{
  "abstract": "Background Pulmonary vessel-related structural (PVRS) abnormalities have been implicated in usual interstitial pneumonia; however, their significance in idiopathic inflammatory myopathy-associated interstitial lung disease (IIM-ILD) remains unclear. In this two-centre study, we evaluated the associations between PVRS parameters on high-resolution computed tomography (HRCT) and rapid progression and prognosis in patients with IIM-ILD.Methods A total of 578 IIM-ILD patients (412 females; median age, 53 years) were included from retrospective ILD cohorts of two centres. Artificial intelligence (AI)-based quantification was performed on baseline HRCT to assess PVRS and interstitial lesions. The value of PVRS for rapid progression and prognosis was first evaluated in the cohort from the first centre using logistic regression, Kaplan-Meier analysis and Cox models. An independent cohort of 64 patients (43 female; median age, 54 years) from the second centre then served to validate the generalisability of the PVRS-based model of IIM-ILD progression.Results In the first-centre cohort, 249 patients with rapidly progressive ILD (RP-ILD) showed significantly elevated mean pulmonary vascular diameter (mPVD) (p<0.05), shorter vascular-pleural distances, greater PVRS volume and higher standard deviation of pulmonary vascular diameter (sdPVD) (p<0.001) compared with non-RP-ILD patients. Multivariate analysis identified age (HR 1.03, 95% CI 1.01 to 1.06), ground glass opacity percentage (HR 1.04, 95% CI 1.02 to 1.06) and sdPVD at 6 mm and 18 mm from the pleura as independent risk factors for poor prognosis in antisynthetase syndrome (ASS) patients (concordance index (C-index)=0.819). In contrast, for patients with antimelanoma differentiation-associated gene 5 antibody-positive dermatomyositis (MDA5+DM), the independent risk factors were age (HR 1.06, 95% CI 1.02 to 1.11), mPVD at 6 mm from the pleura and lactic dehydrogenase levels (C-index=0.835). When applied to the external validation cohort, the respective multivariate Cox models yielded C-indices of 0.841 for ASS and 0.814 for MDA5+DM, confirming their generalisability.Conclusions Our study demonstrates that baseline PVRS parameters on HRCT are robust imaging biomarkers associated with rapid progression and poor prognosis in IIM-ILD. These findings underscore the clinical relevance of PVRS assessment in risk stratification and management.",
  "authors": [
    {
      "affiliations": [
        "Immune Dysfunction and Pulmonary Fibrosis Joint Laboratory for Clinical Medicine, Capital Medical University, Beijing, China",
        "National Center for Respiratory Medicine",
        "State Key Laboratory of Respiratory Health and Multimorbidity",
        "National Clinical Research Center for Respiratory Diseases",
        "Institute of Respiratory Medicine, Chinese Academy of Medical Sciences",
        "Department of Pulmonary and Critical Care Medicine, Center of Respiratory Medicine, China-Japan Friendship Hospital, Beijing, China"
      ],
      "name": "Yuhui Qiang"
    },
    {
      "affiliations": [
        "China-Japan Friendship Hospital (Institute of Clinical Medical Sciences), Chinese Academy of Medical Sciences & Peking Union Medical College, Beijing, China"
      ],
      "name": "Hongyi Wang"
    },
    {
      "affiliations": [
        "Department of Pulmonary and Critical Care Medicine, General Hospital of Ningxia Medical University, Yinchuan, China"
      ],
      "name": "Xiaoyan Yang"
    },
    {
      "affiliations": [
        "China-Japan Friendship Hospital (Institute of Clinical Medical Sciences), Chinese Academy of Medical Sciences & Peking Union Medical College, Beijing, China",
        "Department of Radiology, China-Japan Friendship Hospital, Beijing, China"
      ],
      "name": "Yifei Ni"
    },
    {
      "affiliations": [
        "Immune Dysfunction and Pulmonary Fibrosis Joint Laboratory for Clinical Medicine, Capital Medical University, Beijing, China",
        "Department of Radiology, China-Japan Friendship Hospital, Beijing, China"
      ],
      "name": "Jianping Wang"
    },
    {
      "affiliations": [
        "China-Japan Friendship Hospital (Institute of Clinical Medical Sciences), Chinese Academy of Medical Sciences & Peking Union Medical College, Beijing, China",
        "Department of Radiology, China-Japan Friendship Hospital, Beijing, China"
      ],
      "name": "Anqi Liu"
    },
    {
      "affiliations": [
        "China-Japan Friendship Hospital (Institute of Clinical Medical Sciences), Chinese Academy of Medical Sciences & Peking Union Medical College, Beijing, China",
        "Department of Radiology, China-Japan Friendship Hospital, Beijing, China"
      ],
      "name": "Jie Du"
    },
    {
      "affiliations": [
