{
  "abstract": "Background: Patients with systemic sclerosis (SSc) may be affected by pulmonary veno-occlusive disease (PVOD), a poorly characterized form of group I pulmonary arterial hypertension (PAH). This scoping review aimed to systematically map the literature regarding SSc-PVOD to identify key clinical features, knowledge gaps, and future research areas. Methods: A systematic literature search was conducted (January 1970 to May 2026) across PubMed, Scopus, and Cochrane databases following the PRISMA-ScR guidelines. We included adult patients with right heart catheterization (RHC)-confirmed SSc-PAH and a concurrent/subsequent diagnosis of PVOD established via pulmonary edema following vasodilators, highly suggestive chest HRCT findings, or histopathology. Results: A total of 20 articles were included, encompassing 63 SSc-PVOD patients (87.2% females). Chest HRCT revealed ≥2 highly suggestive abnormalities (centrilobular ground-glass opacities, interlobular septal thickening, mediastinal lymphadenopathy) in 46/54 patients. Almost all patients had mPAP > 35 mmHg at diagnosis, and 12/25 had DLCO < 30%. Lung tissue was obtained in 13 patients (11 post-mortem); predominant histological findings were venous luminal narrowing (11/13), arteriole fibrosis (9/13), and thickened interalveolar septae (9/13). Notably, 20 out of 24 patients treated with pulmonary vasodilators developed pulmonary edema. Overall, two patients underwent lung transplantation, and 33 (52%) died. Conclusions: SSc-PVOD mimics classic SSc-PAH at RHC but carries a high risk of PAH-treatment-induced pulmonary edema. Early non-invasive recognition is vital to avoid drug-related harm and prompt transplant referral. Novel anti-remodeling therapies should be explored.",
  "abstract_shown": true,
  "affiliations": [
    "Rheumatology Unit, Department of Medicine-DIMED, Padova University Hospital, 35128 Padova, Italy.",
    "Cardiology Unit, Department of Cardiac, Thoracic, Vascular Sciences and Public Health, Padova University Hospital, 35128 Padova, Italy.",
    "Unit of Advanced Clinical and Translational Imaging, Department of Cardiac, Thoracic, Vascular Sciences and Public Health-DCTV, University of Padova, 35128 Padova, Italy.",
    "Department of Surgery, Oncology and Gastroenterology (DiSCOG), Padova University Hospital, 35128 Padova, Italy.",
    "Department of Cardiac, Thoracic, Vascular Sciences and Public Health, Padova University Hospital, 35128 Padova, Italy."
  ],
  "article_type": "review article",
  "arxiv": null,
  "author_total": 11,
  "authors": [
    {
      "affs": [
        0
      ],
      "name": "Francesco Carta"
    },
    {
      "affs": [
        0
      ],
      "name": "Beatrice Moccaldi"
    },
    {
      "affs": [
        1
      ],
      "name": "Giulia Famoso"
    },
    {
      "affs": [
        2
      ],
      "name": "Chiara Giraudo",
      "orcid": "0000-0003-0924-5883"
    },
    {
      "affs": [
        0
      ],
      "name": "Anna Cuberli"
    },
    {
      "affs": [
        0
      ],
      "name": "Marco Binda"
    },
    {
      "affs": [
        0
      ],
      "name": "Andrea Benini",
      "orcid": "0009-0009-7725-0626"
    },
    {
      "affs": [
        1
      ],
      "name": "Martina Perazzolo Marra"
    },
    {
      "affs": [
        0,
        3
      ],
      "name": "Roberta Ramonda",
      "orcid": "0000-0002-9683-8873"
    },
    {
      "affs": [
        1
      ],
      "name": "Laura De Michieli",
      "orcid": "0000-0003-0574-4052"
    },
    {
      "affs": [
        0,
        4
      ],
      "name": "Elisabetta Zanatta"
    }
  ],
  "bibtex": "https://research.draconitic.com/paper/f7dbz075eqdr2.bib",
  "cited_by": 0,
  "doi": "10.3390/jcdd13100506",
  "fulltext_url": "https://europepmc.org/articles/PMC13705322",
  "id": "f7dbz075eqdr2",
  "issns": [
    "2308-3425"
  ],
  "landing_url": "https://europepmc.org/article/MED/42930902",
  "language": "eng",
  "licence": "CC-BY",
  "licence_label": "CC BY",
  "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": "W7212280619",
  "pdf_url": "https://europepmc.org/articles/PMC13705322?pdf=render",
  "pmcid": "PMC13705322",
  "pmid": "42930902",
  "published": "2026-10-08",
  "publisher": null,
  "ris": "https://research.draconitic.com/paper/f7dbz075eqdr2.ris",
  "sources": [
    {
      "first_seen": "2026-10-30T12:22:01Z",
      "native_id": "MED:42930902",
      "source": "europepmc",
      "url": "https://europepmc.org/article/MED/42930902"
    }
  ],
  "subjects": [
    {
      "field": "medicine",
      "origin": "journal-openalex:2705|Cardiac Valve Diseases and Treatments",
      "subfield": "cardiovascular"
    }
  ],
  "title": "Pulmonary Veno-Occlusive Disease in Systemic Sclerosis: A Scoping Review of the Literature.",
  "title_text": "Pulmonary Veno-Occlusive Disease in Systemic Sclerosis: A Scoping Review of the Literature.",
  "type": "peer-reviewed",
  "updated_at": "2026-10-30T12:22:01Z",
  "url": "https://research.draconitic.com/paper/f7dbz075eqdr2",
  "venue": "Journal of cardiovascular development and disease",
  "versions": []
}