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Idiopathic Pulmonary Fibrosis and Pulmonary Hypertension

Connecting the Dots

¹ Advanced Lung Disease and Transplant Program, Inova Fairfax Hospital, Falls Church, Virginia; ² Division of Pulmonary, Allergy, and Critical Care Medicine, Duke University, Durham, North Carolina; and ³ Department of Pathology, Johns Hopkins University School of Medicine, Baltimore, Maryland

Correspondence and requests for reprints should be addressed to Steven D. Nathan, M.D., Medical Director, Advanced Lung Disease and Transplant Program, Inova Heart & Vascular Institute, Inova Fairfax Hospital, 3300 Gallows Road, Falls Church, VA 22042. E-mail: steven.nathan{at}inova.com

ABSTRACT

Idiopathic pulmonary fibrosis (IPF) has a poor prognosis and a course that is unpredictable. Pulmonary hypertension may complicate the course of IPF and potentially impact prognosis. There are multiple factors that might influence the onset and severity of pulmonary hypertension in IPF. The relationship between the physiologic and pathobiologic manifestations of the progressive fibrotic process and interceding pulmonary hypertension has not been well defined. This article serves to explore these relationships and to hypothesize about the possible linkage between these entities. From a prognostic standpoint, recent evidence suggests this to be important to assess for pulmonary hypertension in patients with IPF. The appropriate triggers for evaluating for pulmonary hypertension and the best method of detection require further study. Despite the relative ease of noninvasive methods, such as echocardiography, right-heart catheterization remains the best diagnostic test. The appeal of pulmonary hypertension in IPF is that it may be an enticing therapeutic target in a disease that otherwise does not have any proven effective therapies. Which agent(s) might be useful and when they should be implemented mandate the appropriate studies being performed. Some of the data presented in this article have previously been reported in abstract form only.

Key Words: pulmonary fibrosis • hypertension, pulmonary • pulmonary function tests • cytokines

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