Management of fibrosing alveolitis with polymyositis dermatomyositis

Abstract
A 38-year-old man with polymyositis-dermatomyositis developed acute life-threatening fibrosing alveolitis. We report a two year follow-up of this patient, during which control of alveolitis was obtained using prednisolone, and was maintained with the addition of cyclophosphamide. Relapse of myositis responded to increased prednisolone. Serial measurements of vital capacity and transfer factor assisted management of alveolitis, but changes in vital capacity preceded changes in transfer factor, and were more convenient to perform during frequent outpatient review.