Pulmonary Hypertension in Idiopathic Pulmonary Fibrosis: Epidemiology and Clinical Correlates

Shorr, Andrew F.; Cors, Cindy; Lettieri, Christopher J.; Helman, Donald L.; Nathan, Steven D.

doi:10.1378/chest.128.4_meetingabstracts.218s-a

Cited by 2 publications

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“…Data from the UNOS showed that 45% of patients with IPF listed for transplant have right-heart catheterization evidence of PH, with 9% of patients having a mean pulmonary arterial pressure (mPAP) of greater than 40 mm Hg (22). This wide range in the prevalence of PH reported likely reflects the timing of the measurement during the course of the patient's disease, with patients who are later in their disease course manifesting more evidence of PH.…”

Section: Prevalence Of Ph In Ipfmentioning

confidence: 99%

Idiopathic Pulmonary Fibrosis and Pulmonary Hypertension

Nathan¹,

Noble²,

Tuder³

2007

Am J Respir Crit Care Med

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195

139

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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.

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Section: Prevalence Of Ph In Ipfmentioning

confidence: 99%

Idiopathic Pulmonary Fibrosis and Pulmonary Hypertension

Nathan¹,

Noble²,

Tuder³

2007

Am J Respir Crit Care Med

Self Cite

195

139

View full text Add to dashboard Cite

show abstract

“…11 An analysis of the UNOS registry revealed 45% of IPF patients listed for transplantation were found to have evidence of PH on RHC. 30 A further study of those with advanced IPF referred for transplantation assessment found a 31.6% incidence of PH. 24 The presence and severity of PH are possibly better markers of disease severity than pulmonary function, specifically lung volumes.…”

Section: Pulmonary Hypertensionmentioning

confidence: 96%

Interstitial Lung Disease and Lung Transplantation

O’Beirne

Counihan

Keane

2010

Semin Respir Crit Care Med

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Idiopathic pulmonary fibrosis (IPF) is the most common of the idiopathic interstitial pneumonias and carries the worst prognosis. Currently the best treatment option is lung transplantation. Historically patients with IPF had poor outcomes following referral for lung transplant due to high waiting-list mortality. The introduction of the lung allocation score in the United States in 2005 has reduced 1 year waiting-list mortality from 21% to 11% and also led to IPF becoming the most common diagnosis for lung transplantation. Although prioritizing all patients with IPF on the waiting list has led to a dramatic decline in waiting-list mortality, further improvements may be made by prioritizing which patients with IPF should be transplanted, with an emphasis on 6-minute walk testing, biomarkers, and the presence of pulmonary hypertension rather than traditional pulmonary function tests. The choice of surgical procedure and the prediction and management of posttransplant complications may also influence long-term outcomes.

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Pulmonary Hypertension in Idiopathic Pulmonary Fibrosis: Epidemiology and Clinical Correlates

Cited by 2 publications

References 0 publications

Idiopathic Pulmonary Fibrosis and Pulmonary Hypertension

Idiopathic Pulmonary Fibrosis and Pulmonary Hypertension

Interstitial Lung Disease and Lung Transplantation

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