Multidimensional Clinical Deterioration in Interstitial Lung Disease: Longitudinal Concordance of Frailty, Patient-Reported Outcomes, and Lung Function in a Multinational European Registry Cohort Study
10 affiliations
- 1European IPF/ILD Registry and Biobank (eurILDreg), Giessen, Germany
- 2Center for Interstitial and Rare Lung Diseases, Department of Internal Medicine, Justus-Liebig-University Giessen, UGMLC, Member of the DZL, Giessen, Germany
- 3Institute of Medical Informatics, Justus-Liebig-University Giessen, Giessen, Germany
- 4Department of Medicine V, LMU University Hospital, LMU Munich, Comprehensive Pneumology Center Munich (CPC-M), Member of the DZL, Munich, Germany
- 5Service de Pneumologie, Centre Constitutif Maladies Pulmonaires Rares, Hôpital Bichat, Université Paris Cité, Paris, France
- 6Regional Referral Centre for Rare Lung Diseases, A.O.U. Policlinico G. Rodolico-San Marco, University of Catania, Catania, Italy
- 7ILD Unit, Pneumology Department, Hospital Universitari de Bellvitge, IDIBELL, Hospitalet de Llobregat, Barcelona, Spain
- 8Agaplesion Lung Clinic Waldhof Elgershausen, Greifenstein, Germany
- 9Institute for Lung Health, Justus-Liebig-University Giessen, Member of the DZL, Giessen, Germany
- 10Cardio-Pulmonary Institute (CPI), Giessen, Germany
Abstract
Introduction: In interstitial lung disease (ILD), progression is tracked along lung physiology, patient-reported outcome measures (PROMs), and frailty, and prior registries report that these dimensions correlate at the group level. Whether deterioration on one axis identifies the same individual patients as another has not been examined.
Methods: In 371 adults from the multinational European ILD Registry (eurILDreg), spanning the full ILD spectrum, baseline-to-last-visit change was classified as decline at the minimal clinically important difference across eight instruments (CFS, MRC dyspnoea, K-BILD, LCQ, EQ-5D-5L, cough VAS, FVC %pred, DLCO %pred). Patient-level agreement was quantified by Cohen’s κ, interpreted direction-sensitively; a pre-specified window-matched analysis was primary, to account for differinginstrument follow-up windows differed (analysis-specific n 105–178). Outcomes (death or lung transplantation) were ascertained in 312 patients (24 events).
Results: At the individual-patient level, frailty, PROMs, and lung function deteriorated in largely different patients: No pair of the eight instruments exceeded κ = 0.33, and the discordance was not explained by measurement timing, diagnostic heterogeneity (persisting within IPF, κ 0.07–0.26), or a single domain – the two ILD PROMs themselves disagreed (K-BILD × LCQ κ = −0.19). Physiological (FVC-defined) progression was independently associated with K-BILD decline (OR 5.03). A spirometry-invisible subgroup declining on K-BILD while FVC remained stable (22% of 134) carried the highest event rate (13.3%).
Conclusion: In ILD, deterioration in frailty, patient-reported outcomes, and lung physiology identified largely non-overlapping populations at the level of the individual patient, with low agreement even within the patient-reported domain. Because the subgroup with declining patient-reported outcomes but preserved spirometry showed the highest event rate, assessment based on any single measure, and on FVC in particular, may not capture all deteriorating patients. These findings support the integration of validated patient-reported outcomes and frailty measures into multidimensional assessment and indicate that prospective validation of multi-axis decline against clinical outcomes should inform future ILD research and the design of progression criteria.
