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Original Research: Diffuse Lung Disease |

Dynamic Patient CounselingSurvivors With Idiopathic Pulmonary Fibrosis: A Novel Concept in Idiopathic Pulmonary Fibrosis

A. Whitney Brown, MD; Oksana A. Shlobin, MD, FCCP; Nargues Weir, MD, FCCP; Maria C. Albano, MD; Shahzad Ahmad, MD; Mary Smith, BS; Kevin Leslie, MD; Steven D. Nathan, MD, FCCP
Author and Funding Information

From the Advanced Lung Disease and Transplant Program (Drs Brown, Shlobin, Weir, Ahmad, and Nathan and Ms Smith), Department of Medicine, Inova Fairfax Hospital, Falls Church, VA; the Fairfax Radiological Consultants (Dr Albano), Fairfax, VA; and the Mayo Clinic (Dr Leslie), Scottsdale, AZ.

Correspondence to: Steven D. Nathan, MD, FCCP, Advanced Lung Disease and Transplant Program, Department of Medicine, Inova Fairfax Hospital, 330 Gallows Rd, Falls Church, VA 22042; e-mail: steven.nathan@inova.org


Funding/Support: The authors have reported to CHEST that no funding was received for this study.

Reproduction of this article is prohibited without written permission from the American College of Chest Physicians. See online for more details.


Chest. 2012;142(4):1005-1010. doi:10.1378/chest.12-0298
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Background:  The characteristics of long-term survivors with idiopathic pulmonary fibrosis (IPF) have never been fully elucidated. We sought to illustrate the attenuated mortality and describe the characteristics of patients with IPF who survived at least 5 years beyond their initial presentation.

Methods:  Patients with IPF evaluated between 1997 and 2006 were identified through the clinic database. Patients who survived beyond 5 years from the time of their evaluation were compared with those who died or underwent lung transplantation within 5 years. Survival analyses were performed from the time of initial evaluation and contingent on annualized survival thereafter.

Results:  Eighty-seven patients who survived at least 5 years formed the comparator group to whom other patients were contrasted. These patients had a higher BMI, FVC % predicted, FEV1 % predicted, total lung capacity % predicted, and diffusing capacity of lung for carbon monoxide % predicted, but a lower FEV1/FVC ratio and lower mean pulmonary artery pressures. More than one-half of these patients had moderate or severe disease at the time of presentation. Our annualized contingent survival analyses revealed a progressively increasing median survival dependent on the duration of the disease.

Conclusions:  Although we were able to demonstrate differences in our 5-year survivors, rather than being a distinct group, these patients appear to exist within a continuum of improving survival dependent on prior disease duration. This progressively improving time-dependent prognosis mandates the serial reevaluation of an individual patient’s projected outcomes. The implementation of dynamic counseling is an important concept in more accurately predicting life expectancy for patients with IPF who are frequently haunted by the prospects of a dismal survival.

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