Associations of quantitative HRCT-derived scores of interstitial lung disease (ILD) extent with two-year transplant-free survival in patients with progressive fibrosing ILD in the ILD-PRO Registry
Author Block: G. Kim1, A. Swaminathan2, T. Whelan2, M. Neely2, J. Todd2, S. Palmer2, D. Wojdyla2, C. Conoscent3, J. Goldin1; 1Los Angeles, CA/US, 2Durham, NC/US, 3Ridgefield, CT/US
Purpose: Associations between quantitative measures of fibrosis on HRCT and survival in patients with progressive fibrosing ILDs are not well established. The purpose of this study was to evaluate the prognostic value of HRCT-derived scores in the ILD-PRO Registry.
Methods or Background: Patients had an ILD other than IPF, reticular abnormality and traction bronchiectasis, and met criteria for ILD progression within the prior 24 months. HRCT images taken closest to enrolment were analysed following lobar segmentation. A machine-learning algorithm derived the following quantitative scores: quantitative lung fibrosis (QLF); quantitative ground glass (QGG); quantitative honeycomb (QHC); quantitative ILD (QILD: sum of QLF, QGG and QHC). Cox proportional hazards models for time to death or lung transplant were fit and Kaplan-Meier event rates reported.
Results or Findings: Among 331 patients, median QLF, QGG, QHC and QILD scores were 14.8%, 22.7%, 0.07% and 42.3%, respectively. Event rates for death or lung transplant at 2 years in the highest vs lowest tertiles of QLF, QGG, QHC and QILD scores were 31.2% vs 19.9%, 30.4% vs 26.3%, 22.8% vs 19.4%, and 31.7% vs 17.3%, respectively. There were no significant differences in the risk of death or lung transplant across tertiles of any of the scores. The HRs (95% CI) for risk of death or lung transplant for the highest vs lowest tertiles of QLF, QGG, QHC and QILD scores were 2.20 (1.04, 4.66), 1.72 (0.88, 3.37), 0.92 (0.45, 1.90) and 2.38 (1.09, 5.20), respectively.
Conclusion: Patients with progressive fibrosing ILDs in the ILD-PRO Registry who had QLF or QILD scores in the highest tertiles showed trends towards an increased risk of death or lung transplant over 2 years compared with those with scores in the lowest tertiles.
Limitations: The study is limited by only evaluating associations between quantitative HRCT scores and short-term disease progression in patients with progressive pulmonary fibrosis.
Funding for this study: The IPF-PRO/ILD-PRO Registry is supported by Boehringer Ingelheim Pharmaceuticals, Inc (BIPI) and run in collaboration with the Duke Clinical Research Institute (DCRI) and enrolling centers.
Has your study been approved by an ethics committee? Yes
Ethics committee - additional information: The study was approved by the Institutional Review Board of each participating centre: ClinicalTrials.gov Identifier: NCT01915511.