Interstitial lung disease clinics for the management of idiopathic pulmonary fibrosis: a potential advantage to patients. Greater Manchester Lung Fibrosis Consortium.

摘要

BACKGROUND: Idiopathic pulmonary fibrosis (IPF) has a relatively poor prognosis with limited therapeutic intervention. This has led to varying practices, from a nihilistic approach at one end to more aggressive management at the other. However, a dedicated clinic with a multidisciplinary approach may offer advantages to patients with IPF. METHODS: A retrospective observational study was performed to compare patients with a diagnosis of IPF attending a general respiratory clinic between January 1988 and December 1996 to those attending a interstitial lung disease (ILD) clinic between January 1992 and December 1996. The notes were reviewed for (1) confirmation of diagnosis, (2) method of diagnosis, (3) date of initial consultation, and (4) survival. The end point was death, and the number of deaths that occurred up to October 1997 was recorded. RESULTS: The study identified 148 patients with a diagnosis of IPF. Ten patients underwent transplantation and were excluded. Of the remaining 138, 84 patients attended the general respiratory clinic (mean age 65 years: 53 men [63%], 31 women), and 54 attended the ILD clinic (mean age 56 years: 26 men [48%], 28 women). Patients attending the ILD clinic had a median survival of >3714 days. Patients attending the general respiratory clinic had a median survival of 1796 +/- standard error 437 days (CI 940-2652), p = .032 Breslow. Age was an important determinant of outcome. For patients less than 60 years of age (n = 60), the median survival for the ILD clinic was >3700 days, compared to 2535 +/- 577 days (CI 1404-280) in the general respiratory clinic, p = .037 Breslow. There was no difference in survival of patients over 60 years of age. CONCLUSIONS: The study suggests that a dedicated multidisciplinary clinic may result in an improved outcome for patients with IPF, particularly for patients younger than 60 years. This has implications that may facilitate the development of suitably powered therapeutic trials and may affect patient referral for transplantation.