Background Interstitial lung diseases (ILDs) comprise a group of more than 200 different subtypes. They vary widely in terms of incidence, prognosis and treatment, yet real-life data from Germany are sparse.Methods The prospective Exploring Clinical and Epidemiological Characteristics of Interstitial Lung Diseases (EXCITING)-ILD registry included patients with all different ILD subtypes from different healthcare settings. Follow-up ranged from 36 months to 5 years. Data were analysed descriptively. Baseline characteristics, diagnostic and treatment information are presented as absolute numbers and percentages. The Wilcoxon signed-rank sum test was used to quantify differences between groups. Line plots and bar plots were used for graphical presentation.Results A total of 601 patients (60.7% men, mean age 64.3 years) from 32 centres were included in the EXCITING-ILD registry. The most common subtypes were sarcoidosis with 26.6% (n=160) and idiopathic pulmonary fibrosis (IPF) with 25.3% (n=152). Pulmonary hypertension was present in 8.7% of patients (n=52), with high incidences in connective tissue disease-associated ILD (16.3%) and pneumoconiosis (27.3%). The mean forced vital capacity was 76.4% predicted, and the mean DLCO-SB (diffusing capacity for carbon monoxide) was 54.1% predicted. The mean time to diagnosis was 38.8 months (SD 64.4) and was significantly shorter when the diagnosis was made after multidisciplinary discussion (31.6 vs 49.2 months, p<0.001). The frequency of surgical lung biopsies decreased over time in the registry, whereas the proportion of cryobiopsies showed a notable increase. In IPF, the number of patients treated with antifibrotics increased from 35.2% before 2015 to 48.4% in 2019.Conclusion The EXCITING-ILD registry describes the frequency of ILD subtypes, ILD-related impairments, selected comorbidities and diagnostic and treatment patterns in a representative German population.

Abstract Background Interstitial lung diseases (ILD) comprise a heterogeneous group of mainly chronic lung diseases with different disease trajectories. Progression (PF-ILD) occurs in up to 50% of patients and is associated with increased mortality. Methods The EXCITING-ILD (Exploring Clinical and Epidemiological Characteristics of Interstitial Lung Diseases) registry was analysed for disease trajectories in different ILD. The course of disease was classified as significant (absolute forced vital capacity FVC decline > 10%) or moderate progression (FVC decline 5–10%), stable disease (FVC decline or increase < 5%) or improvement (FVC increase ≥ 5%) during time in registry. A second definition for PF-ILD included absolute decline in FVC % predicted ≥ 10% within 24 months or ≥ 1 respiratory-related hospitalisation. Risk factors for progression were determined by Cox proportional-hazard models and by logistic regression with forward selection. Kaplan-Meier curves were utilised to estimate survival time and time to progression. Results Within the EXCITING-ILD registry 28.5% of the patients died (n = 171), mainly due to ILD (n = 71, 41.5%). Median survival time from date of diagnosis on was 15.5 years (range 0.1 to 34.4 years). From 601 included patients, progression was detected in 50.6% of the patients (n = 304) with shortest median time to progression in idiopathic NSIP (iNSIP; median 14.6 months) and idiopathic pulmonary fibrosis (IPF; median 18.9 months). Reasons for the determination as PF-ILD were mainly deterioration in lung function (PFT; 57.8%) and respiratory hospitalisations (40.6%). In multivariate analyses reduced baseline FVC together with age were significant predictors for progression (OR = 1.00, p < 0.001). Higher GAP indices were a significant risk factor for a shorter survival time (GAP stage III vs. I HR = 9.06, p < 0.001). A significant shorter survival time was found in IPF compared to sarcoidosis (HR = 0.04, p < 0.001), CTD-ILD (HR = 0.33, p < 0.001), and HP (HR = 0.30, p < 0.001). Patients with at least one reported ILD exacerbation as a reason for hospitalisation had a median survival time of 7.3 years (range 0.1 to 34.4 years) compared to 19.6 years (range 0.3 to 19.6 years) in patients without exacerbations (HR = 0.39, p < 0.001). Conclusion Disease progression is common in all ILD and associated with increased mortality. Most important risk factors for progression are impaired baseline forced vital capacity and higher age, as well as acute exacerbations and respiratory hospitalisations for mortality. Early detection of progression remains challenging, further clinical criteria in addition to PFT might be helpful.

