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 Vol. 17 No. 4, April 2007
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AAP Grand Rounds 17:45-46 (2007)
© 2007 American Academy of Pediatrics

RHEUMATOLOGY

Juvenile Systemic Sclerosis: Still a Serious Disease

Source: Martini G, Foeldvari I, Russo R, et al. Systemic sclerosis in childhood: clinical and immunologic features of 153 patients in an international database. Arthritis Rheum. 2006;54:3971–3978; doi:10.1002/art.22207[Medline]

The first 20% of the full text of this article appears below.

Systemic sclerosis (SSc) has been a poorly understood rheumatic disease for which only limited treatment options have been available. In addition to skin induration and sclerodactyly, other symptoms include Raynaud phenomenon (RP), interstitial lung disease, pulmonary hypertension, cardiac arrhythmia and failure, renal crisis, hypertension, and gastroesophageal dysmotility. RP can be severe enough to cause digital pitting and infarct. Prevalence has been reported to be in the range of 15–24 per 100,000 persons, with up to 10% of adults reporting onset of symptoms during childhood.1 Morbidity and mortality remain high, with a mean adult survival reported to be approximately 12 years from diagnosis; mortality is most commonly secondary to renal, pulmonary, or cardiac disease.2 Information about pediatric patients with SSc is even more lacking than in adults, and there remain no accepted criteria for the diagnosis of juvenile SSc.

The authors of this study from multiple institutions used the 1980 American College of Rheumatology preliminary criteria to define the population of pediatric patients (disease onset <16 years of age) with SSc, . . . [Full Text of this Article]

Suzanne C. Li, MD, PhD, FAAP
Pediatric Rheumatology, Joseph M. Sanzari Children’s Hospital at HUMC, Hackensack, NJ, and Pediatrics, UMDNJ, Newark, NJ

 





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