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Behr, J. ; Nathan, S.D.*

Pulmonary hypertension in interstitial lung disease: Screening, diagnosis and treatment.

Curr. Opin. Pulm. Med. 27, 396-404 (2021)
DOI PMC
Open Access Green as soon as Postprint is submitted to ZB.
PURPOSE OF REVIEW: Pulmonary vascular disease resulting in pulmonary hypertension in the context of interstitial lung disease (PH-ILD) is a common complication that presents many challenges in clinical practice. Despite recent advances, the pathogenetic interplay between parenchymal and vascular disease in ILD is not fully understood. This review provides an overview of the current knowledge and recent advances in the field. RECENT FINDINGS: Clinical trials employing the phosphodiesterase-5-inhibitor sildenafil delivered negative results whereas riociguat showed harmful effects in the PH-ILD population. More recently, inhaled treprostinil showed positive effects on the primary endpoint (six-min walk-distance) in the largest prospective randomized placebo-controlled trial to date in this patient population. Additionally, a pilot trial of ambulatory inhaled nitric oxide suggests beneficial effects based on the novel endpoint of actigraphy. SUMMARY: In view of these novel developments this review provides an overview of the status quo of screening, diagnosis and management of pulmonary vascular disease and PH in patients with ILD.
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Publication type Article: Journal article
Document type Scientific Article
Corresponding Author
Keywords Diagnosis ; Interstitial Lung Disease ; Pulmonary Hypertension ; Screening ; Treatment; Brain Natriuretic Peptide; Placebo-controlled Trial; Arterial-hypertension; Right Heart; Systemic-sclerosis; Exercise Capacity; 6-minute Walk; Double-blind; Nt-probnp; Fibrosis
ISSN (print) / ISBN 1070-5287
Quellenangaben Volume: 27, Issue: 5, Pages: 396-404 Article Number: , Supplement: ,
Publisher Lippincott Williams & Wilkins
Publishing Place Philadelphia
Non-patent literature Publications
Reviewing status Peer reviewed