Journal
PEDIATRIC CLINICS OF NORTH AMERICA
Volume 65, Issue 4, Pages 757-+Publisher
W B SAUNDERS CO-ELSEVIER INC
DOI: 10.1016/j.pcl.2018.04.002
Keywords
Juvenile localized scleroderma; Juvenile systemic sclerosis; Pediatric scleroderma; Morphea; Pediatric rheumatology; Extracutaneous involvement; Morbidity
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Funding
- Bristol-Myers Squibb
- Childhood Arthritis and Rheumatology Research Alliance (CARRA)
- Arthritis Foundation
- Scleroderma Foundation
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Scleroderma is a rare disease that has two main forms: localized scleroderma (LS) and systemic sclerosis (SSc). Both are chronic diseases, can present in different patterns (subtypes), and are associated with extracutaneous involvement in pediatric patients. Morbidity and mortality is much worse for juvenile SSc, with patients at risk for life-threatening lung, heart, and other visceral organ fibrosis and vasculopathy. Mortality is extremely rare in juvenile LS, but morbidity is common, with patients at risk for severe disfigurement and functional impairment. Scleroderma treatment is directed toward controlling inflammation and managing specific problems. Early diagnosis can greatly improve outcome.
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