Pulmonary hypertension due to interstitial lung disease.

Abstract:

PURPOSE OF REVIEW:Pulmonary hypertension has been reported to complicate the course of a number of fibrotic lung diseases, including idiopathic pulmonary fibrosis, chronic hypersensitivity pneumonitis and nonspecific interstitial pneumonitis. Most commonly, mild elevations in the mean pulmonary artery pressure are seen in patients with advanced pulmonary fibrosis. However, some patients may develop severe pulmonary hypertension, which appears out of proportion to the degree of their restrictive lung disease. RECENT FINDINGS:The benefits of pulmonary vasodilator therapy have yet to be established in pulmonary hypertension complicating fibrotic lung disease. In fact, one recent clinical trial examining riociguat in patients with pulmonary hypertension complicating idiopathic interstitial pneumonias was terminated early for an increased risk of death or hospitalization. Multiple clinical trials on this topic are currently ongoing, including studies examining inhaled pulmonary vasodilator therapies. SUMMARY:The development of pulmonary hypertension is associated with increased exertional oxygen requirements, worsened functional capacity and attenuated life expectancy. It is hoped that continued research will find an effective therapy for this condition, which will improve quality of life and extend life expectancy in patients with this condition.

journal_name

Curr Opin Pulm Med

authors

King CS,Nathan SD

doi

10.1097/MCP.0000000000000599

subject

Has Abstract

pub_date

2019-09-01 00:00:00

pages

459-467

issue

5

eissn

1070-5287

issn

1531-6971

pii

00063198-201909000-00011

journal_volume

25

pub_type

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