Presentation: Similar Outcome and Organ Involvement Patter of Patients with Juvenile and Young Adult Onset Systemic Sclerosis. Data from the Eustar Database (2007)

1 Similar Outcome and Organ Involvement Patter of Patients with Juvenile and Young Adult Onset Systemic Sclerosis. Data from the Eustar Database

Introduction
Juvenile Systemic Sclerosis (jSSc) is a rare disease in childhood. Around 10% of patients develop the disease before the age of 16 years. The aim of the study was to explore the
long term outcome and clinical characteristics of adult patients of juvenile onset in the EUSTAR Systemic Scleroderma (aSSc) cohort. As a control population, we selected patients with young adult onset from the EULAR Slceroderma Trial and Research (EUSTAR) cohort with disease onset between the age of 20th and 40th year of life.
Methods
From the established EUSTAR database, in which at the time of the evaluation of data in April 2006 around 5000 patients were registered, two patient groups were evaluated, patients with a diagnosis of jSSc, who are adults now (aJSSc), and patients who were diagnosed between the age 20 to 40 (aSSc). Demographic data of the patients, organ involvement and outcome of the disease was examined.
Results
60 patients with ajSSc and 910 with aSSc were evaluated. In the ajSSc group the mean age of disease onset was 12.4 years (range 2-15.9 years) and in the aSSc group 32 years (range 20-40 years). The mean follow up was 17.64 years (1.8 to 54.8 years) in the ajSSc and 12.49 (range 2 to 47.5 years) in the aSSc. The disease was active at the time of last follow up in 26.6% in the ajSSc and in 24.8% in the aSSc group. The disease subsets were similar. In the ajSSc group had 40% a diffuse, 46.7% a limited subset and 8.3% an overlap syndrome. In the aSSc group had 37.8% a diffuse, 52.5% a limited subset and 8.6% an overlap syndrome. The antibody profile was also comparable with the exemption of anti-centromere antibodies being positive in 5% in the ajSSc and 26.9% in the aSSc group (p=0.0059). The organ involvement showed also a very similar pattern in the two groups of patients at the time of the last follow up.
Discussion
The subset distribution in the ajSSc and in aSSc cohort is similar and the organ involvement pattern as well. Only the percentage of anti-centromere antibodies is significantly lower in the ajSSc cohort. These similarities support the hypothesis that the ajSSc patients represent a selected surviving part of the complete group of the paediatric patients (survival bias), because 90% of the paediatric age group jSSc patients have a diffuse subset.

 I. Foeldvari, None; A. Tyndall, None; Z. Francesco, None; U. Mueller-Ladner, None; L. Czirjak, None; O. Kowal-Bielecka, None; D. Farge Bancel, None; M. Matucci-Cerinic, None.

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