TY - JOUR
T1 - Vasoactive drugs for the treatment of pulmonary hypertension associated with interstitial lung diseases
T2 - A systematic review
AU - Bongiovanni, Gabriele
AU - Tonutti, Antonio
AU - Stainer, Anna
AU - Nigro, Mattia
AU - Kellogg, Dean L.
AU - Nambiar, Anoop
AU - Gramegna, Andrea
AU - Mantero, Marco
AU - Voza, Antonio
AU - Blasi, Francesco
AU - Aliberti, Stefano
AU - Amati, Francesco
N1 - Publisher Copyright:
© Author(s) (or their employer(s)) 2024. Re-use permitted under CC BY-NC. No commercial re-use. See rights and permissions. Published by BMJ.
PY - 2024/3/13
Y1 - 2024/3/13
N2 - 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.
AB - 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.
KW - Interstitial Fibrosis
KW - Primary Pulmonary Hypertension
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U2 - 10.1136/bmjresp-2023-002161
DO - 10.1136/bmjresp-2023-002161
M3 - Article
C2 - 38479818
AN - SCOPUS:85187737412
SN - 2052-4439
VL - 11
JO - BMJ Open Respiratory Research
JF - BMJ Open Respiratory Research
IS - 1
M1 - e002161
ER -