PT  - JOURNAL ARTICLE
AU  - Waxman, Aaron B.
AU  - Elia, Davide
AU  - Adir, Yochai
AU  - Humbert, Marc
AU  - Harari, Sergio
TI  - Recent advances in the management of pulmonary hypertension with interstitial lung disease
AID  - 10.1183/16000617.0220-2021
DP  - 2022 Sep 30
TA  - European Respiratory Review
PG  - 210220
VI  - 31
IP  - 165
4099  - http://err.ersjournals.com/content/31/165/210220.short
4100  - http://err.ersjournals.com/content/31/165/210220.full
SO  - EUROPEAN RESPIRATORY REVIEW2022 Sep 30; 31
AB  - Pulmonary hypertension (PH) is known to complicate various forms of interstitial lung disease (ILD), including idiopathic pulmonary fibrosis, the interstitial pneumonias and chronic hypersensitivity pneumonitis. Pathogenesis of PH-ILD remains incompletely understood, and probably has overlap with other forms of pre-capillary pulmonary hypertension. PH-ILD carries a poor prognosis, and is associated with increased oxygen requirements, and a decline in functional capacity and exercise tolerance. Despite most patients having mild–moderate pulmonary hypertension, more severe pulmonary hypertension and signs of right heart failure are observed in a subset of cases. Clinical suspicion and findings on pulmonary function, computed tomography and echocardiography are often the initial steps towards diagnosis. Definitive diagnosis is obtained by right heart catheterisation demonstrating pre-capillary pulmonary hypertension. Drugs approved for pulmonary arterial hypertension have been investigated in several randomised controlled trials in PH-ILD patients, leading to discouraging results until the recent INCREASE study. This review provides an overview of the current understanding, approach to diagnosis and recent advances in treatment.Pulmonary hypertension complicates various forms of ILD and carries a poor prognosis. Diagnosis requires clinical suspicion and right heart catheterisation. Treatment remains limited, although results of the INCREASE study show promise. https://bit.ly/3mBokJv