Changes in Phosphatidylglycerol in Bronchoalveolar Lavage Fluids from Patients with Cryptogenic Fibrosing Alveolitis

In view of recent evidence that changes in phospholipid components of pulmonary surfactant occur in bleomycin-induced pulmonary fibrosis, the aim of this study was to examine whether similar changes occur in patients with idiopathic pulmonary fibrosis. We have analyzed phospholipid profiles in bronchoalveolar lavage (BAL) fluids from a group of 32 patients with “lone” cryptogenic fibrosing alveolitis (CFA) compared with 17 control subjects. Prior to treatment, resembling the findings in bleomycin fibrosis, many CFA patients had decreased proportions of phosphatidylglycerol (PC) (percent of total phospholipid; median 10.5, range 0 to 35.1 compared with 18.2, range 9.6 to 24.2 for the control group of similar age range and smoking habits; p<0.01). Following the initial lavage, the patients were treated with prednisolone. The initial percentage of PG levels did not predict response to prednisolone, but serial lavage studies conducted in 14 patients suggest that an early and sustained increase in percentage of PG following commencement of prednisolone is associated with clinical improvement. The reasons for the proportional reductions in phosphatidylglycerol, and whether they result in functional deficiency of surfactant in CFA, need to be explored. (Chest 1989; 95:82-89)