title: Systemic sclerosis in childhood: clinical and immunologic features of 153 patients in an international database.
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 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.
authors.affiliation: University of Padua, Padua, Italy.
authors.forename: Giorgia
authors.initials: G
authors.lastname: Martini
authors.forename: Ivan
authors.initials: I
authors.lastname: Foeldvari
authors.forename: Ricardo
authors.initials: R
authors.lastname: Russo
authors.forename: Ruben
authors.initials: R
authors.lastname: Cuttica
authors.forename: Anne
authors.initials: A
authors.lastname: Eberhard
authors.forename: Angelo
authors.initials: A
authors.lastname: Ravelli
authors.forename: Thomas J A
authors.initials: TJ
authors.lastname: Lehman
authors.forename: Sheila Knupp Feitosa
authors.initials: SK
authors.lastname: de Oliveira
authors.forename: Gordana
authors.initials: G
authors.lastname: Susic
authors.forename: Galina
authors.initials: G
authors.lastname: Lyskina
authors.forename: Dana
authors.initials: D
authors.lastname: Nemcova
authors.forename: Robert
authors.initials: R
authors.lastname: Sundel
authors.forename: Fernanda
authors.initials: F
authors.lastname: Falcini
authors.forename: Herman
authors.initials: H
authors.lastname: Girschick
authors.forename: Ana Paula
authors.initials: AP
authors.lastname: Lotito
authors.forename: Antonella
authors.initials: A
authors.lastname: Buoncompagni
authors.forename: Flavio
authors.initials: F
authors.lastname: Sztajnbok
authors.forename: Sulaiman M
authors.initials: SM
authors.lastname: Al-Mayouf
authors.forename: Ilonka
authors.initials: I
authors.lastname: Orbàn
authors.forename: Clodoveo
authors.initials: C
authors.lastname: Ferri
authors.forename: Balu H
authors.initials: BH
authors.lastname: Athreya
authors.forename: Patricia
authors.initials: P
authors.lastname: Woo
authors.forename: Francesco
authors.initials: F
authors.lastname: Zulian
mesh_terms: 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
chemical_list: D001323:Autoantibodies