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Disease course, frequency of relapses and survival of 73 patients with juvenile or adult dermatomyositis

A. Ponyi^1,2, T. Constantin¹, Z. Balogh³, Z. Szalai⁴, G. Borgulya¹, K. Molnár⁴, I. Tefner³, M. Garami¹, G. Fekete², K. Dankó²

¹Second Department of Pediatrics, Faculty of Medicine, Semmelweis University, Budapest; ²Third Department of Internal Medicine, Division of Clinical Immunology, Medical and Health Science Center, University of Debrecen, Debrecen; ³National Institute of Rheumatology and Physiotherapy, Department of General and Pediatric Rheumatology, Budapest; ⁴Heim Pál Children\rquote s Hospital, Department of Dermatology, Budapest, Hungary.

ABSTRACT
Objective
Our aim is to present the disease course, frequency of relapses and survival of juvenile and adult dermatomyositis (JDM/DM) patients.

Methods
Analysis was performed using data on 73 patients. The median follow-up for 38 JDM patients was 32 months and 78 months for 35 adult DM patients.

Results
23/38 JDM patients (60%) had monophasic, 12/38 (31.6%) had polycyclic and 3/38 (7.9%) had chronic disease. Among children treated only with glucocorticoids, 12/20 (60%) had monophasic and 8/20 (40%) had polycyclic disease. 10/17 (58.8%) children, who required second-line immunosuppressive agents, had monophasic and 4/17 (23.5%) had polycyclic disease. 18/35 DM (51.4%) patients had monophasic, 13/35 (37.1%) had polycyclic, 1/35 (2.9%) had chronic disease and 3/35 (8.6%) had fulminant myositis. Among DM patients requiring only glucocorticoids, 12/20 (60%) were monophasic and 8/20 (40%) were polycyclic. In patients requiring second-line immunosuppressive agents, 6/15 patients (40%) had monophasic and 5/15 (33.3%) had polycyclic disease. Among patients with polycyclic disease, the risk of relapse was higher during first year than later in the disease course. None of the JDM patients have died, while 4 disease-specific deaths occurred in adult patients. There was no significant difference between the survival of JDM and DM patients.

Discussion
There was no correlation between relapse-free survival and the initial therapeutic regimen. Many of our patients had polycyclic or chronic disease. As relapses can occur after a prolonged disease-free interval, patients should be followed for at least 2 years. Although we found a favourable survival rate, further investigations are needed to assess functional outcome.

Key words
Juvenile and adult dermatomyositis, disease course, relapse risk, survival analysis.

Andrea Ponyi and Tamás Constantin participated equally in this work, therefore they are both considered as first authors of this article. The study was coordinated by György Fekete and Katalin Dankó.
Please address correspondence to: Andrea Ponyi, MD, 2^nd Department of Pediatrics, Semmelweis University, Tüzoltó u. 7-9, H-1094 Budapest, Hungary.
E-mail: ponyi@gyer2.sote.hu