Juvenile localized scleroderma: clinical and epidemiological features in 750 children. An international study

Juvenile localized scleroderma: clinical and epidemiological features in 750 children. An international study

Autor Zulian, F. Google Scholar
Athreya, B. H. Google Scholar
Laxer, R. Google Scholar
Nelson, A. M. Google Scholar
Oliveira, SKF de Google Scholar
Punaro, M. G. Google Scholar
Cuttica, R. Google Scholar
Higgins, G. C. Google Scholar
Van Suijlekom-Smit, LWA Google Scholar
Moore, T. L. Google Scholar
Lindsley, C. Google Scholar
Garcia-Consuegra, J. Google Scholar
Hilário, Maria Odete Esteves Autor UNIFESP Google Scholar
Lepore, L. Google Scholar
Silva, C. A. Google Scholar
Machado, C. Google Scholar
Garay, S. M. Google Scholar
Uziel, Y. Google Scholar
Martini, G. Google Scholar
Foeldvari, I Google Scholar
Peserico, A. Google Scholar
Woo, P. Google Scholar
Harper, J. Google Scholar
PRES Google Scholar
Instituição Univ Padua
AI Du Pont Hosp Children
Hosp Sick Children
Mayo Clin
Inst Puericultura & Pediat Martagao Gesteira
Univ Texas
Hosp Gen Ninos Pedro de Elizalde
Childrens Hosp
Sophia Childrens Univ Hosp
Cardinal Glennon Childrens Hosp
Univ Kansas
Hosp Univ La Paz
Universidade Federal de São Paulo (UNIFESP)
IRCCS Burlo Garofalo
Universidade de São Paulo (USP)
Ak Eilbek
Meir Med Ctr
Hosp Sor Maria Ludovica
Fac Med Botucatu
Great Ormond St Hosp Children
Dermatol Clin
Resumo Objective. Juvenile localized scleroderma (JLS) includes a number of conditions often grouped together. With the long-term goal of developing uniform classification criteria, we studied the epidemiological, clinical and immunological features of children with JLS followed by paediatric rheumatology and dermatology centres.Methods. A large, multicentre, multinational study was conducted by collecting information on the demographics, family history, triggering environmental factors, clinical and laboratory features, and treatment of patients with JLS.Results. Seven hundred and fifty patients with JLS from 70 centres were enrolled into the study. the disease duration at diagnosis was 18 months. Linear scleroderma (LS) was the most frequent subtype (65%), followed by plaque morphea (PM) (26%), generalized morphea (GM) (7%) and deep morphea (DM) (2%). As many as 15% of patients had a mixed subtype. Ninety-one patients (12%) had a positive family history for rheumatic or autoimmune diseases; 100 (13.3%) reported environmental events as possible trigger. ANA was positive in 42.3% of the patients, with a higher prevalence in the LS-DM subtype than in the PM-GM subtype. Scl70 was detected in the sera of 3% of the patients, anticentromere antibody in 2%, anti-double-stranded DNA in 4%, anti-cardiolipin antibody in 13% and rheumatoid factor in 16%. Methotrexate was the drug most frequently used, especially during the last 5 yr.Conclusion. This study represents the largest collection of patients with JLS ever reported. the insidious onset of the disease, the delay in diagnosis, the recognition of mixed subtype and the better definition of the other subtypes should influence our efforts in educating trainees and practitioners and help in developing a comprehensive classification system for this syndrome.
Assunto scleroderma
scleroderma en coup de sabre
progressive hemifacial atrophy
Parry-Romberg syndrome
Idioma Inglês
Data 2006-05-01
Publicado em Rheumatology. Oxford: Oxford Univ Press, v. 45, n. 5, p. 614-620, 2006.
ISSN 1462-0324 (Sherpa/Romeo, fator de impacto)
Editor Oxford Univ Press
Extensão 614-620
Fonte http://dx.doi.org/10.1093/rheumatology/kei251
Direito de acesso Acesso aberto Open Access
Tipo Artigo
Web of Science WOS:000236998500023
URI http://repositorio.unifesp.br/handle/11600/28859

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