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Division of Pediatric Rheumatology, Hospital for Special Surgery, Sanford Weill Medical College, Cornell University, 535 East 70th Street, New York, NY 10021, USA. goldscout@aol.com
Progressive systemic sclerosis (PSS), or scleroderma, is a rare disease in the pediatric population. Many children with PSS have significant involvement of their internal organs, which leads to decreased survival. Because of the infrequency of the condition and delayed diagnosis, there are no large studies to evaluate therapy for PSS in children. Treatment is controversial in the adult literature, and its applicability to children is unclear. Only through collaborative efforts will researchers be able to clearly delineate the etiopathogenesis of PSS, and gather information from multicenter studies to ultimately provide appropriate and effective care for children with PSS.
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