{
    "abstract": "Pulmonary hypertension (PH) is the leading cause of death among patients with systemic sclerosis (SSc). Recently, early therapeutic intervention to improve the prognosis was suggested, and the definition of PH was recently revised by lowering the cut-off value of mean pulmonary arterial pressure (mPAP) from \u2265\u200925 to >\u200920 mmHg. However, the optimal threshold for therapeutic intervention remains unclear. We aim to evaluate the prognosis of patients with SSc and its relationship with mPAP.\nFor this non-interventional retrospective and prospective cohort study, we enrolled patients with SSc or scleroderma spectrum disorders accompanied by other connective tissue diseases who underwent right heart catheterization (RHC) for suspected PH from 2010 to 2023. The date of the first RHC was defined as the baseline. Enrolled patients were classified into three groups based on their mPAP at the first RHC (\u2264\u200920, 21-24, and \u2265\u200925 mmHg) and are being observed from baseline up to three years. The primary endpoint is the time between the first RHC and the first hospitalisation or death due to worsening PH.\nThis study was approved by the Ethics Committee of Hokkaido University Hospital. A total of 229 patients were enrolled from 12 participating centres, with 41 prospectively followed up and 188 retrospectively followed up. The number of patients in each group (an mPAP of \u2264\u200920, 21-24, and \u2265\u200925 mmHg) is 79, 26, and 124, respectively. The observation is expected to be completed by December 2026. Findings will be disseminated at scientific conferences, peer-reviewed journals.\nThe findings of this study that we will obtain are expected to provide important information that will lead to improvements in the diagnosis of PH and the prognosis of patients.\nThis study was approved by the Ethics Committee of Hokkaido University Hospital (approval number 022-0109). It has been registered in the Japan Registry of Clinical Trials as jRCT1010220025 since November 7, 2022.",
    "authors": [
        {
            "affiliation": "Department of Rheumatology, Endocrinology and Nephrology, Faculty of Medicine and Graduate School of Medicine, Hokkaido University, Sapporo, Japan.",
            "firstname": "Shuhei",
            "initials": "S",
            "lastname": "Takeyama"
        },
        {
            "affiliation": "Division of Rheumatology, Department of Internal Medicine, Keio University School of Medicine, Tokyo, Japan.",
            "firstname": "Hironari",
            "initials": "H",
            "lastname": "Hanaoka"
        },
        {
            "affiliation": "Department of Cardiovascular, Renal and Metabolic Medicine, Sapporo Medical University, Sapporo, Japan.",
            "firstname": "Akiyoshi",
            "initials": "A",
            "lastname": "Hashimoto"
        },
        {
            "affiliation": "Department of Rheumatology, Tohoku University Hospital, Sendai, Japan.",
            "firstname": "Yusho",
            "initials": "Y",
            "lastname": "Ishii"
        },
        {
            "affiliation": "Third Department of Internal Medicine, Obihiro-Kosei Hospital, Obihiro, Japan.",
            "firstname": "Yuka",
            "initials": "Y",
            "lastname": "Shimizu"
        },
        {
            "affiliation": "Division of Cardiology, Nephrology, Pulmonology and Neurology, Department of Internal Medicine, Asahikawa Medical University, Asahikawa, Japan.",
            "firstname": "Toshiharu",
            "initials": "T",
            "lastname": "Takeuchi"
        },
        {
            "affiliation": "Department of Internal Medicine, Tomakomai City Hospital, Tomakomai, Japan.",
            "firstname": "Shuhei",
            "initials": "S",
            "lastname": "Shimoyama"
        },
        {
            "affiliation": "Department of Allergy and Rheumatology, Nippon Medical School Graduate School of Medicine, Tokyo, Japan.",
            "firstname": "Masataka",
            "initials": "M",
            "lastname": "Kuwana"
        },
        {
            "affiliation": "Division of Rheumatology, Department of Internal Medicine, Tokyo Women's Medical University School of Medicine, Tokyo, Japan.",
            "firstname": "Tomoaki",
            "initials": "T",
            "lastname": "Higuchi"
        },
        {
            "affiliation": "Department of Internal Medicine, Japanese Red Cross Kitami Hospital, Kitami, Japan.",
            "firstname": "Masaru",
