Juvenile systemic sclerosis in children (clinical case)

[Juvenile Systemic Sclerosis].

mento cutâneo, evoluindo para áreas de despigmentação cutânea em ‘sal e pimenta’ (Fig. 1), e aparecimento de úlceras digitais (Fig. 2). Actualmente medicada com metotrexato subcutâneo, metilprednisolona e bosentan. A ESJ tem uma incidência de 0,27 casos por milhão de crianças1 e apresenta particularidades que a distinguem das formas adultas de esclerose sistémica.2 A abordagem terapêutica em id...

Juvenile-onset systemic sclerosis: children are not small adults.

The majority of childhood-onset rheumatic diseases differ markedly in presentation and management from their equivalent adult conditions, and juvenile-onset systemic sclerosis (JSSc) is no exception. Chronic ill health from any cause in childhood impacts heavily, not only on physical growth and development, but also in terms of social, educational and psychological development. Even localized s...

Systemic sclerosis in children

Factors affecting survival in juvenile systemic sclerosis.

OBJECTIVES To determine whether demographic, clinical and immunological features may predict the outcome in juvenile SSc (JSSc). METHODS Clinical and laboratory characteristics of patients with JSSc collected from paediatric rheumatology centres worldwide were analysed. First, univariate tests identified those features significantly related with fatal outcome, and then multivariate logistic r...

Juvenile systemic sclerosis: review of 15 patients

Results Results: Fifteen patients were identified and included in the analysis, 3 of them were overlap syndromes. Eleven girls (73%), 13 (87%) Caucasians, with a mean age at diagnosis of 11.1±3.0 (3–15) years and a mean disease duration of 7.2±4.2 years (8 months-17 years). In 14 (93%) cases, the first symptom attributable to JSSc was Raynaud’s phenomenon, followed by arthritis and/or puffy han...