Cryptogenic fibrosing alveolitis: clinical features and their influence on survival

Abstract
A retrospective analysis of 220 cases fulfilling criteria for cryptogenic fibrosing alveolitis (CFA) attending the Brompton Hospital between 1955 and 1973 has been carried out and patients have been followed for between four and 21 years. The frequency of various clinical features confirms previous reports. The 2: 1 male preponderance was similar in all age groups. The mean age at presentation was 54 years ± 12 SD; 202 (92%) of the patients presented with dyspnoea, the severity of which was related to the reduction in vital capacity (p2 (p1, FVC, and TLC, the presence of “connective tissue” disorders, autoantibodies, smoking history, cough, sputum, crackles, clubbing, ESR, or immunoglobulins.