Document Detail

Juvenile and young adult-onset systemic sclerosis share the same organ involvement in adulthood: data from the EUSTAR database.
MedLine Citation:
PMID:  22728264     Owner:  NLM     Status:  Publisher    
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 patients.
Ivan Foeldvari; Alan Tyndall; Francesco Zulian; Ulf Müller-Ladner; László Czirjak; Chris Denton; Ottilia Kowal-Bielecka; Dominique Farge Bancel; Marco Matucci-Cerinic
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Publication Detail:
Type:  JOURNAL ARTICLE     Date:  2012-6-22
Journal Detail:
Title:  Rheumatology (Oxford, England)     Volume:  -     ISSN:  1462-0332     ISO Abbreviation:  -     Publication Date:  2012 Jun 
Date Detail:
Created Date:  2012-6-25     Completed Date:  -     Revised Date:  -    
Medline Journal Info:
Nlm Unique ID:  100883501     Medline TA:  Rheumatology (Oxford)     Country:  -    
Other Details:
Languages:  ENG     Pagination:  -     Citation Subset:  -    
Hamburger Zentrum für Kinder- und Jugendrheumatologie, Kompetenz Zentrum für Sklerodermie im Kindesalter, Kompetenz Zentrum für autoimmune Uveitis im Kindesalter, Am Klinikum Eilbek, Dehnhaide, Hamburg, Department of Rheumatology, University Basel, Basel, Switzerland, Pediatric Rheumatology Unit, University of Padua, Padua, Italy, Department of Rheumatology and Clinical Immunology, Justus-Liebig University Giessen, Bad Nauheim, Hesse, Germany, Department of Rheumatology and Immunology, University of Pécs, Pécs, Hungary, Centre for Rheumatology and Connective Tissue Diseases, UCL Medical School, London, UK, Department of Rheumatology and Internal Medicine, Medical University in Bialystok, Bialystok, Poland, Unité de Médecine Interne et Pathologie Vasculaire, Inserm U976, Hopital Saint-Louis, AP-HP,Paris-7 Université Denis Diderot, Paris, France, Department of Medicine and Department of Biomedicine, Division of Rheumatology, AOUC, Denothe Center, University of Florence, Florence, Italy.
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