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 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 < 0.05). The presence of ground-glass opacities (GGO) and predominant lower lung distribution of abnormalities on initial CTs were associated with increased survival (all p < 0.05). Conclusion Progression occurred in 56.7% of ASyS-ILD patients presenting with non-fibrotic patterns. Fibrotic patterns tended to persist. Age and radiographic progression were associated with reduced survival while the initial presence of GGO and predominant lower lobe distribution were associated with increased survival. Key Points • In ASyS-ILD, initial non-fibrotic patterns such as OP, cNSIP, or OP-cNSIP tended to progress to fNSIP. • Fibrotic patterns such as fNSIP or UIP in ASyS-ILD tended to persist without pattern changes. • GGO and lower lung predominance on initial CT were associated with better survival while advanced baseline age and radiographic ILD progression during follow-up were associated with decreased survival..

Medienart:

E-Artikel

Erscheinungsjahr:

2023

Erschienen:

2023

Enthalten in:

Zur Gesamtaufnahme - volume:33

Enthalten in:

European radiology - 33(2023), 7 vom: 14. Feb., Seite 4746-4757

Sprache:

Englisch

Beteiligte Personen:

Wu, Wei [VerfasserIn]
Collins, Bridget F. [VerfasserIn]
Gardner, Gregory C. [VerfasserIn]
Hippe, Daniel S. [VerfasserIn]
Ho, Lawrence A. [VerfasserIn]
Raghu, Ganesh [VerfasserIn]
Pipavath, Sudhakar N. J. [VerfasserIn]

Links:

Volltext [lizenzpflichtig]

Themen:

Antisynthetase syndrome
Disease progression
Lung diseases, interstitial
Pulmonary fibrosis
Tomography, X-ray computed

RVK:

RVK Klassifikation

Anmerkungen:

© The Author(s), under exclusive licence to European Society of Radiology 2023. Springer Nature or its licensor (e.g. a society or other partner) holds exclusive rights to this article under a publishing agreement with the author(s) or other rightsholder(s); author self-archiving of the accepted manuscript version of this article is solely governed by the terms of such publishing agreement and applicable law.

doi:

10.1007/s00330-023-09439-w

funding:

Förderinstitution / Projekttitel:

PPN (Katalog-ID):

OLC2144057346

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