{
  "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.",
  "abstract_shown": true,
  "affiliations": [
    "Pulmonary Hypertension Center, International University of Health and Welfare Mita Hospital",
    "Department of Allergy and Rheumatology, Nippon Medical School Graduate School of Medicine"
  ],
  "article_type": null,
  "arxiv": null,
  "author_total": 5,
  "authors": [
    {
      "affs": [
        0
      ],
      "name": "Yuichi Tamura"
    },
    {
      "affs": [
        0
      ],
      "name": "Sarasa Isobe"
    },
    {
      "affs": [
        0
      ],
      "name": "Hiroyuki Fujii"
    },
    {
      "affs": [
        1
      ],
      "name": "Yuichiro Shirai"
    },
    {
      "affs": [
        1
      ],
      "name": "Masataka Kuwana"
    }
  ],
  "bibtex": "https://research.draconitic.com/paper/4pd6pkvpn8yp2.bib",
  "cited_by": 0,
  "doi": "10.3389/fcvm.2026.1956123",
  "fulltext_url": "https://europepmc.org/articles/PMC13685168",
  "id": "4pd6pkvpn8yp2",
  "issns": [
    "2297-055X"
  ],
  "landing_url": "https://www.frontiersin.org/articles/10.3389/fcvm.2026.1956123/full",
  "language": "eng",
  "licence": "CC-BY-4.0",
  "licence_label": "CC BY 4.0",
  "licence_source": "article",
  "licence_url": "https://creativecommons.org/licenses/by/4.0/",
  "metadata_licence": null,
  "native_subjects": [
    [
      "openalex",
      "2740|Pulmonary Hypertension Research and Treatments"
    ]
  ],
  "openalex": "W7220853898",
  "pdf_url": "https://europepmc.org/articles/PMC13685168?pdf=render",
  "pmcid": "PMC13685168",
  "pmid": "42910119",
  "published": "2026-10-07",
  "publisher": "Frontiers Media SA",
  "ris": "https://research.draconitic.com/paper/4pd6pkvpn8yp2.ris",
  "sources": [
    {
      "first_seen": "2026-10-30T12:20:10Z",
      "native_id": "10.3389/fcvm.2026.1956123",
      "source": "crossref",
      "url": "https://www.frontiersin.org/articles/10.3389/fcvm.2026.1956123/full"
    },
    {
      "first_seen": "2026-10-30T12:20:14Z",
      "native_id": "MED:42910119",
      "source": "europepmc",
      "url": "https://europepmc.org/article/MED/42910119"
    }
  ],
  "subjects": [
    {
      "field": "medicine",
      "origin": "issn:2297-055X",
      "subfield": "cardiovascular"
    }
  ],
  "title": "Case Report: Upfront sotatercept-based triple therapy in high-risk systemic sclerosis-associated pulmonary arterial hypertension",
  "title_text": "Case Report: Upfront sotatercept-based triple therapy in high-risk systemic sclerosis-associated pulmonary arterial hypertension",
  "type": "peer-reviewed",
  "updated_at": "2026-10-30T12:20:14Z",
  "url": "https://research.draconitic.com/paper/4pd6pkvpn8yp2",
  "venue": "Frontiers in Cardiovascular Medicine",
  "versions": []
}