| Popis: |
Background: Fibrosing interstitial lung diseases other than IPF can develop a progressive phenotype (PF-ILD). While recent clinical trials have gained significant insights into management aspects for PF-ILD, different definitions were applied. We therefore aimed to compare patient cohorts according to these definitions and associated outcomes Methods: 587 fILDs with a follow up > 24 months were identified at our tertiary ILD referral Center. Patient characteristics and outcomes were assessed retrospectively and categorized as PF-ILD according to INBUILD (IN), RELIEF (RE) and Cottin (CO; ERR 2018). Results: Patients had HP (39%), iNSIP (13%), uILD (22%), CTD-ILD (24%), and others (1%). 53.4% were male, mean age 65±11.8 years, mean FVC% 72.8±2, mean DLCO% 47.3±1. The percentage of PF-ILD differed according to the 3 criteria (CO, RE, IN) (41%, 32%, 48%, fig). Acute exacerbations occurred more frequently in PF-ILD (25.6%, 23.9%, 24.9%) than in non PF-ILD (p 12 months while RELIEF identified a worse cohort. Conclusions: This study reveals that the categorization as PF-ILD differs between definitions which has important prognostic and therapeutic implications. Prospective studies are needed to confirm these results and international accepted criteria for PF-ILD have to be defined. |
