The prognosis of cryptogenic fibrosing alveolitis

Abstract

The course of cryptogenic fibrosing alveolitis (CFA) in 96 patients is described. The mean survival from the onset of the first symptom until death was 47·4 months in all the 59 fatal cases, 49·6 months in those dying from CFA only, and 48·4 months in those dying from CFA in whom the course of the illness was not influenced by corticosteroids. There was a wide variation in the natural course of the disorder, varying from death within a year of the onset of symptoms in five patients to prolonged survival with slow or arrested progress in four patients not treated with corticosteroids. Most patients died from CFA but five developed bronchial carcinoma and two died after lung biopsy.

The main factor influencing the prognosis was the effectiveness of corticosteroid drugs. Only 16% of the treated patients had a worthwhile (grade 2) response to these drugs and the five-year survival of this group was 67%. In those patients in whom the course of the illness was uninfluenced by corticosteroids the five-year survival rate was only 20%. The histological appearances on lung biopsy gave a rough guide to the likely effect of corticosteroid therapy.

Other factors influencing the prognosis were the degree of dyspnoea on presentation, the vital capacity, and the presence of arterial hypoxaemia. Patients who had had symptoms for more than four years before the first hospital attendance had a relatively good prognosis.

By contrast, neither the extent of radiographic abnormality nor the presence of circulating rheumatoid and antinuclear factors influenced subsequent survival.