Prevalence and mortality of chronic extracellular matrix dysregulated diseases: a nationwide register-based study
19 affiliations
- 1Nordic Bioscience, Herlev, Denmark.
- 2Department of Public Health, Section for Health Data Science and AI, University of Copenhagen, Copenhagen, Denmark.
- 3Department of Pharmacy, LEO Foundation Center for Cutaneous Drug Delivery, University of Copenhagen, Copenhagen, Denmark.
- 4Division of Rheumatology, University of Michigan, Ann Arbor, MI, USA.
- 5Department of Gastroenterology, Hepatology and Nutrition, Digestive Diseases Institute, Cleveland Clinic, Cleveland, OH, USA.
- 6Department of Inflammation and Immunity, Lerner Research Institute, Cleveland Clinic Foundation, Cleveland, OH, USA.
- 7Program for Global Translational Inflammatory Bowel Disease, Cleveland Clinic, Cleveland, OH, USA.
- 8Friedrich-Alexander-Universität Erlangen-Nürnberg and Universitätsklinikum Erlangen, 91054, Erlangen, Germany.
- 9Leeds Institute of Rheumatic and Musculoskeletal Medicine, University of Leeds, Leeds, UK.
- 10National Institute for Health and Care Research Leeds Biomedical Research Centre, Leeds, UK.
- 11Department of Cancer Biology, University of Texas MD Anderson Cancer Center, Houston, TX, USA.
- 12Department of Bioengineering, Rice University, Houston, TX, USA.
- 13Department of Electrical and Computer Engineering, Rice University, Houston, TX, USA.
- 14Department of Pathology, University of Texas Medical Branch, Galveston, TX, USA.
- 15Institute for Liver Research, Icahn School of Medicine at Mount Sinai, New York, USA.
- 16Department of Cardiology, Cardiovascular Research Institute Maastricht, University of Maastricht & Maastricht University Medical Centre, Maastricht, Netherlands.
- 17Department of Cardiovascular Sciences, Centre for Vascular and Molecular Biology, KU Leuven, Leuven, Belgium.
- 18Hastings Centre for Pulmonary Research and Division of Pulmonary, Critical Care and Sleep Medicine, Keck School of Medicine, University of Southern California, Los Angeles, CA, USA.
- 19National Heart and Lung Institute, Imperial College London, London, UK.
Abstract
Background: Extracellular matrix (ECM) dysregulation, manifesting as fibrosis or tissue destruction, is a hallmark of many chronic diseases, leading to organ dysfunction and mortality. However, ECM-related diseases are rarely considered as a unified group, and their burden remains poorly defined. We aimed to quantify the burden of ECM-related diseases by estimating prevalence and mortality. Methods: We conducted a register-based study using Danish health registers. Chronic diseases were classified as ECM-related, either fibrotic or tissue destruction diseases, based on ICD-10 codes by field experts. Prevalence was estimated using the Danish National Patient Register, and mortality using the Danish Register of Causes of Death. Analyses were stratified by sex, and key analyses were replicated using the UK Biobank cohort (Application ID 31823). Findings: Among 1701 ICD-10 level-two disease codes, we identified 194 fibrotic diseases and 137 tissue destruction diseases, totalling 331 ECM-related diseases. 19% (1120068/5945094) of the population had a fibrotic disease, 30% (1772148/5945094) a tissue destruction disease, and 38% (2250048/5945094) an ECM-related disease, with higher prevalence among women. Mortality rates recorded as underlying causes were 381, 159 and 540 per 100,000 individuals for fibrotic, tissue destruction, and combined ECM-related diseases, accounting for 40% (22715/56795), 17% (9513/56795) and 57% (32228/56795) of deaths. Mortality was higher when ECM-related diseases were recorded as contributing causes of death. Mortality was higher among men. Interpretation: ECM-related diseases impose a substantial burden through high prevalence and mortality. Recognition of ECM dysregulation as a disease mechanism may inform therapeutic strategies and public health planning. Funding: Innovation Fund Denmark.
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BibTeX
@article{christiansen2026prevalence,
title = {{Prevalence and mortality of chronic extracellular matrix dysregulated diseases: a nationwide register-based study}},
author = {Rosa C Christiansen and Cecilie L Bager and Andrea Heinz and Anne C Bay-Jensen and Diana J Leeming and Dinesh Khanna and Federica Genovese and Florian Rieder and Georg Schett and Joachim H Mortensen and Kim Henriksen and Nicholas Willumsen and Philip G Conaghan and Raghu Kalluri and Scott L Friedman and Signe Holm Nielsen and Signe Larsen and Stephane Heymans and Toby M Maher and Søren Brunak and Morten A Karsdal},
journal = {The Lancet Regional Health. Europe},
year = {2026},
month = oct,
doi = {10.1016/j.lanepe.2026.101901},
url = {https://doi.org/10.1016/j.lanepe.2026.101901},
note = {CC BY-NC},
}
RIS
TY - JOUR
TI - Prevalence and mortality of chronic extracellular matrix dysregulated diseases: a nationwide register-based study
AU - Rosa C Christiansen
AU - Cecilie L Bager
AU - Andrea Heinz
AU - Anne C Bay-Jensen
AU - Diana J Leeming
AU - Dinesh Khanna
AU - Federica Genovese
AU - Florian Rieder
AU - Georg Schett
AU - Joachim H Mortensen
AU - Kim Henriksen
AU - Nicholas Willumsen
AU - Philip G Conaghan
AU - Raghu Kalluri
AU - Scott L Friedman
AU - Signe Holm Nielsen
AU - Signe Larsen
AU - Stephane Heymans
AU - Toby M Maher
AU - Søren Brunak
AU - Morten A Karsdal
PY - 2026
DA - 2026/10/23
JO - The Lancet Regional Health. Europe
DO - 10.1016/j.lanepe.2026.101901
UR - https://doi.org/10.1016/j.lanepe.2026.101901
AB - Background: Extracellular matrix (ECM) dysregulation, manifesting as fibrosis or tissue destruction, is a hallmark of many chronic diseases, leading to organ dysfunction and mortality. However, ECM-related diseases are rarely considered as a unified group, and their burden remains poorly defined. We aimed to quantify the burden of ECM-related diseases by estimating prevalence and mortality. Methods: We conducted a register-based study using Danish health registers. Chronic diseases were classified as ECM-related, either fibrotic or tissue destruction diseases, based on ICD-10 codes by field experts. Prevalence was estimated using the Danish National Patient Register, and mortality using the Danish Register of Causes of Death. Analyses were stratified by sex, and key analyses were replicated using the UK Biobank cohort (Application ID 31823). Findings: Among 1701 ICD-10 level-two disease codes, we identified 194 fibrotic diseases and 137 tissue destruction diseases, totalling 331 ECM-related diseases. 19% (1120068/5945094) of the population had a fibrotic disease, 30% (1772148/5945094) a tissue destruction disease, and 38% (2250048/5945094) an ECM-related disease, with higher prevalence among women. Mortality rates recorded as underlying causes were 381, 159 and 540 per 100,000 individuals for fibrotic, tissue destruction, and combined ECM-related diseases, accounting for 40% (22715/56795), 17% (9513/56795) and 57% (32228/56795) of deaths. Mortality was higher when ECM-related diseases were recorded as contributing causes of death. Mortality was higher among men. Interpretation: ECM-related diseases impose a substantial burden through high prevalence and mortality. Recognition of ECM dysregulation as a disease mechanism may inform therapeutic strategies and public health planning. Funding: Innovation Fund Denmark.
SN - 2666-7762
ER -