Rheumatology Advance Access published online on December 20, 2005
Rheumatology, doi:10.1093/rheumatology/kei251
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1 Department of Pediatrics, Padova, Italy
* To whom correspondence should be addressed. 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.
Received July 9, 2005
Accepted November 11, 2005
Original Papers
Juvenile localized scleroderma: clinical and epidemiological features in 750 children. An international study
F. Zulian 1 *,
B. H. Athreya 2,
R. Laxer 3,
A. M. Nelson 4,
S. K. Feitosa de Oliveira 5,
M. G. Punaro 6,
R. Cuttica 7,
G. C. Higgins 8,
L. W. A. Van Suijlekom-Smit 9,
T. L. Moore 10,
C. Lindsley 11,
J. Garcia-Consuegra 12,
M. O. Esteves Hilário 13,
L. Lepore 14,
C. A Silva 15,
C. Machado 16,
S. M. Garay 17,
Y. Uziel 18,
G. Martini 1,
I. Foeldvari 19,
A. Peserico 20,
P. Woo 21,
J. Harper 21,
and
for the Juvenile Scleroderma Working Group of the Pediatric Rheumatology European Society (PRES)
2 AI Du Pont Hospital for Children, Wilmington, DE, USA
3 Hospital for Sick Children, Toronto, Canada
4 Mayo Clinic, Rochester, MN, USA
5 Instituto de Puericultura e Pediatria Martagao Gesteira, Rio de Janeiro, Brazil
6 Department of Pediatrics, Dallas, TX, USA
7 Hospital General de Niños Pedro de Elizalde, Buenos Aires, Argentina
8 Children's Hospital, Columbus, OH, USA
9 Erasmus MC, Sophia Children's Hospital, Rotterdam, The Netherlands
10 Cardinal Glennon Children's Hospital, St Louis, MO, USA
11 University of Kansas City Medical Center, Kansas City, KS, USA
12 Hospital Universitario ‘La Paz’, Madrid, Spain
13 Universidade Federal de São Paulo, São Paulo, Brazil
14 IRCCS Burlo Garofalo, Trieste, Italy
15 Instituto da Criança, University of São Paulo, Pompeia São Paulo, Brazil
16 Faculdade de Medicina de Botucatu, São Paulo, São Paulo, Brazil
17 Hospital Sor Maria Ludovica, Buenos Aires, Argentina
18 Meir Medical Center, Kfar Saba, Israel
19 Ak Eilbek, Hamburg, Germany
20 Dermatology Clinic, Padova, Italy
21 Great Ormond Street Hospital, London, UK
F. Zulian, E-mail: zulian{at}pediatria.unipd.it
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