluation to identify patients requiring a more intensive therapy.
- Subjects
FRANCE; BIOPSY; CHILDREN'S health; DERMATOMYOSITIS; FISHER exact test; LONGITUDINAL method; EVALUATION of medical care; MULTIVARIATE analysis; REGRESSION analysis; STATISTICS; LOGISTIC regression analysis; DATA analysis; RETROSPECTIVE studies; SEVERITY of illness index; DATA analysis software; MANN Whitney U Test; PROGNOSIS
- Publication
Rheumatology, 2016, Vol 55, Issue 3, p470
- ISSN
1462-0324
- Publication type
Article
- DOI
10.1093/rheumatology/kev359