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Cited by 113Interstitial Lung Diseases and Idiopathic Pulmonary FibrosisOccupational and environmental lung diseasesTransplantation: Methods and Outcomes

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

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Rudd, R. M, Prescott, R. J, Chalmers, J C, Johnston, I. D A, for the Fibrosing Alveolitis Subcommittee of the Research Committee of the British Thoracic Society·2007·Thorax
DOI 10.1136/thx.2005.045591PMC PMC2111279
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Abstract

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 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.

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Authors
Rudd, R. M., Prescott, R. J., Chalmers, J. C., Johnston, I. D. A., & for the Fibrosing Alveolitis Subcommittee of the Research Committee of the British Thoracic Society
Affiliations
  • London Chest Hospital (GB)
  • University of Edinburgh (GB)
  • University of Nottingham (GB)
Corresponding author
Rudd, R. M
ORCID
Venue
Thorax
Publisher
BMJ
Published
2007-01-01
Volume
62
Issue
1
Pages
62-66
ISSN
0040-6376 (ISSN-L)
DOI
10.1136/thx.2005.045591
PMC
PMC2111279
OpenAlex
W2163595789
Cited by
113
References
42
Citations by year
2026: 1 · 2025: 1 · 2024: 6 · 2023: 1 · 2022: 8 · 2021: 3 · 2020: 5 · 2019: 4 · 2018: 9 · 2017: 6 · 2016: 3 · 2015: 8 · 2014: 9 · 2013: 11 · 2012: 7
FWCI
6.50
Type
journal article
Language
en
Open access
Locations
  • Thorax (publishedVersion)
  • PubMed Central (publishedVersion)
  • PubMed (publishedVersion)
  • HighWire Press Open Archive (submittedVersion)
Topics
  • Health Sciences › Medicine › Pulmonary and Respiratory Medicine › Interstitial Lung Diseases and Idiopathic Pulmonary Fibrosis
  • Health Sciences › Medicine › Pulmonary and Respiratory Medicine › Occupational and environmental lung diseases
  • Health Sciences › Medicine › Surgery › Transplantation: Methods and Outcomes
Keywords
idiopathic pulmonary fibrosis, treatment response, survival, prednisolone, immunosuppressive therapy, lung function, forced vital capacity, prognostic factors, carbon monoxide transfer factor, age of onset, sex differences
MeSH terms
Analysis of Variance, Anti-Inflammatory Agents, Cause of Death, Female, Humans, Immunosuppressive Agents, Male, Prednisolone, Pulmonary Fibrosis, Vital Capacity, Survival Analysis, Treatment Outcome

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