Treatment and survival diversities in different forms of ILD in Germany - EXCITING registry

Background: Real life data on outcome and treatment diversities in different ILD are scant. Method: We analysed prospective data from the German EXCITING registry of consecutive ILD patients in 37 pulmonology centres. Results: Out of 601 patients (60% male, medians: age 64 years, FVC 75%, DLCO 53%), 40% had idiopathic forms (IIP), 28% sarcoidosis, 10% hypersensitivity pneumonitis (HP) and 7% CTD-ILD. At baseline, IIPs were mainly treated with antifibrotic drugs (66%), but also received prednisolone in 49%. Sarcoidosis, HP and CTD-ILD mainly received steroids (85%, 98% and 81%) and rarely other immunosuppressants (azathioprine 12%, 27%, 34%; MTX 15%, 9%, 9%). Non-pharmacological treatments were sparse and did not increase over time: pulmonary rehabilitation at baseline (base) and during follow up (FU) 4%, LTOT at base 18%, increase to 21%, and NIV 4% at base and during FU. Only 3 patients received LTX. At base, 48% (n=288) were hospitalised, in 94% due to ILD, mainly for diagnostic procedures (72%) and rarely for other reasons (pneumonia 12%, AE-ILD 16%). After 6 and 12 months, hospitalisations decreased (n=127) with less frequent ILD association (72% after 6, 42% after 12 months). Yet, non-elective hospitalisations increased (at 6 and 12 months: infections 33% and 30%, AE-ILD 15% and 23%) over time. Mean overall survival by Kaplan-Meier estimates (total cohort 168 months (± 6)) differed in patients between IIP, sarcoidosis and HP with 107 months (±7), 225 (± 6.8), and 166 months (± 18). Conclusions: ILDs differ substantial in treatment forms and outcomes. Notably, non-pharmacological treatments are mainly neglected and despite a high rate of severe ILDs only 3 LTX were performed.