Disease trajectories in interstitial lung diseases – data from the EXCITING-ILD registry
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 E...
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| Format: | Article |
| Language: | English |
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BMC
2024-03-01
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| Series: | Respiratory Research |
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| Online Access: | https://doi.org/10.1186/s12931-024-02731-3 |
| _version_ | 1797266609983717376 |
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| author | 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 Michael Kreuter |
| author_facet | 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 Michael Kreuter |
| author_sort | Katharina Buschulte |
| collection | DOAJ |
| description | 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. |
| first_indexed | 2024-04-25T01:03:25Z |
| format | Article |
| id | doaj.art-0daf0a7e2eff4281bcf049375a56f4e3 |
| institution | Directory Open Access Journal |
| issn | 1465-993X |
| language | English |
| last_indexed | 2024-04-25T01:03:25Z |
| publishDate | 2024-03-01 |
| publisher | BMC |
| record_format | Article |
| series | Respiratory Research |
| spelling | doaj.art-0daf0a7e2eff4281bcf049375a56f4e32024-03-10T12:20:27ZengBMCRespiratory Research1465-993X2024-03-0125111110.1186/s12931-024-02731-3Disease trajectories in interstitial lung diseases – data from the EXCITING-ILD registryKatharina Buschulte0Hans-Joachim Kabitz1Lars Hagmeyer2Peter Hammerl3Albert Esselmann4Conrad Wiederhold5Dirk Skowasch6Christoph Stolpe7Marcus Joest8Stefan Veitshans9Marc Höffgen10Phillen Maqhuzu11Larissa Schwarzkopf12Andreas Hellmann13Michael Pfeifer14Jürgen Behr15Rainer Karpavicius16Andreas Günther17Markus Polke18Philipp Höger19Vivien Somogyi20Christoph Lederer21Philipp Markart22Michael Kreuter23Center for Interstitial and Rare Lung Diseases, Thoraxklinik, University of Heidelberg, German Center for Lung Research (DZL)Medical Clinic II, Pneumology and Intensive Care Medicine, Klinikum KonstanzClinic of Pneumology and Allergology, Center of Sleep Medicine and Respiratory Care, Hospital Bethanien SolingenChest Clinic ImmenhausenOutpatient center for pulmonologyOutpatient center for pulmonologyDepartment of Medicine II, University Hospital BonnOutpatient center for pulmonologyOutpatient center for pulmonology and allergologyOutpatient center for pulmonologyOutpatient center for pulmonologyInstitute of Health Economics and Healthcare Management, Helmholtz Center Munich GmbH, German Research Center for Environmental Health, German Center for Lung Research (DZL), Comprehensive Pneumology Center Munich (CPCM)Institute of Health Economics and Healthcare Management, Helmholtz Center Munich GmbH, German Research Center for Environmental Health, German Center for Lung Research (DZL), Comprehensive Pneumology Center Munich (CPCM)Outpatient center for pulmonologyMedical Clinic II, University of Regensburg and Klinikum DonaustaufDepartment of Medicine V, Comprehensive Pneumology Center, LMU University Hospital, LMU Munich, German Center for Lung Research (DZL)Patient Support Group Lungenfibrose e.V.Medical Clinic II, University Hospital Giessen, Universities of Giessen and Marburg Lung Center (UGMLC), German Center for Lung Research (DZL)Center for Interstitial and Rare Lung Diseases, Thoraxklinik, University of Heidelberg, German Center for Lung Research (DZL)Center for Interstitial and Rare Lung Diseases, Thoraxklinik, University of Heidelberg, German Center for Lung Research (DZL)Mainz Center for Pulmonary Medicine, Departments of Pneumology, ZfT, Mainz University Medical Center and of Pulmonary Critical Care & Sleep Medicine, Marienhaus Clinic MainzCenter for Interstitial and Rare Lung Diseases, Thoraxklinik, University of Heidelberg, German Center for Lung Research (DZL)Pulmonary and Critical Care Medicine, Agaplesion Evangelisches Krankenhaus MittelhessenMainz Center for Pulmonary Medicine, Departments of Pneumology, ZfT, Mainz University Medical Center and of Pulmonary Critical Care & Sleep Medicine, Marienhaus Clinic MainzAbstract 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.https://doi.org/10.1186/s12931-024-02731-3ILDIPFProgressionMortalityRisk factors |
| spellingShingle | 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 Michael Kreuter Disease trajectories in interstitial lung diseases – data from the EXCITING-ILD registry Respiratory Research ILD IPF Progression Mortality Risk factors |
| title | Disease trajectories in interstitial lung diseases – data from the EXCITING-ILD registry |
| title_full | Disease trajectories in interstitial lung diseases – data from the EXCITING-ILD registry |
| title_fullStr | Disease trajectories in interstitial lung diseases – data from the EXCITING-ILD registry |
| title_full_unstemmed | Disease trajectories in interstitial lung diseases – data from the EXCITING-ILD registry |
| title_short | Disease trajectories in interstitial lung diseases – data from the EXCITING-ILD registry |
| title_sort | disease trajectories in interstitial lung diseases data from the exciting ild registry |
| topic | ILD IPF Progression Mortality Risk factors |
| url | https://doi.org/10.1186/s12931-024-02731-3 |
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