        "Immune Dysfunction and Pulmonary Fibrosis Joint Laboratory for Clinical Medicine, Capital Medical University, Beijing, China",
        "National Center for Respiratory Medicine",
        "State Key Laboratory of Respiratory Health and Multimorbidity",
        "National Clinical Research Center for Respiratory Diseases",
        "Institute of Respiratory Medicine, Chinese Academy of Medical Sciences",
        "Department of Pulmonary and Critical Care Medicine, Center of Respiratory Medicine, China-Japan Friendship Hospital, Beijing, China"
      ],
      "name": "Linfeng Xi"
    },
    {
      "affiliations": [
        "Immune Dysfunction and Pulmonary Fibrosis Joint Laboratory for Clinical Medicine, Capital Medical University, Beijing, China",
        "National Center for Respiratory Medicine",
        "State Key Laboratory of Respiratory Health and Multimorbidity",
        "National Clinical Research Center for Respiratory Diseases",
        "Institute of Respiratory Medicine, Chinese Academy of Medical Sciences",
        "Department of Pulmonary and Critical Care Medicine, Center of Respiratory Medicine, China-Japan Friendship Hospital, Beijing, China"
      ],
      "name": "Yinan Hu"
    },
    {
      "affiliations": [
        "Immune Dysfunction and Pulmonary Fibrosis Joint Laboratory for Clinical Medicine, Capital Medical University, Beijing, China",
        "National Center for Respiratory Medicine",
        "State Key Laboratory of Respiratory Health and Multimorbidity",
        "National Clinical Research Center for Respiratory Diseases",
        "Institute of Respiratory Medicine, Chinese Academy of Medical Sciences",
        "Department of Pulmonary and Critical Care Medicine, Center of Respiratory Medicine, China-Japan Friendship Hospital, Beijing, China"
      ],
      "name": "Yanhong Ren"
    },
    {
      "affiliations": [
        "Immune Dysfunction and Pulmonary Fibrosis Joint Laboratory for Clinical Medicine, Capital Medical University, Beijing, China",
        "National Center for Respiratory Medicine",
        "State Key Laboratory of Respiratory Health and Multimorbidity",
        "National Clinical Research Center for Respiratory Diseases",
        "Institute of Respiratory Medicine, Chinese Academy of Medical Sciences",
        "Department of Pulmonary and Critical Care Medicine, Center of Respiratory Medicine, China-Japan Friendship Hospital, Beijing, China"
      ],
      "name": "Bingbing Xie"
    },
    {
      "affiliations": [
        "Immune Dysfunction and Pulmonary Fibrosis Joint Laboratory for Clinical Medicine, Capital Medical University, Beijing, China",
        "National Center for Respiratory Medicine",
        "State Key Laboratory of Respiratory Health and Multimorbidity",
        "National Clinical Research Center for Respiratory Diseases",
        "Institute of Respiratory Medicine, Chinese Academy of Medical Sciences",
        "Department of Pulmonary and Critical Care Medicine, Center of Respiratory Medicine, China-Japan Friendship Hospital, Beijing, China"
      ],
      "name": "Shiyao Wang"
    },
    {
      "affiliations": [
        "Department of Radiology, General Hospital of Ningxia Medical University, Yinchuan, China"
      ],
      "name": "Li Zhu"
    },
    {
      "affiliations": [
        "Immune Dysfunction and Pulmonary Fibrosis Joint Laboratory for Clinical Medicine, Capital Medical University, Beijing, China",
        "National Center for Respiratory Medicine",
        "State Key Laboratory of Respiratory Health and Multimorbidity",
        "National Clinical Research Center for Respiratory Diseases",
        "Institute of Respiratory Medicine, Chinese Academy of Medical Sciences",
        "Department of Pulmonary and Critical Care Medicine, Center of Respiratory Medicine, China-Japan Friendship Hospital, Beijing, China"
      ],
      "name": "Jing Geng"
    },
    {
      "affiliations": [
        "Department of Radiology, China-Japan Friendship Hospital, Beijing, China"
      ],
      "name": "Min Liu"
    },
    {
      "affiliations": [
        "Immune Dysfunction and Pulmonary Fibrosis Joint Laboratory for Clinical Medicine, Capital Medical University, Beijing, China",
        "National Center for Respiratory Medicine",
        "State Key Laboratory of Respiratory Health and Multimorbidity",
        "National Clinical Research Center for Respiratory Diseases",
        "Institute of Respiratory Medicine, Chinese Academy of Medical Sciences",
        "Department of Pulmonary and Critical Care Medicine, Center of Respiratory Medicine, China-Japan Friendship Hospital, Beijing, China"
      ],
      "name": "Huaping Dai"
    }
  ],
  "title": "Prognostic value of pulmonary vessel-related structures in rapid progression of idiopathic inflammatory myopathy-associated interstitial lung disease: a retrospective study from two centres",
  "uid": "88595959-290b-5df5-b571-3c772acd8f49"
}