Chest
Volume 140, Issue 1, July 2011, Pages 221-229
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Special Features
Long-term Course and Prognosis of Idiopathic Pulmonary Fibrosis in the New Millennium

https://doi.org/10.1378/chest.10-2572Get rights and content

The American Thoracic Society and European Respiratory Society guidelines for the diagnosis and treatment of idiopathic pulmonary fibrosis (IPF) have been published recently. However, the influence, practical application, and utility of the prior consensus statement for IPF have never been evaluated. Demographics, diagnostic criteria, pulmonary function data, and disposition of patients with IPF evaluated at an interstitial lung disease center between 2000 and 2009 were analyzed. Enrollment in clinical drug trials, lung transplantation, and mortality also were assessed. A total of 521 patients with IPF were evaluated, with pulmonary function testing available in 446. In the 64% of patients without surgical lung biopsy, the most common major criterion not fulfilled was bronchoscopy. Lung transplantation was performed in 16.1% of patients, whereas 27.4% of prescreened patients were enrolled in a prospective drug study. Patients with mild, moderate, and severe disease categorized by FVC % predicted had median survivals of 55.6, 38.7, and 27.4 months, respectively. The attrition rate of patients who survived beyond 5 years was attenuated in subsequent years. IPF remains a deadly disease with a poor prognosis. Bronchoscopy does not appear to be required for an accurate diagnosis. A minority of patients were accommodated within a clinical trial or with transplantation. Categorization by baseline FVC % predicted effectively discriminates groups with different long-term outcomes. Our analysis supports the view that the value of statements also can be realized in the subsequent demonstration of their impact on patient management, which might enable further refinements in a continuous, iterative rediscovery process.

Section snippets

Materials and Methods

We performed a review of all patients with IPF who were seen at the Inova Fairfax Hospital Interstitial Lung Disease Clinic between January 2000 and November 2009. The clinic maintains a database of all patients evaluated, with data entered prospectively at the time of their initial evaluation. This database was used as the source in identifying the cohort. When available, chart reviews were performed for missing data to provide as complete a data set as possible. Patients were given a

Baseline Data and Demographics

There were 521 patients with IPF who were seen and evaluated in the clinic during the 10-year period. The demographics of the cohort and PFT data at the time of initial evaluation, which were available in 446 of the patients, are shown in Table 1. The date of the PFT was in many cases different from the date of the patient's initial evaluation. Most of the PFTs (273 of 446, 61.2%) were within 90 days of the initial evaluation, with a median time difference of 45.7 days. In most cases (337 of

Discussion

IPF is a disease with a poor prognosis that appears unchanged over the past decade in comparison with the pre-ATS/ERS statement period. The median survival in our nontransplantation IPF cohort of about 3.5 years is very similar to survival rates reported prior to the publication of the statement.2, 3, 4 Of patients who were considered for enrollment in a prospective therapeutic study, only 27% qualified despite the availability and contiguity of multiple clinical trials, whereas only 16% of the

Acknowledgments

Financial/nonfinancial disclosure: The authors have reported to CHEST the following conflicts of interest: Dr Nathan has served as a consultant for InterMune, Gilead, Bayer, United Therapeutics, and Actelion. He also has received research funding from InterMune, Gilead, Bayer, United Therapeutics, and Actelion. Dr du Bois has served as a consultant, study steering committee member, or chairman of a steering committee for Actelion, Boehringer-Ingelheim, Bayer, Genzyme, InterMune, and

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