Chest
Clinical Investigations: BAC, UIP, SODSurvival in Patients With Cryptogenic Fibrosing Alveolitis: A Population-Based Cohort Study
Section snippets
Materials and Methods
The patients were identified as part of a case-control study of occupational causes of cryptogenic fibrosing alveolitis. Details of patient identification, data collection, and diagnostic criteria have been published elsewhere.6 We attempted to identify all patients with cryptogenic fibrosing alveolitis attending nine centers within the Trent Region of England at the outset of the study (the prevalent cases), and all new patients seen during an 18-month recruitment period (the incident cases),
Results
A total of 168 (105 male) prevalent cases and 76 (56 male) incident cases were identified. Open lung biopsy was performed in 31 (12.7%) cases, and the mean age of these cases was significantly lower than those who did not have this procedure (52.4 years vs 69.0 years; p<0.001). The proportion of prevalent cases who underwent open lung biopsy was higher than that for incident cases, but the opposite was true for HRCT (Table 2). Incident cases tended to be older, and were more likely to be male,
Discussion
In this study, we have shown that the median survival of newly diagnosed cases of cryptogenic fibrosing alveolitis is 2.9 years, and as such is not only substantially lower than that of prevalent cases, but also considerably worse than currently recognized.3, 4, 5
To establish our population of patients, we identified patients attending a range of secondary and tertiary referral centers within the Trent region of England, and thus believe that these cases are typical of patients presenting to
ACKNOWLEDGMENTS
The authors thank Dr. Sarah Firby (actuary) for statistical advice, the lung function technicians at the nine centers involved in this study for assisting with patient identification, and the consultant physicians who allowed us to approach their patients. We would also like to thank Nottinghamshire, Derbyshire, Leicestershire, Lincolnshire, and South Yorkshire Family Health Services Authorities for help with identification of control subjects, the General Practitioners, and the Medical
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Supported by the Medical Research Council.
revision accepted July 14, 1997.