Therapeutic approaches in the treatment of juvenile dermatomyositis in patients with recent-onset disease and in those experiencing disease flare: An international multicenter PRINTO study

Authors

Rachana Hasija, Università degli Studi di Genova
Angela Pistorio, Università degli Studi di Genova
Angelo Ravelli, Università degli Studi di Genova
Erkan Demirkaya, Università degli Studi di GenovaFollow
Raju Khubchandani, Jaslok Hospital and Research Centre
Dinara Guseinova, Università degli Studi di Genova
Clara Malattia, Università degli Studi di Genova
Helena Canhao, Universidade de Lisboa
Liora Harel, Schneider Children's Hospital
Dirk Foell, Westfälische Wilhelms-Universität Münster
Carine Wouters, KU Leuven– University Hospital Leuven
Carmen De Cunto, Instituto Universitario del Hospital Italiano de Buenos Aires
Christian Huemer, Landeskrankenhaus Bregenz
Yukiko Kimura, The Joseph M. Sanzari Children's Hospital
Harald Mangge, Medizinische Universität Graz
Carlo Minetti, Università degli Studi di Genova
Ellen Berit Nordal, University Hospital of North Norway
Pierre Philippet, Centre Hospitalier Chretien
Rosaria Garozzo, Università degli Studi di Catania
Alberto Martini, Università degli Studi di Genova
Nicolino Ruperto, Università degli Studi di Genova

Document Type

Article

Publication Date

10-1-2011

Journal

Arthritis and Rheumatism

Volume

63

Issue

10

First Page

3142

Last Page

3152

URL with Digital Object Identifier

10.1002/art.30475

Abstract

Objective To evaluate response to therapy over a 24-month period in a large prospective international cohort of patients with juvenile dermatomyositis (DM). Methods The study included 145 patients with recent-onset juvenile DM and 130 juvenile DM patients experiencing disease flare, all of whom were <18 years old. Disease activity parameters and therapeutic approaches in 4 geographic areas were analyzed at baseline and at 6, 12, and 24 months. Response was assessed according to the Pediatric Rheumatology International Trials Organization (PRINTO) juvenile DM response criteria, and data were reported "as observed" and in the intent-to-treat (ITT) population. Results Patients with recent-onset juvenile DM at baseline had higher baseline disease activity and greater improvement over 24 months when compared to juvenile DM patients experiencing disease flare at baseline. Methotrexate (MTX) or high-dose corticosteroids were administered more frequently to patients with recent-onset juvenile DM, compared to juvenile DM patients experiencing disease flare, who were more likely to receive cyclosporine. Compared to patients from Western and Eastern Europe, a higher proportion of patients from South and Central America and North America received pulse steroids, and the average steroid dosage was higher in the North American and South and Central American patients. The use of MTX was similar in all 4 regions, while cyclosporin A was more frequently used in Western Europe. In the "as observed" analysis, 57.9% of the patients with recent-onset juvenile DM and 36.4% of the patients experiencing disease flare (P < 0.001) reached at least a 70% response by PRINTO criteria at 6 months; these proportions had increased at month 24 to 78.4% and 51.2%, respectively (P < 0.001). Corresponding results of the ITT analysis were much lower, with only one-third of the patients able to maintain the initial assigned therapy over 24 months. Conclusion Patients with recent-onset juvenile DM are more likely to achieve significant clinical improvement over 24 months, when compared to patients experiencing flares of juvenile DM. Internationally, various therapeutic approaches are used to treat this disease. Copyright © 2011 by the American College of Rheumatology.