Abstract

BackgroundAcute exacerbation of idiopathic inflammatory myopathies-associated interstitial lung disease (AE-IIM-ILD) is a significant event associated with increased morbidity and mortality. However, few studies investigated the potential prognostic factors contributing to mortality in patients who experience AE-IIM-ILD. ObjectivesThe purpose of our study was to comprehensively investigate whether high-resolution computed tomography (HRCT) findings predict the 1-year mortality in patients who experience AE-IIM-ILD. MethodsA cohort of 69 patients with AE-IIM-ILD was retrospectively created. The cohort was 79.7 % female, with a mean age of 50.7. Several HRCT features, including total interstitial lung disease extent (TIDE), distribution patterns, and radiologic ILD patterns, were assessed. A directed acyclic graph (DAG) was used to evaluate the statistical relationship between variables. The Cox regression method was performed to identify potential prognostic factors associated with mortality. ResultsThe HRCT findings significantly associated with AE-IIM-ILD mortality include TIDE (HR per 10%-increase, 1.64; 95%CI, 1.29–2.1, p < 0.001; model 1: C-index, 0.785), diffuse distribution pattern (HR, 3.75, 95%CI, 1.5–9.38, p = 0.005; model 2: C-index, 0.737), and radiologic diffuse alveolar damage (DAD) pattern (HR, 6.37, 95 % CI, 0.81–50.21, p = 0.079; model 3: C-index, 0.735). TIDE greater than 58.33 %, diffuse distribution pattern, and radiologic DAD pattern correlate with poor prognosis. The 90-day, 180-day, and 1-year survival rates of patients who experience AE-IIM-ILD were 75.3 %, 66.3 %, and 63.3 %, respectively. ConclusionHRCT findings, including TIDE, distribution pattern, and radiological pattern, are predictive of 1-year mortality in patients who experience AE-IIM-ILD.

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