[Juvenile dermatomyositis]

Basic aim of this paper is presentation of probable epidemiological characteristics of juvenile dermatomyositis (JDM) in children of Croatia, due to the fact that severity of condition in majority of patients require treatment in our Department of Pediatrics, KBC Zagreb. Our intention is to present guidelines of current diagnostic approach from recent literature, with special accent on therapy. JDM was previously considered as infaust disease, while current therapy has favourable prognosis for complete resolution, as the only one mesenchymopathy with such prognosis. In period between 1988 and 1999 we treated 18 patients with JDM in our Department of Pediatrics. Thirteen cases were male and 5 female (2.6:1). Thirteen patients had clinical course of isolated JDM (72%), while five had JDM inside overlap syndrome (with other mesenchymopathies) (28%). Average age at time of diagnosis for whole group was 10 years (6-14), for girls 10.6 years (6-14) and boys 8.4 years (7-10). At the present time 5 patients (28%) are considered as cured, 7 patients are in remission with low dosis of steroids (39%) and one girl had relaps of JDM after therapy stopage. Three children die (16%), one girl due to respiratory failure and two girls with pulmonary embolia. Two girls have some signs of JDM with dominant clinical signs of sistemic sclerodermia and one girl has signs of generalized morphea. One boy has dominant muscular calcinosis with contractures of large joints, despite treatment and normal laboratory findings. JDM was not discussed in rheumatic literature in Croatia for more then 15 years.