The impact of coexisting connective tissue disease on survival in patients with fibrosing alveolitis

Open Access
Publisher Website
Google Scholar
Abstract
Objectives. Previous reports have suggested that patients who have fibrosing alveolitis in association with a connective tissue disease (FA‐CTD) have a better prognosis than patients with ‘lone’ cryptogenic fibrosing alveolitis (LCFA). The present study was designed to compare the survival of a general population‐based sample of patients with FA‐CTD and LCFA both with each other and with the general population. Methods. A survival analysis was performed using data for 107 patients with FA‐CTD, 872 with LCFA and 5958 controls matched for age, sex and general practice, drawn from the General Practice Research Database. The data were analysed using Cox regression, adjusting for a number of potential confounders, including age, gender, smoking habit and use of oral corticosteroids. Results. The median follow‐up period was 2.1 yr and during this time 54 (50%) patients with FA‐CFA, 386 (44%) patients with LCFA and 601 (10%) controls died. The mortality rates for patients with FA‐CTD, LCFA and the controls were 284, 270 and 41 per 1000 person‐yr respectively. After adjusting for age, gender, smoking habit and exposure to oral corticosteroids, patients with FA‐CTD had a marginally worse survival than patients with LCFA (hazard ratio 1.20, 95% confidence interval 0.90–1.61). Compared with the general population controls, patients with either LCFA or FA‐CTD had a considerably worse prognosis (hazard ratio 5.56, 95% confidence interval 4.77–6.49). Conclusions. The median survival in patients with fibrosing alveolitis is less then 3 yr. We found no evidence to support previous reports of a better prognosis amongst patients with FA‐CTD.