            "initials": "M",
            "lastname": "Yoshimura"
        },
        {
            "affiliation": "Department of Rheumatology and Clinical Immunology, Sapporo City General Hospital, Sapporo, Japan.",
            "firstname": "Hiroshi",
            "initials": "H",
            "lastname": "Kataoka"
        },
        {
            "affiliation": "Department of Hematology and Rheumatology, Tohoku Medical and Pharmaceutical University, Sendai, Japan.",
            "firstname": "Yuko",
            "initials": "Y",
            "lastname": "Shirota"
        },
        {
            "affiliation": "Data Science Center, Promotion Unit, Institute of Health Science Innovation for Medical Care, Hokkaido University Hospital, Sapporo, Japan.",
            "firstname": "Kazufumi",
            "initials": "K",
            "lastname": "Okada"
        },
        {
            "affiliation": "Data Science Center, Promotion Unit, Institute of Health Science Innovation for Medical Care, Hokkaido University Hospital, Sapporo, Japan.",
            "firstname": "Yoichi M",
            "initials": "YM",
            "lastname": "Ito"
        },
        {
            "affiliation": "Department of Rheumatology, Endocrinology and Nephrology, Faculty of Medicine and Graduate School of Medicine, Hokkaido University, Sapporo, Japan.",
            "firstname": "Ryo",
            "initials": "R",
            "lastname": "Hisada"
        },
        {
            "affiliation": "Department of Rheumatology, Endocrinology and Nephrology, Faculty of Medicine and Graduate School of Medicine, Hokkaido University, Sapporo, Japan.",
            "firstname": "Kazuro",
            "initials": "K",
            "lastname": "Kamada"
        },
        {
            "affiliation": "Division of Rheumatology, Department of Internal Medicine, Keio University School of Medicine, Tokyo, Japan.",
            "firstname": "Sho",
            "initials": "S",
            "lastname": "Ishigaki"
        },
        {
            "affiliation": "Department of Internal Medicine, Tomakomai City Hospital, Tomakomai, Japan.",
            "firstname": "Tetsuya",
            "initials": "T",
            "lastname": "Horita"
        },
        {
            "affiliation": "Department of Rheumatology, Endocrinology and Nephrology, Faculty of Medicine and Graduate School of Medicine, Hokkaido University, Sapporo, Japan.",
            "firstname": "Tatsuya",
            "initials": "T",
            "lastname": "Atsumi"
        },
        {
            "affiliation": "Department of Rheumatology, Endocrinology and Nephrology, Faculty of Medicine and Graduate School of Medicine, Hokkaido University, Sapporo, Japan. ktmasaru@med.u-toyama.ac.jp.\nThe First Department of Internal Medicine, University of Toyama Faculty of Medicine, Toyama, Japan. ktmasaru@med.u-toyama.ac.jp.",
            "firstname": "Masaru",
            "initials": "M",
            "lastname": "Kato"
        },
        {
            "affiliation": null,
            "firstname": null,
            "initials": null,
            "lastname": null
        }
    ],
    "conclusions": null,
    "copyrights": "\u00a9 2025. The Author(s).",
    "doi": "10.1186/s41927-025-00474-2",
    "journal": "BMC rheumatology",
    "keywords": [
        "Mean pulmonary arterial pressure",
        "Pulmonary hypertension",
        "Right heart catheterization",
        "Systemic sclerosis"
    ],
    "methods": null,
    "publication_date": "2025-02-27",
    "pubmed_id": "40012072\n36017548\n32975145\n36053564\n37713430\n38581672\n16840400\n18931333\n21769843\n23950183\n23687283\n30545968\n23280155\n22307943\n31818805\n29777655\n22127844\n24092682\n31903831\n9324032\n22553077\n1090839\n27789466\n24232702\n33693238\n29447737\n31196948\n34001311\n19282344",
    "results": "This study was approved by the Ethics Committee of Hokkaido University Hospital. A total of 229 patients were enrolled from 12 participating centres, with 41 prospectively followed up and 188 retrospectively followed up. The number of patients in each group (an mPAP of \u2264\u200920, 21-24, and \u2265\u200925 mmHg) is 79, 26, and 124, respectively. The observation is expected to be completed by December 2026. Findings will be disseminated at scientific conferences, peer-reviewed journals.",
    "title": "A cohort study in HigAshi-nippon of Pulmonary hyPertensIoN in systEmic SclerosiS (HAPPINESS study): protocol and baseline data for an observational study.",
    "xml": "<Element 'PubmedArticle' at 0x7885032ebb50>"
}