A Practical Approach to Juvenile Dermatomyositis and Juvenile Scleroderma

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• 作者：Liza J. McCann ; Clare E. Pain
• 关键词：Juvenile dermatomyositis ; Juvenile localised scleroderma ; Juvenile systemic scleroderma ; Mixed connective tissue disease ; Overlap connective tissue disease
• 刊名：The Indian Journal of Pediatrics
• 出版年：2016
• 出版时间：February 2016
• 年：2016
• 卷：83
• 期：2
• 页码：163-171
• 全文大小：483 KB
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• 作者单位：Liza J. McCann (1)
  Clare E. Pain (1)
  
  1. Department of Rheumatology, Alder Hey Children’s NHS Foundation Trust, Eaton Road, Liverpool, L12 2AP, UK
  
• 刊物主题：Pediatrics; Gynecology;
• 出版者：Springer India
• ISSN：0973-7693

文摘

Juvenile dermatomyositis and juvenile scleroderma are rare multisystem autoimmune disorders. Although they share some pathognomonic hallmarks with adult onset myositis or scleroderma, there are significant differences in presentation, characteristics and associated features when the diseases present in childhood. In view of this, and the rarity of the conditions, it is important for care to be led by teams with expertise in pediatric rheumatology conditions. Prognosis has improved significantly in the West; likely due to early diagnosis and aggressive treatment with immunosuppressive medications. However, this trend is not replicated in the developing world. Early recognition of these diseases is crucial to achieve rapid and sustained remission and prevent disease or medication associated complications. This article aims to provide a practical overview for recognition, diagnosis and treatment of these conditions. Keywords Juvenile dermatomyositis Juvenile localised scleroderma Juvenile systemic scleroderma Mixed connective tissue disease Overlap connective tissue disease