Systemic sclerosis in childhood: Clinical and immunologic features of 153 patients in an international database

✍ Scribed by Giorgia Martini; Ivan Foeldvari; Ricardo Russo; Ruben Cuttica; Anne Eberhard; Angelo Ravelli; Thomas J. A. Lehman; Sheila Knupp Feitosa de Oliveira; Gordana Susic; Galina Lyskina; Dana Nemcova; Robert Sundel; Fernanda Falcini; Herman Girschick; Ana Paula Lotito; Antonella Buoncompagni; Flavio Sztajnbok; Sulaiman M. Al-Mayouf; Ilonka Orbàn; Clodoveo Ferri; Balu H. Athreya; Patricia Woo; Francesco Zulian

• Publisher: John Wiley and Sons
• Year: 2006
• Tongue: English
• Weight: 108 KB
• Volume: 54
• Category: Article
• ISSN: 0004-3591
• DOI: 10.1002/art.22207

✦ Synopsis

Abstract

Objective

To determine the clinical and immunologic features of systemic sclerosis (SSc) in a large group of children and describe the clinical evolution of the disease and compare it with the adult form.

Methods

Data on 153 patients with juvenile SSc collected from 55 pediatric rheumatology centers in Europe, Asia, and South and North America were analyzed. Demographic, clinical, and immunologic characteristics of children with juvenile SSc at the onset, at diagnosis, and during the disease course were evaluated.

Results

Raynaud's phenomenon was the most frequent symptom, followed by skin induration in ∼75% of patients. Musculoskeletal symptoms were present in one‐third of patients, and the most frequently involved internal organs were respiratory and gastrointestinal, while involvement of renal, cerebral, and cardiovascular systems was extremely rare. Antinuclear antibodies were present in the sera of 81% of patients. Anti–topoisomerase I (Scl‐70) and anticentromere antibodies were found to be positive in 34% and 7.1% of patients, respectively. Involvement of the respiratory, gastrointestinal, and cardiovascular systems was more frequent and occurred earlier in patients who died than in those who survived. Compared with the adult form, juvenile SSc appears to be less severe, with the involvement of fewer internal organs, particularly at the time of diagnosis, and has a less characterized immunologic profile.

Conclusion

This study provides information on the largest collection of patients with juvenile SSc ever reported. Juvenile SSc appears to be less severe than in adults because children have less internal organ involvement, a less specific autoantibody profile, and a better long‐term outcome.