Highlights
- •Serial CT variables such as change in extent of fibrosis and pulmonary artery size are predictive of negative outcomes.
- •A novel variable, the right lower lobe anterior bronchial angle, was described. Change predicts negative outcomes.
- •In addition to clinical, pulmonary function test, and single CT variables, serial CT variables predict CTD-ILD progression.
Abstract
Purpose
Progressive fibrosing interstitial lung disease (PF-ILD) is a phenotype defined by
rapid clinical progression towards respiratory failure. While idiopathic pulmonary
fibrosis is the archetype of PF-ILD, connective tissue disease associated interstitial
lung disease (CTD-ILD) can also manifest as PF-ILD. Few studies have described the
value of serial computed tomography (CT) in predicting clinical progression of ILD.
We explore which single and serial clinical and radiographic variables, in particular
serial CT variables and a novel variable, the right lower lobe anterior bronchial
angle (RLL-ABA), best predict mortality, oxygen requirement, hospital admissions,
and lung transplant in CTD-ILD.
Methods
This is a single-center retrospective study of 84 patients with a history of CTD-ILD.
Cox survival analysis was used to predict two endpoints, all-cause mortality and composite
negative outcomes (CNO): new oxygen requirement, respiratory admission, lung transplant,
and death.
Results
On serial CT, change in pulmonary artery (PA) size and RLL-ABA were predictive of
mortality and CNO, and change in fibrosis was predictive of mortality alone. On single
CT, the extent of fibrosis, PA size, and PA to aorta ratio were predictive of mortality
and CNO. Among clinical variables, oxygen requirement, forced vital capacity (FVC),
change in FVC, and worsening shortness of breath were predictive of mortality and
CNO, and diffusing capacity for carbon monoxide was predictive of mortality alone.
Conclusions
In addition to clinical and single CT variables, serial CT measurements such as change
in extent of fibrosis, PA size, PA to aorta ratio, and RLL-ABA were predictive of
mortality and CNO.
Abbreviations:
CNO (Composite negative outcomes), CT (computed tomography), CTD (connective tissue disease), CTD-ILD (connective tissue disease-related interstitial lung disease), DLCO (diffusing capacity for carbon monoxide), HRCT (high resolution computed tomography), IPF (idiopathic pulmonary fibrosis), ILD (interstitial lung disease), NSIP (nonspecific interstitial pneumonia), OP (organizing pneumonia), PF-ILD (progressive fibrosing interstitial lung disease), PA (pulmonary artery), PFT (pulmonary function test), RLL ABA (right lower lobe anterior bronchial angle), UIP (usual interstitial pneumonia)Keywords
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Article info
Publication history
Published online: December 02, 2022
Accepted:
November 28,
2022
Received in revised form:
November 17,
2022
Received:
July 26,
2022
Identification
Copyright
© 2022 Elsevier Inc. All rights reserved.
