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临床风湿病学幼年系统性硬皮病最佳实践复习 - 更新和实践要点。

Review for best practice in clinical rheumatology juvenile systemic sclerosis - Updates and practice points.

机构信息

Hamburg Centre for Pediatric and Adolescence Rheumatology, Centre for Treatment of Scleroderma and Uveitis in Childhood and Adolescence, Teaching Unit of the Asklepios Campus of the Semmelweis Medical School, Budapest, An der Schön Klinik Hamburg Eilbek, Dehnhaide 120, 22081, Hamburg, Germany.

Division of Pediatric Rheumatology, UPMC & University of Pittsburgh Scleroderma Center, Pediatric Scleroderma Clinic, University of Pittsburgh |UPMC Children's Hospital of Pittsburgh, 4401 Penn Ave, Pittsburgh, PA, 15224, USA.

出版信息

Best Pract Res Clin Rheumatol. 2021 Sep;35(3):101688. doi: 10.1016/j.berh.2021.101688. Epub 2021 Apr 22.

Abstract

Juvenile systemic sclerosis (jSSc) is a rare, severe autoimmune disease associated with life-threatening multiorgan inflammatory-driven fibrosis. Recognition early in the disease process, when treatment is more effective, is critical. We outline insights from the authors, who specialize and host jSSc cohorts, combined with recent literature review combining available juvenile-onset and applicable adult-onset studies regarding SSc evaluation, which can be extrapolated to children. Practice tips are provided for each main organ system.

摘要

儿童系统性硬化症(jSSc)是一种罕见的严重自身免疫性疾病,与危及生命的多器官炎症驱动的纤维化有关。在疾病过程早期识别,此时治疗效果更佳,这一点至关重要。我们概述了专门从事 jSSc 队列研究的作者的观点,并结合最近的文献综述,综合了关于 SSc 评估的青少年发病和适用的成人发病研究,这些研究可以外推至儿童。针对每个主要器官系统提供了实践技巧。

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