Course of treated juvenile dermatomyositis

Abstract

Sixty-six patients with possible juvenile dermatomyositis (JDMS) were observed at the Children's Hospital of Los Angeles from 1960 to 1982. In patients initially given high doses of corticosteroids followed by low-dose therapy, three different clinical courses had previously been observed: monocyclic, polycyclic, and chronic continuous. We reviewed the records of 32 patients who met study criteria. The course of JDMS was monocyclic in eight children, chronic polycyclic in 10, and chronic continuous in 14. Of these children, 25 are well and not receiving medication; one has mild JDMS, without corticosteroid therapy; four have active JDMS despite corticosteroid therapy (one is severely handicapped); and two have died. Our results support the improved prognosis of JDMS after corticosteroid therapy, but also the great clinical variability of the disease. Understanding of this variability, as reflected in the three disease courses, facilitates physician choice of the optimal treatment with the least drug toxicity for the individual patient, continuing efforts to clarify the disease pathogenesis, and research efforts to improve current treatment programs for the patient with severe JDMS.