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Publication Details
AFRICAN RESEARCH NEXUS
SHINING A SPOTLIGHT ON AFRICAN RESEARCH
immunology and microbiology
Systemic sclerosis in childhood: Clinical and immunologic features of 153 patients in an international database
Arthritis and Rheumatism, Volume 54, No. 12, Year 2006
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Description
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. © 2006, American College of Rheumatology.
Authors & Co-Authors
Martini, Giorgia
Italy, Padua
Università Degli Studi Di Padova
Foeldvári, Ivan
Unknown Affiliation
Russo, Ricardo Alberto Guillermo
Argentina, Buenos Aires
Fundacion Hospital de Pediatria Professor Dr. Juan P. Garrahan
Cuttica, Rubén J.
Argentina, Buenos Aires
Hospital General de Niños Pedro de Elizalde
Eberhard, Anne
United States, New Hyde Park
Schneider Children's Hospital
Ravelli, Angelo
Serbia, Genoa
Istituto Di Ricovere e Cura a Carattere Scientifico Irccs G. Gaslini
Lehman, Thomas J.A.
United States, New York
Weill Cornell Medicine
de Oliveira, Sheila Knupp Feitosa
Serbia, Rio de Janeiro
Instituto de Puericultura e Pediatria Martagao Gesteira
Sušić, Gordana Z.
Serbia, Belgrade
Institute of Rheumatology
Lyskina, Galina
Serbia, Moscow
Hospital of Childhood Diseases
Nemcova, Dana
Czech Republic, Prague
Všeobecná Fakultní Nemocnice V Praze
Sundel, Robert
United States, Boston
Boston Children's Hospital
Falcini, Fernanda
Italy, Florence
Azienda Ospedaliero-universitaria Meyer
Girschick, Hermann Josef
Germany, Wurzburg
Universitätsklinikum Würzburg
Lotito, Ana Paula
Brazil, Sao Paulo
Universidade de São Paulo
Buoncompagni, Antonella
Serbia, Genoa
Istituto Di Ricovere e Cura a Carattere Scientifico Irccs G. Gaslini
Sztajnbok, Flávio Roberto
Brazil, Rio de Janeiro
Hospital Universitario Pedro Ernesto
Al-Mayouf, Sulaiman Mohammed
Saudi Arabia, Riyadh
King Faisal Specialist Hospital and Research Centre
Orbàn, Ilonka
Hungary, Budapest
National Institute of Rheumatology and Physiotherapy Budapest
Ferri, Clodoveo
Italy, Modena
Università Degli Studi Di Modena e Reggio Emilia
Athreya, Balu H.
United States, Wilmington
Alfred I. Dupont Hospital for Children
Woo, Patricia M.
United Kingdom, London
Great Ormond Street Hospital for Children Nhs Foundation Trust
Zulian, Francesco
Italy, Padua
Università Degli Studi Di Padova
Statistics
Citations: 185
Authors: 23
Affiliations: 20
Identifiers
Doi:
10.1002/art.22207
ISSN:
00043591
Research Areas
Maternal And Child Health
Noncommunicable Diseases