Background Interstitial lung diseases (ILD) comprise a heterogeneous group of mainly chronic lung diseases with more than 200 entities and relevant differences in disease course and prognosis. Little data is available on hospitalisation patterns in ILD. Methods The EXCITING-ILD (Exploring Clinical and Epidemiological Characteristics of Interstitial Lung Diseases) registry was analysed for hospitalisations. Reasons for hospitalisation were classified as all cause, ILD-related and respiratory hospitalisations, and patients were analysed for frequency of hospitalisations, time to first non-elective hospitalisation, mortality and progression-free survival. Additionally, the risk for hospitalisation according to GAP index and ILD subtype was calculated by Cox proportional-hazard models as well as influencing factors on prediction of hospitalisation by logistic regression with forward selection. Results In total, 601 patients were included. 1210 hospitalisations were recorded during the 6 months prior to registry inclusion until the last study visit. 800 (66.1%) were ILD-related, 59.3% of admissions were registered in the first year after inclusion. Mortality was associated with all cause, ILD-related and respiratory-related hospitalisation. Risk factors for hospitalisation were advanced disease (GAP Index stages II and III) and CTD (connective tissue disease)-ILDs. All cause hospitalisations were associated with pulmonary hypertension (OR 2.53, p = 0.005). ILD-related hospitalisations were associated with unclassifiable ILD and concomitant emphysema (OR = 2.133, p = 0.001) as well as with other granulomatous ILDs and a positive smoking status (OR = 3.082, p = 0.005). Conclusion Our results represent a crucial contribution in understanding predisposing factors for hospitalisation in ILD and its major impact on mortality. Further studies to characterize the most vulnerable patient group as well as approaches to prevent hospitalisations are warranted.

In patients with chronic obstructive pulmonary disease (COPD), sleep is often fragmented while, conversely, the use of sleep medications is of concern in these patients due to potential impairment of nocturnal breathing. This randomised, double‐blind, placebo‐controlled, two‐period crossover study was conducted to evaluate the effect of the new dual orexin receptor antagonist daridorexant on night‐time respiratory function and sleep in patients with moderate COPD. In each period, the highest Phase‐III dose of 50 mg daridorexant or placebo was administered once daily in the evening for 5 consecutive days. The primary endpoint was peripheral oxygen saturation (SpO2) during total sleep time (TST) after last dosing. Night‐time respiratory function and sleep were further evaluated based on the apnea–hypopnea index (AHI), sleep duration, and objective sleep parameters. Pharmacokinetics, safety, and tolerability were also assessed. Primary endpoint analysis revealed no significant mean treatment difference (i.e. daridorexant – placebo) for SpO2 during TST as it was 0.18% (90% confidence interval: −0.21 to 0.57). There was also no difference from placebo for SpO2 during non‐rapid eye movement (REM) and REM sleep at Night 5 and after first dosing. The AHI was slightly increased compared to placebo, but not to a clinically meaningful extent. In addition, daridorexant improved objective sleep parameters (i.e. prolonged TST, increased sleep efficiency, and decreased wake after sleep onset), reached expected plasma concentrations, and was safe and well tolerated. In conclusion, single and multiple doses of 50 mg daridorexant do not impair night‐time respiratory function and improves sleep in patients with moderate COPD.

Background: Real life data on outcome of patients with PF-ILD are scant. Method: The prospective German EXCITING registry was evaluated for patients with PF-ILD, defined as either decline in vital capacity (VC) ≥ 10 % or respiratory hospitalisation within 24 months. Only patients with ≥2 VC measures in 24 months were analysed. Results: Out of 601 patients, 128 patients with PF-ILD were identified. Baseline characteristics were 47% male, age 69 years, 47% ex-/smokers, mean VC 75%, DLCO 40%, 6MWD 371 m, GAP index I 45%, II 31%, III 20%. Patients had non-specific interstitial pneumonia (16%), unclassifiable ILD (15%), hypersensitivity pneumonitis (31%), ILD in connective tissue diseases (21%), asbestos (5%), other ILDs (12%). 5-year survival rates for PF-ILD were 78%, compared to 65% for IPF (n=152, 76% male, 74 years, VC 72%, DLCO 46%) and 85% for non-PF ILD (n=45; 58% male, 67 years, VC 68%, DLCO 49%) (fig.). In a cox regression model (comparator IPF), hazard ratios for death were 0.62 (0.42-0.9) for PF-ILD (p=0.013) and 0.29 (0.13-0.64) for non-PF-ILD (p=0.002). In a supervised learning approach age ≥55 years and BMI < 34 were predictors of PF. An alternative PF definition (VC decline ≥ 5 % in 24 months) was discrepant in patient assignment in 17%. Conclusions: PF-ILD is associated with a significantly impaired survival compared to non-PF ILD patients, but better than IPF.

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.

Background Available epidemiological data suggest that IPF is a frequent and unfavourable fibrosing ILD. Yet, data on outcome differences between IPF and other ILDs are scant.