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FRI0187 The clinical profile of juvenile systemic sclerosis (jssc)
  1. MN Starovoytova,
  2. NG Guseva
  1. Depatment of Microcirculation and Inflammation, Institute of Rheumatology, RAMS, Moscow, Russia

Abstract

Background JSSc represents the unique form of SSc characterised by variable f clinical picture. JSSc has specific features maintained during adult period of life.

Objectives The aim of the study was to determine the clinical profile of JSSc.

Methods Sixty pts with JSSc aged from 14 to 54 years (mean 25.1+7.2 yrs) with the onset of the disease at the age of 1 to 16 years (mean 11.4+3.8 yrs) were analysed. Duration of disease was 13.1+7.1, range 1–39 yrs.

Results We revealed three clinical variants of JSSc. The first variant (55% of pts) is characterised by the combination of localised scleroderma (morfhea, hemiform-typed) and systemic manifestation such as Raynaud's phenomenon (78%), arthritis (41%), lungs involvement (66%), heart disease (58%), gastrointestinal tract involvement (60%). The visceral signs were moderate and founded by careful clinical instrumental investigation. In the other variant (38% pts) the clinical manifestation of JSSc were similar to adult lSSc with the prevalence of vascular disorders and digital ischaemic changes, limited skin lesion, visceral organ involvement. Musculoskeletal manifestations (arthralgia, arthritis, flexon contractures) were also seen in this group. The pts hadƒslow progressive form of disease. The third variant (17% of pts) may be regarded as overlap syndrome SSc with polymiositis or/and rheumatoid arthritis. The state in these pts mainly depended on the prevailing disease process.

Conclusion The definition of the clinical variants of JSSc may provide the correct diagnosis and adequate treatment.

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