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Risk of Pulmonary Diseases in Osteogenesis Imperfecta in Denmark: A Register-Based Cohort Study
Jane Dahl Andersen1, Marie Louise Lyster2, Mette Kathrine Holst3
1Department of Internal Medicine, Lillebaelt Hospital, Kolding, Denmark.
Chest
|September 19, 2024
Summary
Patients with Osteogenesis Imperfecta (OI) face a higher risk of respiratory hospitalizations, particularly older females and younger males. Early diagnosis and treatment of lung complications in OI patients are crucial.
Area of Science:
- Pulmonary Medicine
- Genetics
- Epidemiology
Background:
- Osteogenesis Imperfecta (OI) is a rare genetic disorder affecting collagen type I, a key component of lung tissue.
- Impaired collagen impacts respiratory system integrity and function.
Purpose of the Study:
- To investigate if patients with OI have higher hospital admission rates for pulmonary diseases compared to the general population.
- To assess respiratory disease burden in OI patients.
Main Methods:
- Nationwide, register-based cohort study in Denmark (1995-2018).
- Included 862 OI patients and 4,283 controls.
- Analyzed admissions for asthma, COPD, pneumonia, and medication use (bronchodilators, antibiotics).
Main Results:
- Higher admission rates for respiratory diseases in OI patients compared to controls.
- Highest relative risk in females aged 65+ (IRR 1.91) and males aged 0-18 (IRR 4.92).
- Increased bronchodilator use in OI cohort; no difference in antibiotic use.
Conclusions:
- OI patients exhibit an elevated relative risk of respiratory hospitalizations despite overall low admission rates.
- Highlights the need for prompt diagnosis and management of respiratory issues in individuals with OI.
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