Original Articles
Interstitial lung disease clinics for the management of idiopathic pulmonary fibrosis: a potential advantage to patients

https://doi.org/10.1016/S1053-2498(99)00050-9Get rights and content

Abstract

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.

Section snippets

Materials and methods

A general respiratory clinic is defined here as a clinic with a wide case mix of respiratory diseases (e.g., emphysema, asthma) in which patients with IPF are seen by a consultant, resident, or fellow. A clinical nurse specialist is not aligned to the clinic. There is direct access to cardiothoracic surgical opinion, and radiology and pathology opinions are given by specialists with a declared interest in respiratory diseases. The clinic is not aligned to a transplant program.

An ILD clinic is

Results

The study followed 148 patients (86 men, 62 women, mean age 61 years) with a confirmed diagnosis of IPF. Of these, 10 underwent transplantation and were excluded from final analysis, leaving 138 patients (79 men, 59 women, mean age 61 years). Twenty-four were diagnosed on the basis of typical clinical features (prior to HRCT), 54 were diagnosed on the basis of HRCT scanning; and 60 had a surgical lung biopsy, each of which had variegate lung fibrosis consistent with UIP. Eighty-four patients

Discussion

Current medical management has largely failed to demonstrate a significant advantage for patients with IPF. The result of this study indicates that a specific clinic for patients with IPF may offer a significantly improved outcome, particularly in patients younger than 60 years of age. Such a clinic may facilitate the collection of patients for the development of suitably powered randomized studies.

Age is a potential confounding factor when evaluating the results. The patients attending the

Acknowledgements

The Greater Manchester Lung Fibrosis Consortium: Dr. G. Archer, Stepping Hill Hospital, Stockport; Dr. T. Baker, Maelor Hospital, Wrexham; Dr. B. Bradley, Bolton General Hospital, Manchester; Dr. A. Burton, Royal Preston Hospital, Preston; Dr. K. B. Carroll, Wythenshawe Hospital, Manchester; Dr. M. Chopra, Tameside Hospital, Manchester; Dr. J. Earis, Fazakerley Hospital, Liverpool; Dr. J. J. Egan, Wythenshawe Hospital, Manchester; Dr. M. Finnegan, Bury General Hospital, Bury; Dr. Jim Finnerty,

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    Supported by South Manchester Endowment Fund. Dr. S. Lok is supported by New Heart New Start. We would like to thank Dr. Brian Farragher, from the Department of Medical Statistics, for his advice.

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