• Jane Dahl Andersen, Marie Louise Lyster, Mette Kathrine Holst, Daniel Pilsgaard Henriksen, Anders Christensen, Christian B Laursen, Antonella Forlino, and Lars Folkestad.
• Department of Internal Medicine, Lillebaelt Hospital, Kolding, Denmark.
• Chest. 2024 Sep 17.

BackgroundOsteogenesis imperfecta (OI) is a rare hereditary disease mainly resulting in reduced or altered collagen type I. Collagen type I is a major constituent of the respiratory system, and normal collagen type I is vital for pulmonary tissue function.Research QuestionDo patients with OI have increased admission rates resulting from pulmonary diseases compared with the general population?Study Design And MethodsThis was a register-based, nationwide cohort study, including all patients with OI in Denmark and a reference population. From January 1, 1995, through December 31, 2018, we evaluated the rates of admissions resulting from asthma, COPD, and pneumonia as well as the use of bronchodilator drugs and antibiotics comparing individuals with OI with the reference population.ResultsWe included 862 individuals with OI and 4,283 people from the reference population covering 15,952 and 79,471 person-years of observation, respectively, in the two cohorts. The admissions rate (incidence rate [IR]) was highest in women with OI 65 years of age or older, with 56.3 admissions per 1,000 person-years and 29.4 admissions per 1,000 person-years in the reference population (amounting to an admissions incident rate ratio [IRR] of 1.91 [95% CI, 1.38-2.70]). The highest admission rate in men with OI was found among participants 0 to 18 years of age, with an IR of 30.4 per 1,000 person-years compared with an IR of 7.7 per 1,000 person-years in the reference population (IRR, 4.92 [95% CI, 3.79-6.38]). We found a higher proportion of long-acting and short-acting bronchodilator drug users in the OI cohort, but no increased use of antibiotics.InterpretationOverall, the admission rates for respiratory diseases were low in the OI cohort, but a higher relative risk of hospitalizations resulting from respiratory disease compared with the general population. Timely diagnosis and treatment of respiratory complications in individuals with OI is warranted.Copyright © 2024 The Author(s). Published by Elsevier Inc. All rights reserved.

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