Interims analysis of the EXCITING-ILD registry (registry for exploring clinical and epidemiological characteristics of interstitial lung diseases)

Background: The epidemiologic knowledge on interstitial lung diseases (ILD) is limited. The multi-centre ILD registry “EXCITING” collects data on characteristics, management and outcomes of all ILDs. Method: Since 10/2014 prevalent and incident ILD patients are recruited prospectively. Results: Until 01/2016 201 patients were included: 62% male, median age 68 years, 57% current/ex-smokers, 52% with symptoms > 6 months before diagnosis. Median FVC was 72%, DLCO 51%, GAP-ILD-Index 0-1 28%, 2-3 25%, 4-5 30%, 6-8 17%. Diagnostic procedures were: 91% CT (49% HRCT), 88% pulmonary-function-testing, 75% BAL, 24% surgical lung biopsies; 58% multidisciplinary. Patients had following diseases: IIP 41% (IPF 32%, NSIP 3%, DIP 2%, COP 2%), hypersensitivity pneumonitis 18%, CTD-ILD 6%, sarcoidosis 23%, unclassifiable 6%, LAM 1%, drug-induced ILD 2%; PAP 1%, 6 had familial forms. Relevant comorbidities were 24% GERD, 8% PH, 11% emphysema. Drugs used were Azathioprine 9%, Prednisolone 64%, NAC 16%, Pirfenidone 17%, Nintedanib 14%, Cyclophosphamide 3%, MTX 7%, MMF 1%, Rituximab 1%, Sirolimus 1%, clinical trials 9%. Further therapies included physiotherapy 2%, non-invasive ventilation 7%, long-term-oxygen 24%, pulmonary rehabilitation 7%; 0.6% listed for lung transplant. In the preceding 6 months prior to inclusion 47% were hospitalised, 65% of these for ILD reasons including pneumonia 7%, AE-ILD 18% and pneumothorax 4%. Conclusions: The current analysis allows first insights into diagnostics and therapy of ILDs in Germany. Unexpectedly, many patients present with severe disease (GAP-ILD index) and ILD associated hospitalizations are frequent.