Sotatercept as a Treatment for Pulmonary Hypertension Associated With Connective Tissue Disease and ILD.
- 1Respiratory Department, Hospital Universitario Ramón y Cajal, Madrid, Spain.
- 2Instituto Ramón y Cajal de Investigación Sanitaria (IRYCIS), Madrid, Spain.
- 3Pulmonary Hypertension Unit, Hospital Universitario 12 de Octubre, Madrid, Spain.
Abstract
Sotatercept is a drug approved for pulmonary arterial hypertension (PAH) that improves mortality, right ventricular function, functional class, NT-proBNP, and 6-minute walk distance. However, no clinical trials support its use in patients with pulmonary hypertension (PH) and interstitial lung disease (ILD), and respiratory adverse effects have been reported due to the development of intrapulmonary shunts. We report a 65-year-old woman with stable fibrotic ILD associated with Sjögren-antisynthetase overlap syndrome who developed severe precapillary PH. Despite treatment with inhaled treprostinil and tadalafil, she showed no clinical or hemodynamic improvement. Sotatercept and macitentan were initiated to improve hemodynamics and serve as a bridge to transplantation. After three months, the patient achieved functional class I, BNP reduction, normalization of echocardiographic PH findings, and marked hemodynamic improvement. Despite concerns regarding sotatercept-associated intrapulmonary shunts and hypoxemia, basal oxygen saturation improved from 93% to 96%. Bilateral lung transplantation was performed without immediate complications. This case supports further evaluation of the efficacy and safety profile of sotatercept in PH with concomitant ILD.
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BibTeX
@article{vargasursua2026sotatercept,
title = {{Sotatercept as a Treatment for Pulmonary Hypertension Associated With Connective Tissue Disease and ILD.}},
author = {Fernando Vargas-Ursúa and Alejandro Cruz-Utrilla and Andrés Tenes},
journal = {Open respiratory archives},
year = {2026},
month = oct,
doi = {10.1016/j.opresp.2026.100685},
url = {https://europepmc.org/article/MED/42959352},
note = {CC BY-NC-ND},
}
RIS
TY - JOUR
TI - Sotatercept as a Treatment for Pulmonary Hypertension Associated With Connective Tissue Disease and ILD.
AU - Fernando Vargas-Ursúa
AU - Alejandro Cruz-Utrilla
AU - Andrés Tenes
PY - 2026
DA - 2026/10/05
JO - Open respiratory archives
DO - 10.1016/j.opresp.2026.100685
UR - https://europepmc.org/article/MED/42959352
AB - Sotatercept is a drug approved for pulmonary arterial hypertension (PAH) that improves mortality, right ventricular function, functional class, NT-proBNP, and 6-minute walk distance. However, no clinical trials support its use in patients with pulmonary hypertension (PH) and interstitial lung disease (ILD), and respiratory adverse effects have been reported due to the development of intrapulmonary shunts. We report a 65-year-old woman with stable fibrotic ILD associated with Sjögren-antisynthetase overlap syndrome who developed severe precapillary PH. Despite treatment with inhaled treprostinil and tadalafil, she showed no clinical or hemodynamic improvement. Sotatercept and macitentan were initiated to improve hemodynamics and serve as a bridge to transplantation. After three months, the patient achieved functional class I, BNP reduction, normalization of echocardiographic PH findings, and marked hemodynamic improvement. Despite concerns regarding sotatercept-associated intrapulmonary shunts and hypoxemia, basal oxygen saturation improved from 93% to 96%. Bilateral lung transplantation was performed without immediate complications. This case supports further evaluation of the efficacy and safety profile of sotatercept in PH with concomitant ILD.
SN - 2659-6636
ER -