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

Gabriele Bongiovanni, Antonio Tonutti, Anna Stainer, Mattia Nigro, Dean L. Kellogg, Anoop Nambiar, Andrea Gramegna, Marco Mantero, Antonio Voza, Francesco Blasi, Stefano Aliberti, Francesco Amati

Research output: Contribution to journalArticlepeer-review


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.

Original languageEnglish (US)
Article numbere002161
JournalBMJ Open Respiratory Research
Issue number1
StatePublished - Mar 13 2024


  • Interstitial Fibrosis
  • Primary Pulmonary Hypertension

ASJC Scopus subject areas

  • Pulmonary and Respiratory Medicine


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