Case Report: Upfront sotatercept-based triple therapy in high-risk systemic sclerosis-associated pulmonary arterial hypertension
- 1Pulmonary Hypertension Center, International University of Health and Welfare Mita Hospital
- 2Department of Allergy and Rheumatology, Nippon Medical School Graduate School of Medicine
Abstract
Systemic sclerosis-associated pulmonary arterial hypertension (SSc-PAH) carries a poor prognosis, and treatment decisions are often complicated by coexisting left-heart disease, lung disease, chronic thromboembolic disease, or pulmonary veno-occlusive disease-like vasculopathy. The role of sotatercept as part of initial therapy in treatment-naive SSc-PAH has not been established. We report a 60-year-old man with diffuse cutaneous systemic sclerosis, first recognized at referral 3 years after the onset of skin symptoms, and high-risk precapillary PAH. Careful evaluation showed no clinically significant parenchymal lung disease, chronic thromboembolic disease, or post-capillary physiology. Upfront therapy was started with macitentan, tadalafil, and sotatercept, with short-term dobutamine support for low-output hemodynamics. After 3 months, right heart catheterization showed marked improvement: pulmonary vascular resistance decreased from 12.6 to 3.7 Wood units, mean pulmonary arterial pressure decreased from 43 to 30 mmHg, and cardiac index increased from 1.50 to 2.90 L/min/m². World Health Organization functional class improved from III to II, and risk status shifted to low risk by REVEAL Lite 2, COMPERA 2.0, and the Japanese Circulation Society/Japanese Pulmonary Circulation and Pulmonary Hypertension Society 2025 model. No sotatercept-related adverse events were observed. This case documents the short-term course of upfront sotatercept-based triple therapy in carefully selected high-risk precapillary SSc-PAH.
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BibTeX
@article{tamura2026case,
title = {{Case Report: Upfront sotatercept-based triple therapy in high-risk systemic sclerosis-associated pulmonary arterial hypertension}},
author = {Yuichi Tamura and Sarasa Isobe and Hiroyuki Fujii and Yuichiro Shirai and Masataka Kuwana},
journal = {Frontiers in Cardiovascular Medicine},
year = {2026},
month = oct,
doi = {10.3389/fcvm.2026.1956123},
url = {https://www.frontiersin.org/articles/10.3389/fcvm.2026.1956123/full},
note = {CC BY 4.0},
}
RIS
TY - JOUR
TI - Case Report: Upfront sotatercept-based triple therapy in high-risk systemic sclerosis-associated pulmonary arterial hypertension
AU - Yuichi Tamura
AU - Sarasa Isobe
AU - Hiroyuki Fujii
AU - Yuichiro Shirai
AU - Masataka Kuwana
PY - 2026
DA - 2026/10/07
JO - Frontiers in Cardiovascular Medicine
DO - 10.3389/fcvm.2026.1956123
UR - https://www.frontiersin.org/articles/10.3389/fcvm.2026.1956123/full
AB - Systemic sclerosis-associated pulmonary arterial hypertension (SSc-PAH) carries a poor prognosis, and treatment decisions are often complicated by coexisting left-heart disease, lung disease, chronic thromboembolic disease, or pulmonary veno-occlusive disease-like vasculopathy. The role of sotatercept as part of initial therapy in treatment-naive SSc-PAH has not been established. We report a 60-year-old man with diffuse cutaneous systemic sclerosis, first recognized at referral 3 years after the onset of skin symptoms, and high-risk precapillary PAH. Careful evaluation showed no clinically significant parenchymal lung disease, chronic thromboembolic disease, or post-capillary physiology. Upfront therapy was started with macitentan, tadalafil, and sotatercept, with short-term dobutamine support for low-output hemodynamics. After 3 months, right heart catheterization showed marked improvement: pulmonary vascular resistance decreased from 12.6 to 3.7 Wood units, mean pulmonary arterial pressure decreased from 43 to 30 mmHg, and cardiac index increased from 1.50 to 2.90 L/min/m². World Health Organization functional class improved from III to II, and risk status shifted to low risk by REVEAL Lite 2, COMPERA 2.0, and the Japanese Circulation Society/Japanese Pulmonary Circulation and Pulmonary Hypertension Society 2025 model. No sotatercept-related adverse events were observed. This case documents the short-term course of upfront sotatercept-based triple therapy in carefully selected high-risk precapillary SSc-PAH.
SN - 2297-055X
ER -