title: Update on juvenile systemic sclerosis.
abstract: Juvenile systemic sclerosis is an orphan disease of the macro- and microvasculature with an unknown etiology. Although pediatric and adult classification systems have been developed, neither has been validated yet. Recent publications have shown a shift in the subset pattern in juvenile-onset adult patients, with a better outcome in these patients as compared with young adult patients. As a first step in assessing activity, damage, and severity, a pediatric severity score--a modification of the adult Medsger scale--was proposed but has not been validated. An activity score also has been developed but has not yet been published. Currently, treatment approaches are based only on adult data, and problems exist in extrapolating these data to a pediatric population.
authors.affiliation: Hamburg Centre for Pediatric and Adolescent 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, foeldvari@t-online.de.
authors.forename: Ivan
authors.initials: I
authors.lastname: Foeldvari
mesh_terms: D017668:Age of Onset; D002648:Child; D006801:Humans; D035583:Rare Diseases; D012595:Scleroderma, Systemic / Q000175:diagnosis* / Q000503:physiopathology; D012720:Severity of Illness Index