167 research outputs found
When is a autologous bone marrow transplantation indicated in the treatment of juvenile systemic sclerorsis? Results of a multinational survey of Pediatric Rheumatologist
Update of the results of a multinational survey regarding diagnosis and treatment of the temporomandibulare joint involvement in juvenile idiopathic arthritis- reflection of the day to day practice
Recent Advances in Systemic Scleroderma in Childhood
Ann Paediatr Rheumatol
Annals of paediatric rheumatolog
http://www.aprjournal.org/
2146-2909 (Print
Juvenile and young adult-onset systemic sclerosis share the same organ involvement in adulthood: data from the EUSTAR database
Objective. The aim of the present study was to explore the long-term outcome and clinical characteristics of adult patients with juvenile onset in the EULAR Scleroderma Trials and Research (EUSTAR) cohort and compare them with adult patients with onset between 20 and 40 years of age. 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. Results. 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. Conclusion. The subset distribution in the 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 patient
S2k guideline: Diagnosis and management of cutaneous lupus erythematosus – Part 2: Therapy, risk factors and other special topics
S2k guideline: Diagnosis and management of cutaneous lupus erythematosus – Part 1: Classification, diagnosis, prevention, activity scores
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