Disease trajectories in interstitial lung diseases - data from the EXCITING-ILD registry
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 Epidem...
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| Hauptverfasser: | , , , , , , , , , , , , , , , , , , , , , , , |
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| Dokumenttyp: | Article (Journal) |
| Sprache: | Englisch |
| Veröffentlicht: |
06 March 2024
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| In: |
Respiratory research
Year: 2024, Jahrgang: 25, Pages: 1-11 |
| ISSN: | 1465-993X |
| DOI: | 10.1186/s12931-024-02731-3 |
| Online-Zugang: | Verlag, kostenfrei, Volltext: https://doi.org/10.1186/s12931-024-02731-3
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| Verfasserangaben: | Katharina Buschulte, Hans-Joachim Kabitz, Lars Hagmeyer, Peter Hammerl, Albert Esselmann, Conrad Wiederhold, Dirk Skowasch, Christoph Stolpe, Marcus Joest, Stefan Veitshans, Marc Höffgen, Phillen Maqhuzu, Larissa Schwarzkopf, Andreas Hellmann, Michael Pfeifer, Jürgen Behr, Rainer Karpavicius, Andreas Günther, Markus Polke, Philipp Höger, Vivien Somogyi, Christoph Lederer, Philipp Markart and Michael Kreuter |
MARC
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| 245 | 1 | 0 | |a Disease trajectories in interstitial lung diseases - data from the EXCITING-ILD registry |c Katharina Buschulte, Hans-Joachim Kabitz, Lars Hagmeyer, Peter Hammerl, Albert Esselmann, Conrad Wiederhold, Dirk Skowasch, Christoph Stolpe, Marcus Joest, Stefan Veitshans, Marc Höffgen, Phillen Maqhuzu, Larissa Schwarzkopf, Andreas Hellmann, Michael Pfeifer, Jürgen Behr, Rainer Karpavicius, Andreas Günther, Markus Polke, Philipp Höger, Vivien Somogyi, Christoph Lederer, Philipp Markart and Michael Kreuter |
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| 520 | |a 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. | ||
| 650 | 4 | |a Hospitalization | |
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| 650 | 4 | |a Idiopathic Pulmonary Fibrosis | |
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| 650 | 4 | |a Lung Diseases, Interstitial | |
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