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

Rudd, Robin M.; Prescott, Robin J.; Chalmers, J. C.; Johnston, Ian D. A.
January 2007
Thorax;Jan2007, Vol. 62 Issue 1, p62
Academic Journal
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 = 0.00 1 analysis of variance (ANOVA)) and had lower initial FVC (p


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