Objective: To determine the safety and efficacy of high dose alternate day (qod) prednisone as therapy in acute systemic onset JRA (SOJRA).
Methods: A retrospective chart review was performed of all active patients with SOJRA at our institutions who began high dose qod prednisone (n = 20; 9 male, 11 female; mean age of onset 6.5 yrs, range 1.2-18.6). Patients were followed for at least one year after initiation of high dose qod prednisone. Disease activity (fever, rash, active joint count, complete blood cell count, erythrocyte sedimentation rate, ESR) and possible side effects of treatment were assessed at each visit.
Results: Within a mean of 2.1 months (range 1-5), systemic features (fever, rash, serositis, coagulopathy) in all patients had resolved and there was significant improvement in laboratory indices of disease activity. Doses ranged from 1 to 5.8 mg/kg qod (actual doses: 50-400 mg qod), with a mean of 3.2 mg/kg. No patient had to restart daily prednisone. The only major side effect was the development of mild cataracts in one patient. Height standard deviation scores (SDS) remained within normal range in all but 2 patients. Clinical improvement was maintained in all patients, as measured by lack of systemic symptoms, and decreased active joint count. Repeated measures of analysis of variance revealed significant improvement in all laboratory tests (white blood cell count, hemoglobin, platelet count, ESR) measured at 0, 6, and 12 months (p < 0.001). Nine of the 20 patients continued qod prednisone more than 12 months beyond the study period (mean 5.2 yrs, range 3-8). The only additional side effects were a vertebral crush fracture in one patient and possible avascular necrosis in another. Height SDS did not change significantly over the 3 year period after the initiation of qod prednisone (p > 0.05).
Conclusion: High dose qod prednisone appears to be effective in controlling the systemic features of SOJRA and was well tolerated. Side effects attributable to corticosteroids, including growth suppression, were minimal.