Abstract
Background: The epidemiologic knowledge on interstitial lung diseases (ILD) is limited. The multi-centre, non-interventional prospective ILD registry “EXCITING” collects data on incidence, prevalence, characteristics, management and outcomes of all different ILDs in Germany.
Method: Since 10/2014 ILD patients are recruited prospectively.
Results: Until 12/2014 51 patients were included: 59% male, median age 67 years, 50% ex-/smokers. IPF 19, EAA 7, CTD-ILD 5, sarcoid 7, unclassifiable 4, DIP 2, IBD-ILD 1, iNSIP 1, LAM 2, COP 1, drug-induced 2; 3 patients had familiar ILD. Diagnostic procedures: 100% interdisciplinary, 96% HRCT, 96% BAL, 26% surgical lung biopsies. Median function tests: FVC 70%, DLCO 44%, 6 MWD 347 m. Relevant comorbidities: 33% GERD, 9% PH, 7% emphysema. ILD-therapies: IPF: 11xPirfenidon, 2xNintedanib, 5xNAC, 1xno drug; EAA: 4xsteroids, 3xwith immunomodulator (IM); CTD-ILD: 1xsteroids, 4xwith IM sarcoid: 1xICS, 3xsteroids, 1xRituximab, 1xMTX; unclassifiable: 2xsteroids, 1xazathioprin, 1xazithromycine, DIP: 1xno drug, 1xMTX, IBD-ILD, NSIP and COP steroid-treatment, LAM 1xsirolimus,1xobservation, drug-induced ILD 2xtermination of drug. 24% received LTOT. During the last 6 months 9% underwent pulmonary rehabilitation, 32% were hospitalized due to their underlying ILD. 7% were listed for lung transplant. 53% were interested in patient support groups.
Conclusions: The current analysis of the EXCITING-ILD registry allows first interesting insights into epidemiology, diagnostic procedures and therapy of different ILD entities in Germany. In future, this registry will help to gain further knowledge into prevalence and incidence of different ILDs, their management, healthcare utilization, associated costs and their outcome.
- Copyright ©ERS 2015