British Thoracic Society Study on cryptogenic fibrosing alveolitis: Response to treatment and survival.
OBJECTIVE
The initial results of a survey of 588 patients with a clinical presentation of cryptogenic fibrosing alveolitis (CFA) also known as idiopathic pulmonary fibrosis, have been published. This article reports further results pertaining to response to treatment and survival.


METHODS
Data on the treatment given and lung function response were collected over 4-6 years. Survival data were collected over 10 years.


RESULTS
Treatment was given to 445 (76%) patients, 55% were given prednisolone alone and the remainder another immunosuppressive agent, usually with prednisolone. Treated patients had worse lung function initially. At 3 months after study entry, treated patients were more likely to have improved forced vital capacity (FVC) than the untreated patients. Patients whose FVC improved were younger (p = 0.001 analysis of variance (ANOVA)) and had lower initial FVC (p<0.001, ANOVA). Patients who responded to treatment at 3 months or at 1 year survived longer than those who remained stable, who in turn survived longer than those who deteriorated (p = 0.002). These differences were largely accounted for by patients with better lung function surviving longer. Younger age at entry, female sex and higher percentage predicted FVC and carbon monoxide transfer factor [corrected] at study entry were associated with greater chances of survival at 4 years. Overall median survival from entry was 2.43 years (95% confidence interval (CI) 2.17 to 3.18).


CONCLUSIONS
About a third of patients with CFA showed improved lung function after initiation of corticosteroid or immunosuppressive treatment, and those who improved survived longer. Poorer lung function, male sex and age are adverse prognostic features. Overall survival was poor.
Rudd
Robin M
RM
London Chest Hospital, Bonner Road, London E2 9JX, UK. dr@robinrudd.com
Prescott
Robin J
RJ
Chalmers
J C
JC
Johnston
Ian D A
ID
D000704:Analysis of Variance; D000893:Anti-Inflammatory Agents / Q000627:therapeutic use*; D002423:Cause of Death; D005260:Female; D006801:Humans; D007166:Immunosuppressive Agents / Q000627:therapeutic use; D008297:Male; D011239:Prednisolone / Q000627:therapeutic use*; D011658:Pulmonary Fibrosis / Q000188:drug therapy* / Q000401:mortality / Q000503:physiopathology; D016019:Survival Analysis; D016896:Treatment Outcome; D014797:Vital Capacity / Q000502:physiology
D000893:Anti-Inflammatory Agents; D007166:Immunosuppressive Agents; D011239:Prednisolone