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

Abstract

The idiopathic inflammatory myopathies are systemic autoimmune diseases characterized by chronic muscle inflammation resulting progressive weakness and frequent involvement of internal organs, mainly the pulmonary, gastrointestinal and cardiac systems. To present clinical characteristics, disease course, frequency of relapses and survival of 79 patients with juvenile or adult dermatomyositis. A national registry of patients with juvenile dermatomyositis was elaborated by the authors in Hungary. The authors summarize data of the register such as signs and symptoms, disease course, frequency of relapses and survival of patients with juvenile dermatomyositis. Analysis was performed using data of 44 patients diagnosed between 1976 and 2004 according to Bohan and Peter's criteria. Survival probability was calculated by Kaplan-Meier method. Data of patients with juvenile dermatomyositis were compared with data of 35 patients with adult dermatomyositis. In view of the disease course, the authors found that more than the half of patients have monophasic disease, while one third of them suffered from polycyclic disease. The risk of the relapse was found to be higher during the first year after the remission. None of the juvenile patients died. Among adult patients, 4 disease-specific deaths occurred. There was no correlation between relapse free survival and initial therapeutic regimen. Many of the patients had polycyclic or chronic disease. As relapses can occur after a prolonged disease-free interval, patients should be followed up for at least 2 years. Despite favourable survival probability, further investigations are needed to assess functional outcome.