Methotrexate in Linear Scleroderma: Long-Term Efficacy in Fifty Children From a Single Pediatric Rheumatology Center.


Journal

Arthritis care & research
ISSN: 2151-4658
Titre abrégé: Arthritis Care Res (Hoboken)
Pays: United States
ID NLM: 101518086

Informations de publication

Date de publication:
09 2021
Historique:
received: 08 10 2019
accepted: 12 05 2020
pubmed: 21 5 2020
medline: 6 10 2021
entrez: 21 5 2020
Statut: ppublish

Résumé

To study disease course and long-term outcome in children with linear scleroderma (SSc) treated with methotrexate (MTX) since diagnosis. The present study was retrospective and cross-sectional and included consecutive children with linear SSc who were treated with MTX for >1 year and were followed up for at least 2 years. Disease course was analyzed by the number of relapses and treatment changes. Relapse-free survival was examined by Kaplan-Meier analysis, comparing patients with linear SSc and those with other juvenile localized scleroderma (JLS) disease subtypes. Disease activity and damage were assessed by the Localized Scleroderma Cutaneous Assessment Tool and thermography. Fifty patients with a mean follow-up duration of 7.8 years and a mean MTX treatment duration of 3.1 years were included. Sixteen percent of patients did not respond to the first course of MTX, and 16% had at least 1 flare. Complete remission was observed in 18.2% of patients who were followed up for 2-5 years, in 80.0% of patients followed up for 10 years, and in 87.5% of patients followed up for >10 years. No significant difference in relapse-free survival between patients with linear SSc and in 17 patients with other JLS disease subtypes was observed. Tissue damage was mild in 42% of patients, moderate in 32%, and severe in 26%. The correlations between severity of tissue damage and linear SSc subtype, disease duration, relapses, and remission were not significant. The relationships between treatment duration and disease relapses (P < 0.05) and severity of tissue damage (P < 0.005) were significant. Most patients with linear SSc who are treated with MTX achieve complete and long-lasting remission. Overall aesthetic and functional sequelae are moderate, most likely because tissue damage is established early and treatment likely stabilizes the damage. Early diagnosis and MTX treatment, as well as long-term monitoring, are crucial to improve outcome and promptly identify flares.

Identifiants

pubmed: 32433808
doi: 10.1002/acr.24260
doi:

Substances chimiques

Immunosuppressive Agents 0
Methotrexate YL5FZ2Y5U1

Types de publication

Journal Article

Langues

eng

Sous-ensembles de citation

IM

Pagination

1259-1263

Informations de copyright

© 2020, American College of Rheumatology.

Références

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Auteurs

Gloria Fadanelli (G)

Santa Chiara Hospital, Trento, Italy.

Anna Agazzi (A)

University of Padova, Padova, Italy.

Fabio Vittadello (F)

University of Padova, Padova, Italy.

Alessandra Meneghel (A)

University of Padova, Padova, Italy.

Francesco Zulian (F)

University of Padova, Padova, Italy.

Giorgia Martini (G)

University of Padova, Padova, Italy.

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