Desquamative fibrosing alveolitis unresponsive to steroid or cytotoxic therapy

Abstract

A case of desquamative fibrosing alveolitis beginning in early infancy is described. The disease was characterized by tachypnoea, hypoxia relieved by O2, absence of signs in the chest or clubbing, and radiological and physiological evidence of hyperinflation. The diagnosis was made by needle biopsy of the lung. Treatment with steroids and immunosuppression was without effect and the infant died at 9 months. The disease has a high mortality in infancy, only 2 out of 9 reported cases having survived. The difference from the usual course in adults and older children is noted.