{
  "abstract": "Introduction The objective of this study is to compare the clinical characteristics and post-lung transplant outcomes of patients with idiopathic inflammatory myopathies (IIM) and systemic sclerosis (SSc) to those with idiopathic pulmonary fibrosis (IPF).Material and Methods We retrospectively analyzed patients with interstitial lung disease (ILD) diagnosed with IIM, SSc, or IPF who underwent lung transplantation at the Vancouver General Hospital and at the Centre Hospitalier de l’Universite de Montreal ILD clinic between 2012 and 2024.Results A total of 22 IIM, 32 SSc, and 64 IPF patients were included. Among IIM patients, 45% had anti-MDA5 dermatomyositis, 32% had anti-synthetase syndrome, 14% had overlap myositis, and 9% had other DM. In the SSc group, 82% had concomitant pulmonary hypertension (PH), with 32% having severe PH. IIM patients most frequently required intensive care unit (ICU) admission prior to transplantation and were more likely to undergo emergency transplantation. Post-transplant, IIM patients experienced the longest ICU stays and hospitalizations compared to SSc and IPF patients. At 1-year follow-up, forced vital capacity was statistically lower in IIM and SSc patients compared to those with IPF, while diffusion capacity was similar across all groups. There were no significant differences in 1-year survival, survival at last follow-up, incidence of chronic lung allograft dysfunction, or malignancy.Conclusions Despite their underlying systemic autoimmune rheumatic diseases, post-transplant survival outcomes of IIM and SSc patients are comparable to those with IPF. However, their pre- and post-transplant courses are more complex, highlighting the need for tailored lung transplant strategies and multidisciplinary management in patients with IIM- and SSc-associated ILD.",
  "authors": [
    {
      "affiliations": [
        "University of British Columbia, Department of Medicine, Vancouver, Canada"
      ],
      "name": "Navid Saleh"
    },
    {
      "affiliations": [
        "University of British Columbia, Department of Medicine, Vancouver, Canada"
      ],
      "name": "Angela Chang"
    },
    {
      "affiliations": [
        "University of British Columbia, Division of Rheumatology, Vancouver, Canada"
      ],
      "name": "Alec Yu"
    },
    {
      "affiliations": [
        "Centre hospitalier de l Universite de Montréal, Division of Rheumatology, Montreal, Canada"
      ],
      "name": "Darya Jalaledin"
    },
    {
      "affiliations": [
        "Centre hospitalier de l Universite de Montréal, Division of Rheumatology, Montreal, Canada"
      ],
      "name": "Sabrina Hoa"
    },
    {
      "affiliations": [
        "Vancouver General Hospital, Division of Respirology, Vancouver, Canada"
      ],
      "name": "Robert Levy"
    },
    {
      "affiliations": [
        "Vancouver General Hospital, Division of Respirology, Vancouver, Canada"
      ],
      "name": "Jennifer Wilson"
    },
    {
      "affiliations": [
        "Centre hospitalier de l Universite de Montréal, Division of Respirology, Montreal, Canada"
      ],
      "name": "Charles Poirier"
    },
    {
      "affiliations": [
        "Vancouver General Hospital, Division of Thoracic Surgery, Vancouver, Canada"
      ],
      "name": "James Choi"
    },
    {
      "affiliations": [
        "Vancouver General Hospital, Division of Thoracic Surgery, Vancouver, Canada"
      ],
      "name": "John Yee"
    },
    {
      "affiliations": [
        "Centre hospitalier de l Universite de Montréal, Division of Rheumatology, Montreal, Canada"
      ],
      "name": "Oceane Landon-Cardinal"
    },
    {
      "affiliations": [
        "University of British Columbia, Division of Rheumatology, Vancouver, Canada"
      ],
      "name": "Hyein Kim"
    },
    {
      "affiliations": [
        "University of British Columbia, Division of Rheumatology, Vancouver, Canada"
      ],
      "name": "Kun Huang"
    }
  ],
  "title": "P.110 Lung transplant outcomes in patients with myositis- and systemic sclerosis-associated interstitial lung disease compared to idiopathic pulmonary fibrosis: a multicenter retrospective analysis",
  "uid": "557f0c10-8768-56e2-a892-4415c05a09d7"
}