British Thoracic Society Study on cryptogenic fibrosing alveolitis: response to treatment and survival

• Published in: Thorax Vol. 62; no. 1; pp. 62 - 66
• Main Authors: Rudd, Robin M, Prescott, Robin J, Chalmers, J C, Johnston, Ian D A
• Format: Journal Article
• Language: English
• Published: London BMJ Publishing Group Ltd and British Thoracic Society 01.01.2007; BMJ; BMJ Publishing Group LTD; BMJ Group
• Subjects: Analysis of Variance; ANOVA; Anti-Inflammatory Agents - therapeutic use; Biological and medical sciences; Carbon monoxide; Cause of Death; CFA; cryptogenic fibrosing alveolitis; Female; forced vital capacity; FVC; Health risk assessment; high-resolution computed tomography; HRCT; Humans; idiopathic interstitial pneumonia; idiopathic pulmonary fibrosis; IIP; Immunosuppressive Agents - therapeutic use; Interstitial Lung Disease; IPF; Lung diseases; Male; Medical prognosis; Medical sciences; Pneumology; Prednisolone - therapeutic use; Pulmonary fibrosis; Pulmonary Fibrosis - drug therapy; Pulmonary Fibrosis - mortality; Pulmonary Fibrosis - physiopathology; reduced carbon monoxide growth factor; Society; Studies; Substance abuse treatment; Survival Analysis; TLCO; Treatment Outcome; UIP; usual interstitial pneumonia; Variance analysis; Vital Capacity - physiology; United Kingdom; Great Britain; Europe; United Kingdom > UK; Response; Prognosis; Treatment; Respiratory disease; Fibrosis; Idiopathic; Cardiovascular disease; Thorax; Society; Survival
• ISSN: 0040-6376; 1468-3296
• DOI: 10.1136/thx.2005.045591

Background and 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 reduced carbon monoxide transfer factor 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.