Skip to main page content
U.S. flag

An official website of the United States government

Dot gov

The .gov means it’s official.
Federal government websites often end in .gov or .mil. Before sharing sensitive information, make sure you’re on a federal government site.

Https

The site is secure.
The https:// ensures that you are connecting to the official website and that any information you provide is encrypted and transmitted securely.

Access keys NCBI Homepage MyNCBI Homepage Main Content Main Navigation
. 2024 Mar 13;11(1):e002161.
doi: 10.1136/bmjresp-2023-002161.

Vasoactive drugs for the treatment of pulmonary hypertension associated with interstitial lung diseases: a systematic review

Gabriele Bongiovanni  1 , Antonio Tonutti  1 , Anna Stainer  1   2 , Mattia Nigro  1 , Dean L Kellogg 3rd  3 , Anoop Nambiar  3 , Andrea Gramegna  4   5 , Marco Mantero  4   5 , Antonio Voza  6 , Francesco Blasi  4   5 , Stefano Aliberti  1   2 , Francesco Amati  7   2
Affiliations

Vasoactive drugs for the treatment of pulmonary hypertension associated with interstitial lung diseases: a systematic review

Gabriele Bongiovanni et al. BMJ Open Respir Res. .

Abstract

Objectives: Vasoactive drugs have exhibited clinical efficacy in addressing pulmonary arterial hypertension, manifesting a significant reduction in morbidity and mortality. Pulmonary hypertension may complicate advanced interstitial lung disease (PH-ILD) and is associated with high rates of disability, hospitalisation due to cardiac and respiratory illnesses, and mortality. Prior management hinged on treating the underlying lung disease and comorbidities. However, the INCREASE trial of inhaled treprostinil in PH-ILD has demonstrated that PH-ILD can be effectively treated with vasoactive drugs.

Methods: This comprehensive systematic review examines the evidence for vasoactive drugs in the management of PH-ILD.

Results: A total of 1442 pubblications were screened, 11 RCTs were considered for quantitative synthesis. Unfortunately, the salient studies are limited by population heterogeneity, short-term follow-up and the selection of outcomes with uncertain clinical significance.

Conclusions: This systematic review underscores the necessity of establishing a precision medicine-oriented strategy, directed at uncovering and addressing the intricate cellular and molecular mechanisms that underlie the pathophysiology of PH-ILD.

Prospero registration number: CRD42023457482.

Keywords: Interstitial Fibrosis; Primary Pulmonary Hypertension.

PubMed Disclaimer

Conflict of interest statement

Competing interests: GB has nothing to disclose. AT has nothing to disclose. AS reports personal fees from Boehringer Ingelheim, outside the submitted work. MN has nothing to disclose. DLK has nothing to disclose. AN reports grants, personal fees and non-financial support from Boehringer Ingelheim, personal fees from United Therapeutics, grants from Nitto Denko and personal fees from Roche-Genentech, outside the submitted work. AG reports personal fees from Vertex, personal fees from Chiesi and personal fees from Zambon, outside the submitted work. MM has nothing to disclose. AV has nothing to disclose. FB reports grants and personal fees from AstraZeneca, personal fees from Chiesi, personal fees from GlaxoSmithKline, personal fees from Grifols, personal fees from Guidotti, grants and personal fees from Insmed, grants and personal fees from Menarini, personal fees from Novartis, personal fees from Om Pharma, personal fees from Pfizer, personal fees from Sanofi, personal fees from Vertex, personal fees from Viatris and personal fees from Zambon, outside the submitted work. SA reports personal fees from Bayer Healthcare, personal fees from Grifols, personal fees from AstraZeneca, personal fees from Zambon, grants and personal fees from Chiesi, grants and personal fees from Insmed, personal fees from GlaxoSmithKline, personal fees from Menarini, personal fees from ZetaCube and grants from Fisher & Paykel, outside the submitted work. FA reports personal fees from Boehringer Ingelheim, outside the submitted work.

Figures

Figure 1
Figure 1
Identification of studies via databases. COPD, chronic obstructive pulmonary disease; RCT, randomised controlled trial.
See this image and copyright information in PMC

Similar articles

See all similar articles

Cited by

References

    1. Poch D, Mandel J. Pulmonary hypertension. Ann Intern Med 2021;174:ITC49–64. 10.7326/AITC202104200 - DOI - PubMed
    1. Humbert M, Kovacs G, Hoeper MM, et al. . ESC/ERS guidelines for the diagnosis and treatment of pulmonary hypertension. Eur Heart J 2022;43:3618–731. 10.1093/eurheartj/ehac237 - DOI - PubMed
    1. Simonneau G, Montani D, Celermajer DS, et al. . Haemodynamic definitions and updated clinical classification of pulmonary hypertension. Eur Respir J 2019;53:1801913. 10.1183/13993003.01913-2018 - DOI - PMC - PubMed
    1. King CS, Shlobin OA. The trouble with group 3 pulmonary hypertension in interstitial lung disease: dilemmas in diagnosis and the conundrum of treatment. Chest 2020;158:1651–64. 10.1016/j.chest.2020.04.046 - DOI - PubMed
    1. Nikkho SM, Richter MJ, Shen E, et al. . Clinical significance of pulmonary hypertension in interstitial lung disease: a consensus statement from the pulmonary vascular research Institute’s innovative drug development initiative-group 3 pulmonary hypertension. Pulm Circ 2022;12:e12127. 10.1002/pul2.12127 - DOI - PMC - PubMed

Publication types

MeSH terms