Related
- Incident health-related and social-disruption events, cumulative burden, and depressive symptom risk in adults aged 50 years and older: a multinational longitudinal cohort study
- Influenza-associated acute respiratory deterioration fulfilling acute exacerbation criteria in hospitalized patients with interstitial lung disease: a single-center retrospective cohort study
- Beyond Symptom Deterioration: A Multidimensional Framework for Psychotherapy Safety
- Emotional Improvement Despite General Clinical Deterioration During Multidisciplinary Rehabilitation in Stiff Person Syndrome: A Longitudinal Case Report
- Household energy, tuberculosis, and poverty: which national signals track the rising global burden of interstitial lung disease? A 204-country longitudinal study, 2000–2021, with projections to 2050
BibTeX
@article{tello2026multidimensional,
title = {{Multidimensional Clinical Deterioration in Interstitial Lung Disease: Longitudinal Concordance of Frailty, Patient-Reported Outcomes, and Lung Function in a Multinational European Registry Cohort Study}},
author = {Silke Tello and Anita C. Windhorst and Heva Mustafa and Poornima Mahavadi and Juergen Behr and Bruno Crestani and Carlo Vancheri and Maria Molina Molina and Andreas Guenther and Ekaterina Krauss},
journal = {Respiration},
year = {2026},
month = sep,
doi = {10.1159/res/aepag007},
url = {https://doi.org/10.1159/res/aepag007},
note = {CC BY-NC 4.0},
}
RIS
TY - JOUR
TI - Multidimensional Clinical Deterioration in Interstitial Lung Disease: Longitudinal Concordance of Frailty, Patient-Reported Outcomes, and Lung Function in a Multinational European Registry Cohort Study
AU - Silke Tello
AU - Anita C. Windhorst
AU - Heva Mustafa
AU - Poornima Mahavadi
AU - Juergen Behr
AU - Bruno Crestani
AU - Carlo Vancheri
AU - Maria Molina Molina
AU - Andreas Guenther
AU - Ekaterina Krauss
PY - 2026
DA - 2026/09/09
JO - Respiration
DO - 10.1159/res/aepag007
UR - https://doi.org/10.1159/res/aepag007
AB - Introduction: In interstitial lung disease (ILD), progression is tracked along lung physiology, patient-reported outcome measures (PROMs), and frailty, and prior registries report that these dimensions correlate at the group level. Whether deterioration on one axis identifies the same individual patients as another has not been examined. Methods: In 371 adults from the multinational European ILD Registry (eurILDreg), spanning the full ILD spectrum, baseline-to-last-visit change was classified as decline at the minimal clinically important difference across eight instruments (CFS, MRC dyspnoea, K-BILD, LCQ, EQ-5D-5L, cough VAS, FVC %pred, DLCO %pred). Patient-level agreement was quantified by Cohen’s κ, interpreted direction-sensitively; a pre-specified window-matched analysis was primary, to account for differinginstrument follow-up windows differed (analysis-specific n 105–178). Outcomes (death or lung transplantation) were ascertained in 312 patients (24 events). Results: At the individual-patient level, frailty, PROMs, and lung function deteriorated in largely different patients: No pair of the eight instruments exceeded κ = 0.33, and the discordance was not explained by measurement timing, diagnostic heterogeneity (persisting within IPF, κ 0.07–0.26), or a single domain – the two ILD PROMs themselves disagreed (K-BILD × LCQ κ = −0.19). Physiological (FVC-defined) progression was independently associated with K-BILD decline (OR 5.03). A spirometry-invisible subgroup declining on K-BILD while FVC remained stable (22% of 134) carried the highest event rate (13.3%). Conclusion: In ILD, deterioration in frailty, patient-reported outcomes, and lung physiology identified largely non-overlapping populations at the level of the individual patient, with low agreement even within the patient-reported domain. Because the subgroup with declining patient-reported outcomes but preserved spirometry showed the highest event rate, assessment based on any single measure, and on FVC in particular, may not capture all deteriorating patients. These findings support the integration of validated patient-reported outcomes and frailty measures into multidimensional assessment and indicate that prospective validation of multi-axis decline against clinical outcomes should inform future ILD research and the design of progression criteria.
SN - 0025-7931
SN - 1423-0356
ER -