ArticleBMJ open respiratory research2025
Prognostic value of pulmonary vessel-related structures in rapid progression of idiopathic inflammatory myopathy-associated interstitial lung disease: a retrospective study from two centres.
Article in BMJ open respiratory research, 2025. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 2 papers.
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2 citing papers in PubMed.
- High-resolution computed tomography assessment of connective tissue disease-associated interstitial lung disease: Advances in diagnosis and prognostic evaluation.World journal of radiology · 2026Review
- A syndromic framework for progressive pulmonary fibrosis.Respiratory research · 2026Review
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16 authors.
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Abstract
backgroundPulmonary 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.
methodsA 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.
resultsIn 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.
conclusionsOur 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.
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