Favourable outcome in 135 children with juvenile systemic sclerosis: results of a multi-national survey

Objective. To increase the current knowledge of the outcome of juvenile sys temic sclerosis (jSSc), which is currently limited. Methods. In order to investigate the patient outcome and prognostic factors , starting October 1994, we distributed questionnaires to 324 paediatric rh eumatology centres. Results. Until 15 May 1998 responses from 46 centres were received, 34 of w hich returned completed questionnaires on a total of 135 patients. One hund red and twenty-two of the 135 patients were Caucasian, 100 were female. The mean age at disease onset was 8.8 yr (s.D. +/- 3.3 yr). The mean disease d uration at the last follow-up was 5 yr(s.D. +/- 3.3 yr). At the last follow -up the disease was still active and required medication in 82 patients, 36 had inactive disease on medication, and 16 were in remission. Ninety per c ent of the living patients were fully active in daily life at the last foll ow-up. Eight of the 135 patients had died. These patients had a median age at onset of the disease of 10.5 yr (range 6.7-15.8 yr). The median disease duration until death was 2 yr (range 1-8 yr). The causes of death were hear t failure (five), renal failure (one), sepsis (one) and in one case the cau se was not defined. The 1 yr survival rate was 99%, the 2 yr was 97% and th e 4 yr was 95%. Systemic sclerosis (SSc) is a rare disease in adults and ch ildren [1]. Patients with juvenile onset represent almost 10% of all patien ts with scleroderma [2]. Of all patients with SSc, 1.5% develop evidence of the disease before the age of 10 yr [3], and an additional 7.2% develop si gns and symptoms between 10 and 19 yr of age [3]. Currently, information on survival and prognostic factors for survival in adult SSc patients is incr easing [4-7], but is still lacking in regard to juvenile SSc (jSSc). To lea rn about jSSc, we distributed a survey to 324 paediatric rheumatology centr es starting October 1994. In the following we present the final collected r esults as of 15 May 1998.