RT Journal Article
SR Electronic
T1 Interstitial lung disease progression after genomic usual interstitial pneumonia testing
JF European Respiratory Journal
JO Eur Respir J
FD European Respiratory Society
SP 2201245
DO 10.1183/13993003.01245-2022
VO 61
IS 4
A1 Chaudhary, Sachin
A1 Weigt, S. Sam
A1 Ribeiro Neto, Manuel L.
A1 Benn, Bryan S.
A1 Pugashetti, Janelle Vu
A1 Keith, Rebecca
A1 Chand, Arista
A1 Oh, Scott
A1 Kheir, Fayez
A1 Ramalingam, Vijaya
A1 Solomon, Joshua J.
A1 Harper, Richart
A1 Lasky, Joseph A.
A1 Oldham, Justin M.
YR 2023
UL https://publications.ersnet.org//content/61/4/2201245.abstract
AB Background A genomic classifier for usual interstitial pneumonia (gUIP) has been shown to predict histological UIP with high specificity, increasing diagnostic confidence for idiopathic pulmonary fibrosis (IPF). Whether those with positive gUIP classification exhibit a progressive, IPF-like phenotype remains unknown.Methods A pooled, retrospective analysis of patients who underwent clinically indicated diagnostic bronchoscopy with gUIP testing at seven academic medical centres across the USA was performed. We assessed the association between gUIP classification and 18-month progression-free survival (PFS) using Cox proportional hazards regression. PFS was defined as the time from gUIP testing to death from any cause, lung transplant, ≥10% relative decline in forced vital capacity (FVC) or censoring at the time of last available FVC measure. Longitudinal change in FVC was then compared between gUIP classification groups using a joint regression model.Results Of 238 consecutive patients who underwent gUIP testing, 192 had available follow-up data and were included in the analysis, including 104 with positive gUIP classification and 88 with negative classification. In multivariable analysis, positive gUIP classification was associated with reduced PFS (hazard ratio 1.58, 95% CI 0.86–2.92; p=0.14), but this did not reach statistical significance. Mean annual change in FVC was −101.8 mL (95% CI −142.7– −60.9 mL; p<0.001) for those with positive gUIP classification and −73.2 mL (95% CI −115.2– −31.1 mL; p<0.001) for those with negative classification (difference 28.7 mL, 95% CI −83.2–25.9 mL; p=0.30).Conclusions gUIP classification was not associated with differential rates of PFS or longitudinal FVC decline in a multicentre interstitial lung disease cohort undergoing bronchoscopy as part of the diagnostic evaluation.While genomic UIP classification is not associated with categorical ILD progression, it is associated with modestly higher 1-year rate of FVC decline http://bit.ly/3ii99Gb