Fatal interstitial pneumonia in juvenile dermatomyositis

A 5-year-old girl presented with a two-month-history of skin rash and general fatigue. She had a slight fever, progressive muscle weakness and liver dysfunction. Gottron's papules on her fingers and purple-reddish papules on her elbows were noted. Serum aldolase levels were highly elevated, however, creatine phosphokinase levels were normal. An MRI revealed abnormal high signal changes in her gluteus minimus muscles. Interstitial pneumonia suddenly developed and she died despite aggressive treatment with methylprednisolone pulse therapy followed by intravenous administration of cyclophosphamide, immunoglobulin and cyclosporine A. Interstitial pneumonia is rare in juvenile dermatomyositis; however, as in adult cases, it can be fatal. In order to prevent severe complications and functional disabilities, early aggressive treatments should be considered when muscle inflammation is refractory to ordinary treatment.