Pulmonary alveolar lipoproteinosis complicating juvenile dermatomyositis

Abstract

A 9 year old boy with juvenile dermatomyositis developed pulmonary alveolar lipoproteinosis. This previously unreported association was treated successfully by bronchoalveolar lavage. Pulmonary alveolar lipoproteinosis is a rare condition of unknown aetiology characterised by accumulation of a lipoproteinaceous material within alveolar spaces.' The diagnosis is confirmed by finding periodic acid-Schiff (PAS) staining material on light microscopy and characteristic lamellar bodies on electron microscopy. Although it has previously been reported to be associated with several immunological abnormalities in children and with haematological malignancies and respiratory infections in adults, an association with autoimmune disease has not been noted. We report a case ofthe disease associated withjuvenile dermatomyositis.