Idiopathic interstitial pneumonia-associated pulmonary hypertension: A target for therapy?

Abstract

Advances in the treatment of idiopathic interstitial pneumonia (IIP) represent an urgent, unmet medical need for patients with this category of diffuse parenchymal lung disease. IIPs involve varying combinations of fibrosis and inflammation of unknown cause and may be associated with pulmonary hypertension (PH). When it occurs, PH is associated with higher oxygen needs, greater functional impairment, and increased mortality. However, whether or when PH is a maladaptive versus adaptive phenomenon remains to be determined. Despite their differing prognoses, it does appear that the IIPs may follow a similar course once PH supervenes. Therefore, it may be worthwhile to explore studies of PH medications in IIP as a group rather than as individual entities. Such a broad approach eliminates the need to nuance specific diagnoses and thereby facilitates study recruitment and broadens the applicability of the results.

• Authors: Steven D Nathan, Jürgen Behr, Vincent Cottin, Harold R Collard, Marius M Hoeper, Fernando J Martinez, Athol U Wells
• Journal: Respiratory medicine (Respir Med) Vol. 122 Suppl 1 Pg. S10-S13 (01 2017) ISSN: 1532-3064 [Electronic] England
• PMID: 27884593 (Publication Type: Journal Article, Research Support, Non-U.S. Gov't)
• Copyright: Copyright © 2016. Published by Elsevier Ltd.

Chemical References

• Enzyme Activators
• Pyrazoles
• Pyrimidines
• Nitric Oxide
• riociguat

Topics

• Cell Proliferation (drug effects)
• Clinical Trials as Topic
• Enzyme Activators (therapeutic use)
• Extracellular Matrix (drug effects)
• Fibroblasts (drug effects, pathology)
• Humans
• Hypertension, Pulmonary (complications, drug therapy)
• Idiopathic Interstitial Pneumonias (complications, drug therapy, pathology)
• Idiopathic Pulmonary Fibrosis (complications)
• Lung Diseases, Interstitial (complications, drug therapy, pathology)
• Nitric Oxide (metabolism)
• Prognosis
• Pyrazoles (metabolism, pharmacology)
• Pyrimidines (metabolism, pharmacology)
• United States