Rheumatology Advance Access originally published online on December 20, 2005
Rheumatology 2006 45(5):614-620; doi:10.1093/rheumatology/kei251
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Juvenile localized scleroderma: clinical and epidemiological features in 750 children. An international study
Department of Pediatrics, Padova, Italy, 1 AI Du Pont Hospital for Children, Wilmington, DE, USA, 2 Hospital for Sick Children, Toronto, Canada, 3 Mayo Clinic, Rochester, MN, USA, 4 Instituto de Puericultura e Pediatria Martagao Gesteira, Rio de Janeiro, Brazil, 5 Department of Pediatrics, Dallas, TX, USA, 6 Hospital General de Niños Pedro de Elizalde, Buenos Aires, Argentina, 7 Children's Hospital, Columbus, OH, USA, 8 Erasmus MC, Sophia Children's Hospital, Rotterdam, The Netherlands, 9 Cardinal Glennon Children's Hospital, St Louis, MO, USA, 10 University of Kansas City Medical Center, Kansas City, KS, USA, 11 Hospital Universitario ‘La Paz’, Madrid, Spain, 12 Universidade Federal de São Paulo, São Paulo, Brazil, 13 IRCCS Burlo Garofalo, Trieste, Italy, 14 Instituto da Criança, University of São Paulo, Pompeia São Paulo, Brazil, 15 Faculdade de Medicina de Botucatu, São Paulo, São Paulo, Brazil, 16 Hospital Sor Maria Ludovica, Buenos Aires, Argentina, 17 Meir Medical Center, Kfar Saba, Israel, 18 Ak Eilbek, Hamburg, Germany, 19 Dermatology Clinic, Padova, Italy and 20 Great Ormond Street Hospital, London, UK.
Correspondence to: F. Zulian, Dipartimento di Pediatria, Università di Padova, Via Giustiniani 3, 35128 Padova, Italy. E-mail: zulian{at}pediatria.unipd.it
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.
KEY WORDS: Scleroderma, Morphea, Scleroderma en coup de sabre, Progressive hemifacial atrophy, Parry–Romberg syndrome
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