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Published ahead of print on January 25, 2007, doi:10.1164/rccm.200608-1153CC

Am. J. Respir. Crit. Care Med., Volume 175, Number 9, May 2007, 875-880

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Submitted on August 15, 2006
Accepted on January 25, 2007

Idiopathic Pulmonary Fibrosis and Pulmonary Hypertension: Connecting the Dots

Steven D Nathan1*, Paul W Noble2, and Rubin M Tuder3

1 Advanced Lung Disease and Transplant Program, Inova Fairfax Hospital, Falls Church, VA, USA, 2 Division of Pulmonary, Allergy and Critical Care Medicine, Duke University, Durham, NC, USA, 3 Department of Pathology, Johns Hopkins University School of Medicine, Baltimore, MD, USA

* To whom correspondence should be addressed. E-mail: steven.nathan{at}inova.com.

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 have not been well defined. This treatise serves to explore these relationships and to hypothesize about the possible linkage between these entities. From a prognostic standpoint, recent evidence suggests it 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 non-invasive 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 paper have previously been reported in abstract form only.
Key words: pulmonary fibrosis, pulmonary hypertension, pulmonary function tests, cytokines




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