Systemic sclerosis in childhood: clinical and immunologic features of 153 patients in an international database.
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 approximately 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.


CONCLUSIONS
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.
Martini
Giorgia
G
University of Padua, Padua, Italy.
Foeldvari
Ivan
I
Russo
Ricardo
R
Cuttica
Ruben
R
Eberhard
Anne
A
Ravelli
Angelo
A
Lehman
Thomas J A
TJ
de Oliveira
Sheila Knupp Feitosa
SK
Susic
Gordana
G
Lyskina
Galina
G
Nemcova
Dana
D
Sundel
Robert
R
Falcini
Fernanda
F
Girschick
Herman
H
Lotito
Ana Paula
AP
Buoncompagni
Antonella
A
Sztajnbok
Flavio
F
Al-Mayouf
Sulaiman M
SM
Orbàn
Ilonka
I
Ferri
Clodoveo
C
Athreya
Balu H
BH
Woo
Patricia
P
Zulian
Francesco
F
D000293:Adolescent; D000328:Adult; D017668:Age of Onset; D001323:Autoantibodies / Q000097:blood; D001327:Autoimmune Diseases / Q000209:etiology / Q000276:immunology / Q000503:physiopathology; D002648:Child; D002675:Child, Preschool; D016208:Databases, Factual; D005260:Female; D006801:Humans; D007223:Infant; D007391:International Cooperation; D008297:Male; D008875:Middle Aged; D009140:Musculoskeletal Diseases / Q000209:etiology / Q000276:immunology / Q000503:physiopathology; D011928:Raynaud Disease / Q000209:etiology / Q000276:immunology / Q000503:physiopathology; D012595:Scleroderma, Systemic / Q000150:complications / Q000276:immunology / Q000401:mortality / Q000503:physiopathology; D015996:Survival Rate
D001323:Autoantibodies