Observational studyJournal of clinical immunology2026
Bronchiectasis in Inborn Errors of Immunity: Prevalence, Predictors, and Cardiopulmonary Complications in a Genetically Characterized Cohort.
Observational study in Journal of clinical immunology, 2026. The graph could read no effect estimate from its abstract, so it casts no vote on the map. It is linked to trial NCT03394053 (Investigating the Mechanistic Biology of Primary Immunodeficiency Disorders), which is not on this map. Not yet cited in PubMed.
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Investigating the Mechanistic Biology of Primary Immunodeficiency Disorders
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5 authors.
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Abstract
purposeBronchiectasis poses a serious but incompletely defined burden in patients with inborn errors of immunity (IEI). We determined its prevalence, independent predictors, and cardiopulmonary complications in a genetically characterized IEI cohort to inform care in this vulnerable population.
methodsWe conducted a cross-sectional analysis of patients enrolled in a National Institutes of Health prospective IEI protocol (2018-2024) who had undergone whole exome or genome sequencing and chest computed tomography (CT). Bronchiectasis was radiologically confirmed, excluding traction bronchiectasis. Predictors were identified by multivariable logistic regression. Cardiopulmonary outcomes included spirometry, lung volumes, diffusing capacity, six-minute walk distance, pulmonary hypertension by echocardiography, and mortality.
resultsOf 229 enrolled patients, 131 were eligible, representing 31 distinct IEIs. Median age at first chest CT was 20 years (IQR 10-33). The most common CT finding was pulmonary nodules (55%). Bronchiectasis prevalence was 27% (95% CI 20-35%). Independent predictors were older age at first CT (aOR 1.04, 95% CI 1.01-1.08), combined immunodeficiency affecting cellular and humoral immunity (aOR 4.30, 95% CI 1.42-14.54), predominantly antibody deficiencies (aOR 5.83, 95% CI 1.66-22.26), and diseases of immune dysregulation (aOR 7.56, 95% CI 1.07-52.72). Patients with bronchiectasis had greater cardiopulmonary impairment across all measured domains and higher mortality (15% vs. 3%; P = 0.046).
conclusionsBronchiectasis is common across IEI diagnostic classes and carries substantial cardiopulmonary morbidity and excess mortality. Older age at first chest CT and specific IUIS classes, particularly predominantly antibody deficiencies and diseases of immune dysregulation, independently predict bronchiectasis, identifying patients who would benefit most from early pulmonary surveillance. TRIAL PROTOCOL: NCT03394053.
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