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. 2024 Mar 6;25(1):113.
doi: 10.1186/s12931-024-02731-3.

Disease trajectories in interstitial lung diseases - data from the EXCITING-ILD registry

Katharina Buschulte  1 , Hans-Joachim Kabitz  2 , Lars Hagmeyer  3 , Peter Hammerl  4 , Albert Esselmann  5 , Conrad Wiederhold  6 , Dirk Skowasch  7 , Christoph Stolpe  8 , Marcus Joest  9 , Stefan Veitshans  10 , Marc Höffgen  11 , Phillen Maqhuzu  12 , Larissa Schwarzkopf  12   13 , Andreas Hellmann  14 , Michael Pfeifer  15 , Jürgen Behr  16 , Rainer Karpavicius  17 , Andreas Günther  18   19 , Markus Polke  20 , Philipp Höger  20 , Vivien Somogyi  21 , Christoph Lederer  20 , Philipp Markart  19   22 , Michael Kreuter  23
Affiliations

Disease trajectories in interstitial lung diseases - data from the EXCITING-ILD registry

Katharina Buschulte et al. Respir Res. .

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.

Keywords: ILD; IPF; Mortality; Progression; Risk factors.

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Conflict of interest statement

KB received payment for lectures from Boehringer Ingelheim and a grant from Sarkoidose-Netzwerk e.V. LH received fees for consulting and lectures from Boehringer-Ingelheim, GSK, AstraZeneca, Pfizer and BMS. PH received fees for lectures from Galapagos, GSK, Boehringer Ingelheim, AstraZeneca, Roche. DS reports fees for lectures or consultations from AstraZeneca, Boehringer Ingelheim, Chiesi, GSK, Janssen, MSD, Sanofi, all outside the submitted work. CS reports fees for lectures GSK, AstraZeneca, Boehringer Ingelheim, Berlin Chemie. MJ reports fees for lectures or consultations from AstraZeneca, Bencard, Boehringer Ingelheim, GSK, HAL Allergy, Sanofi, all outside the submitted work. SV reports fees from Berlin Chemie, GSK, Lilly, Pfizer, and Boehringer-Ingelheim. LS reports consulting fees from Galapagos. JB reports personal fees for lectures and consulting from Astra-Zeneca, Biogen, Boehringer-Ingelheim, BMS, Ferrer, Novartis, Roche, and Sanofi. AG reports grants, leture payments and/or consulting fees from Boehringer Ingelheim, Roche, Lung Therapeutics and Pieris. MP has received payment or honoraria for lectures, presentations or educational events from Boehringer Ingelheim and AstraZeneca. VS received support for attending meetings from CSL Behring. PM received fees for consulting and lectures from Boehringer-Ingelheim and Roche. MK reports grants, consulting fees, or payment for lectures from Boehringer Ingelheim, Galapagos, AstraZeneca, BMS and Roche. All other authors have nothing to disclose. The registry was supported by an unrestricted grant by Roche.

The authors declare no competing interests.

Figures

Fig. 1
Fig. 1
Lineplot with individual changes from baseline in FVC [% predicted]. Progression was classified as described above and is shown in four different colours. Only individuals with non-missing values are shown (n = 558). The frequencies are shown for each follow-up visit (FU) starting with the first follow-up visit (FU 1) after 6 months, FU 2 after 12 months, FU 3 after 18 months, etc. FVC = forced vital capacity
Fig. 2
Fig. 2
Alluvial plot with transitions of progression stages. This figure shows the transition between different stages of progression. These are presented in different colours for significant progression, moderate progression, stable, improvement or missing parameters. The frequencies are shown for each follow-up visit (FU) starting with the first follow-up visit (FU 1) after 6 months, FU 2 after 12 months, FU 3 after 18 months, etc
Fig. 3
Fig. 3
Time to ILD progression in months - Kaplan Meier curve by ILD subtypes of special interest. ILD subtypes of special interest included IPF, iNSIP, COP, uILD, sarcoidosis, HP, CTD-ILD, DI-ILD (n = 530). In addition, iNSIP, COP and uILD were grouped
Fig. 4
Fig. 4
Survival time - Kaplan Meier curve (years 0–10) by IPF, PF-ILD, and stable ILD. The definition for PF-ILD was set as absolute decline in FVC % predicted ≥ 10% within a period of 24 months or at least one respiratory hospitalisation
See this image and copyright information in PMC

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