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Antisynthetase syndrome–related interstitial lung disease (ASyS-ILD): longitudinal imaging findings

Objectives Interstitial lung disease (ILD) impacts mortality in antisynthetase syndrome (ASyS). Computed tomographic (CT) patterns and evolution in ASyS ILD are not well described. We report longitudinal CT patterns in ASyS-ILD and their impact on survival. Methods This is a monocentric retrospectiv...

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Published in:European radiology 2023-07, Vol.33 (7), p.4746-4757
Main Authors: Wu, Wei, Collins, Bridget F., Gardner, Gregory C., Hippe, Daniel S., Ho, Lawrence A., Raghu, Ganesh, Pipavath, Sudhakar N. J.
Format: Article
Language:English
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Summary:Objectives Interstitial lung disease (ILD) impacts mortality in antisynthetase syndrome (ASyS). Computed tomographic (CT) patterns and evolution in ASyS ILD are not well described. We report longitudinal CT patterns in ASyS-ILD and their impact on survival. Methods This is a monocentric retrospective study of 47 patients with ASyS-ILD. Longitudinal CT patterns and fibrosis severity (severity of radiographic features indicating fibrosis) were analyzed by two radiologists in consensus. The association between imaging features and survival was examined using univariate Cox regression analysis. Results In total, 211 CT scans were analyzed with an average of 4 ± 2 CT scans/patient with a median follow-up of 79 months in 47 patients. Non-fibrotic patterns were present initially in 63.8% ( n  = 30) of patients, while fibrotic patterns occurred in 36.2% ( n  = 17). The initial non-fibrotic patterns/abnormalities resolved in 23.3% ( n  = 7), evolved in 6.7% ( n  = 2), persisted in 13.3% ( n  = 4), and progressed in 56.7% ( n  = 17), while initial fibrotic patterns persisted in 82.4% ( n  = 14) and progressed in 17.6% ( n  = 3). Radiographic progression of ILD (progression in CT pattern or increased fibrosis severity) occurred in 53.2% ( n  = 25) of patients. Advanced age and radiographic progression were associated with decreased survival (all p  
ISSN:1432-1084
0938-7994
1432-1084
DOI:10.1007/s00330-023-09439-w