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Review
. 2025 Sep 10:S0012-3692(25)05167-0.
doi: 10.1016/j.chest.2025.07.4107. Online ahead of print.

Pulmonary Hypertension Associated With Interstitial Lung Diseases

Arun Jose  1 , Namita Sood  2 , Jean M Elwing  3 , Bindu Akkanti  4 , Abubakr Bajwa  5 , Roberto Bernardo  6 , Rodolfo A Estrada  7 , Munish Sharma  8 , Francisco J Soto  9 , Adriano R Tonelli  10 , Divya Verma  11 , Janine Vintch  12 , Sandeep Sahay  13 , Oksana A Shlobin  14 ; Pulmonary Vascular Disease (PVD) Section
Affiliations
Free article
Review

Pulmonary Hypertension Associated With Interstitial Lung Diseases

Arun Jose et al. Chest. .
Free article

Abstract

Interstitial lung disease (ILD) is a term encompassing a wide array of pulmonary conditions characterized by inflammation and fibrosis of the pulmonary parenchyma. Pulmonary hypertension (PH) is frequently encountered in patients with fibrotic ILDs and poses unique difficulties for both diagnosis and management. Patients with ILD-associated pulmonary hypertension (ILD-PH) are complex, often ailing and presenting with multiple comorbidities whose individual contributions to the underlying PH can be challenging to disentangle. Evidence supporting treatment with PH-specific medications in ILD-PH is limited. This edition of "How I Do It" presents a longitudinal case-based discussion of ILD-PH to address these challenges, highlight pearls and pitfalls in the diagnostic workup of these patients, and provide a framework for the practical evidence-based approach to accurate diagnosis and management of these challenging patients.

Keywords: diagnosis; interstitial lung disease; pulmonary arterial hypertension; pulmonary hypertension; treatment.

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Conflict of interest statement

Financial/Nonfinancial Disclosures The authors have reported to CHEST the following: A. J. reports serving on the consultant or advisory board of Janssen and Merck, has received research funding from United Therapeutics. N. S. has served on the speakers bureau for Bayer and has received research grant support from United Therapeutics and Insmed. J. M. E. has received research grant support from Janssen, United Therapeutics, Liquidia, Gossamer Bio, Bayer, Merck, Altavant, Aerovate, Pulmovant, and serves on the consultant or advisory board of United Therapeutics, Altavant, Aerovate, Pulmovant, Bayer, Gossamer Bio, Liquidia, Merck, and Janssen. B. A. has served on the consultant or advisory board and speakers bureau for Janssen. R. B. has served on the consultant or advisory board for Merck, Janssen, United Therapeutics. A. R. T. has served on the consultant or advisory board for Janssen and Merck. R. A. E. has served on the consultant or advisory board and speakers bureau for United Therapeutics, Janssen, and Merck, and has served on the consultant or advisory board for Gossamer Bio and Liquidia. J. V. has served as a medical expert witness, on the DSMB for Syneos, and is supported by an NIH grant (U34HL147347: PE-TRACT trial). S. S. has served on the consultant and advisory board for Merck, Janssen, and United Therapeutics, has received research support from United Therapeutics, Keros, Liquidia, and Pulmovant, and directs the pulmonary hypertension program at the Houston Methodist Hospital supported by the Charles and Diane PH Fund. O. A. S. serves on the steering committee for Gossamer, Insmed, and AllRock, has served on the consultant or advisory board of Merck and United Therapeutics, and has served on the speakers bureau for United Therapeutics. None declared (A. B., M. S., F. J. S., D. V.).

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