Juvenile and young adult-onset systemic sclerosis share the same organ involvement in adulthood: data from the EUSTAR database
Foeldvari I et al. – The subset distribution in the juvenile SSc (jSSc) and aSSc cohorts was found to be similar. Only the frequency of ACAs was significantly lower in the jSSc, which supports the hypothesis that the SSc patients with paediatric onset in the adult cohort may represent a distinct subgroup of the complete cohort of paediatric patients.Methods
- From the EUSTAR SSc cohort two patient groups were analysed: patients with juvenile SSc (jSSc) who are adults at present, and patients diagnosed between the age of 20 and 40 years (aSSc).
- Demographic data of the patients, organ involvement and outcome of the disease were examined using the Minimal Essential Data Set database system.
- From 5000 patients in the EUSTAR cohort, 60 patients (1.2%) with jSSc and 910 patients (18%) with aSSc were selected according the inclusion criteria.
- In the jSSc group, the mean age of disease onset was 12.4 years (range 2–15.9 years), and in the aSSc group, the mean age was 32 years (range 20–40 years).
- Disease subsets were similar.
- The antibody profile was also comparable except for ACAs, which were positive in 5% of the jSSc group and 26.9% of the aSSc group (P < 0.005).
- Organ involvement (lung, kidney, joint, muscle and heart) was similar in the two groups of patients at the time of the last follow–up.