{
  "abstract": "Introduction The objective of our observational, retrospective, and monocentric study was to compare the transplant-free survival data of patients with SSc-PAH from our database.Material and Methods In this study, we enrolled 65 patients out of the 808 affected by SSc from our Database. The inclusion criteria of our study required a diagnosis of SSc and pulmonary arterial hypertension (group I) or mixed genesis pulmonary hypertension (group I/III). All patients had a mean PAP >= 25 mmHg at right heart catheterization, and all met the ACR/EULAR 2013 classification criteria for SSc. We divided the patients into two cohorts: A and B. Cohort A included those patients who were diagnosed with hemodynamic pulmonary arterial or mixed hypertension between 1999 and 2010. Cohort B included patients with the same inclusion criteria but who were diagnosed between 2010 and 2021.26 patients belonged to cohort A: 57.7% tested positive for anticentromere antibodies, 38.4% for anti-SCL-70 antibodies, and 3.8% for anti-RNA polymerase III antibodies. All patients in cohort A had a lcSSc, 88.46% were female. Cohort B included 39 patients, of whom 53.8% tested positive for ACA, 41% for anti-SCL 70 antibodies, and 5.1% for anti-RNA polymerase III antibodies. 87.2% had a lcSSc, 89.74% were female.Results In cohort A, 24 out of 26 patients died, of which two from cancer, one from interstitial lung disease, and 21 (80.76%) from complications directly related to pulmonary arterial hypertension. On average, death from pulmonary hypertension occurred 2-4 years after the diagnosis of SSc-PAH. In cohort B, 11 patients died, of which one from cancer, three from cardiac arrest, and seven from pulmonary arterial hypertension (18%).Patients in Cohort B had better clinical, echocardiographic, and hemodynamic characteristics compared to patients in Cohort A, likely due to the earlier diagnosis and the diversity of therapies they were subjected to. In particular, patients in Cohort B had a better functional class, lower serum levels of nt-proBNP, better performance on the 6MWD, better echocardiographic parameters, and better mean PAP at cardiac catheterizationConclusions The results of our study are consistent with the findings of the largest study conducted on the survival of patients with SSc-PAH by Johns Hopkins University. We indeed found an improvement in transplant-free survival in patients from Cohort B compared to patients from Cohort A. Secondly, we found that patients in Cohort B had better clinical, echocardiographic, and hemodynamic baseline characteristics compared to patients in Cohort A.",
  "authors": [
    {
      "affiliations": [
        "Department of Precision Medicine - University of Campania - L. Vanvitelli, Naples, Italy"
      ],
      "name": "Giovanna Cuomo"
    },
    {
      "affiliations": [
        "Department of Precision Medicine - University of Campania - L. Vanvitelli, Naples, Italy"
      ],
      "name": "Carlo Iandoli"
    },
    {
      "affiliations": [
        "Department of Precision Medicine - University of Campania - L. Vanvitelli, Naples, Italy"
      ],
      "name": "Fiorina Tulipano"
    },
    {
      "affiliations": [
        "Department of Precision Medicine - University of Campania - L. Vanvitelli, Naples, Italy"
      ],
      "name": "Gennaro Porcelli"
    },
    {
      "affiliations": [
        "Department of Precision Medicine - University of Campania - L. Vanvitelli, Naples, Italy"
      ],
      "name": "Caterina Naclerio"
    },
    {
      "affiliations": [
        "Department of Precision Medicine - University of Campania - L. Vanvitelli, Naples, Italy"
      ],
      "name": "Giorgia Lo Faso"
    },
    {
      "affiliations": [
        "Department of Precision Medicine - University of Campania - L. Vanvitelli, Naples, Italy"
      ],
      "name": "Ciro Romano"
    }
  ],
  "title": "P.134 Improvement of survival in patients with pulmonary arterial hypertension associated with systemic sclerosis",
  "uid": "9a066acf-fe6d-51f8-9a8f-b9275e431013"
}