Titre
Pulmonary Hypertension Associated with Interstitial Lung Disease: A Review on Diagnosis and Treatment with a Focus on Emerging Therapies.
Type
article
Institution
UNIL/CHUV/Unisanté + institutions partenaires
Périodique
Auteur(s)
Genecand, L.
Auteure/Auteur
Wacker, J.
Auteure/Auteur
Guerreiro, I.
Auteure/Auteur
Lechartier, B.
Auteure/Auteur
Beghetti, M.
Auteure/Auteur
Pohle, S.
Auteure/Auteur
Darie, A.M.
Auteure/Auteur
Lichtblau, M.
Auteure/Auteur
Ulrich, S.
Auteure/Auteur
Guler, S.A.
Auteure/Auteur
Lador, F.
Auteure/Auteur
Groupes de travail
Swiss Society of Pulmonary Hypertension
Liens vers les personnes
Liens vers les unités
ISSN
1423-0356
Statut éditorial
Accepté (sous presse)
Peer-reviewed
Oui
Langue
anglais
Notes
Publication types: Journal Article ; Review
Publication Status: aheadofprint
Publication Status: aheadofprint
Résumé
Pulmonary hypertension (PH) complicating interstitial lung diseases (ILDs) is critical for symptom burden and prognosis. The prevalence of PH varies according to ILD subgroups and severities. Establishing the diagnosis of PH associated with ILDs (PH-ILDs) is complex due to overlapping symptoms, late clinical signs, and poor diagnostic performance of routine noninvasive diagnostic methods.
Treatment options for PH-ILDs are limited and target mainly the underlying parenchymal lung disease. For decades, clinical trials of PH-ILD treatment using pulmonary vasodilators have failed showing no benefits or even harm. Thus, most PH-specific therapies are contra-indicated in PH-ILDs. Recently, the landmark INCREASE trial showed that inhaled treprostinil improved exercise capacity (6-min walking distance) and NT-proBNP, stabilized forced vital capacity, and reduced clinical worsening. Inhaled treprostinil was approved by the FDA in 2021, while approval in Europe and Switzerland is pending.
After its approval in Europe and Switzerland, the optimal use of inhaled treprostinil will require a careful patient selection with comprehensive assessments (including right heart catheterization) by highly specialized expert centers treating patients with both PH and ILDs.
Treatment options for PH-ILDs are limited and target mainly the underlying parenchymal lung disease. For decades, clinical trials of PH-ILD treatment using pulmonary vasodilators have failed showing no benefits or even harm. Thus, most PH-specific therapies are contra-indicated in PH-ILDs. Recently, the landmark INCREASE trial showed that inhaled treprostinil improved exercise capacity (6-min walking distance) and NT-proBNP, stabilized forced vital capacity, and reduced clinical worsening. Inhaled treprostinil was approved by the FDA in 2021, while approval in Europe and Switzerland is pending.
After its approval in Europe and Switzerland, the optimal use of inhaled treprostinil will require a careful patient selection with comprehensive assessments (including right heart catheterization) by highly specialized expert centers treating patients with both PH and ILDs.
PID Serval
serval:BIB_96953A33AF50
PMID
Date de création
2025-05-12T06:16:08.442Z
Date de création dans IRIS
2025-05-21T02:42:40Z