Interstitial lung disease associated with juvenile dermatomyositis: clinical features and efficacy of cyclosporin A

OBJECTIVES: Interstitial lung disease (ILD) is a rare complication of juvenile dermatomyositis (JDM). The aim of this study was to clarify the clinical features of JDM-associated ILD and to evaluate the efficacy of cyclosporin A (CSA). METHODS: We reviewed clinical records of 10 cases of JDM that were admitted to Hokkaido University Hospital between April 1990 and March 2001. RESULTS: Five cases were complicated with ILD, three with interstitial pneumonia and two with bronchiolitis obliterans organizing pneumonia. ILD was associated with active JDM and progressed despite corticosteroid therapy. Testing for anti-Jo-1 antibody was negative in all cases. Respiratory symptoms were initially noticed in only one case. In the other cases, ILD was first detected by routine examination of chest X-ray. All the cases received CSA (3-5 mg/kg/day) in combination with prednisolone. One patient died of respiratory failure, but the others responded well to treatment with CSA. CONCLUSION: ILD should be evaluated carefully in all cases of JDM regardless of respiratory symptoms. CSA is a choice for steroid-resistant cases of JDM-associated ILD.