The impact of coexisting connective tissue disease on survival in patients with fibrosing alveolitis.
OBJECTIVE
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.
Hubbard
R
R
Division of Respiratory Medicine, Nottingham University, Nottingham, UK.
Venn
A
A
D001172:Arthritis, Rheumatoid / Q000401:mortality; D003240:Connective Tissue Diseases / Q000401:mortality*; D003882:Dermatomyositis / Q000401:mortality; D006801:Humans; D008180:Lupus Erythematosus, Systemic / Q000401:mortality; D008947:Mixed Connective Tissue Disease / Q000401:mortality; D015999:Multivariate Analysis; D011658:Pulmonary Fibrosis / Q000401:mortality*; D012595:Scleroderma, Systemic / Q000401:mortality; D016019:Survival Analysis