Predictive Factors for and Complications of Bronchiectasis in Common Variable Immunodeficiency Disorders

• Published in: Journal of clinical immunology Vol. 42; no. 3; pp. 572 - 581
• Main Authors: Sperlich, Johannes M., Grimbacher, Bodo, Soetedjo, Veronika, Workman, Sarita, Burns, Siobhan O., Lowe, David M., Hurst, John R.
• Format: Journal Article
• Language: English
• Published: New York Springer US 01.04.2022; Springer Nature B.V
• Subjects: Biomedical and Life Sciences; Biomedicine; Bronchiectasis; Bronchiectasis - epidemiology; Bronchiectasis - etiology; Cohort Studies; Common variable immunodeficiency; Common Variable Immunodeficiency - complications; Common Variable Immunodeficiency - epidemiology; Computed tomography; Humans; Immune system; Immunoglobulin G; Immunoglobulin M; Immunoglobulins; Immunology; Infections; Infectious Diseases; Internal Medicine; Medical Microbiology; Original; Original Article; Patients; Quality of Life; Respiratory function; Respiratory tract diseases; Respiratory Tract Infections - complications; Respiratory Tract Infections - epidemiology; Immunoglobulin M; Bronchiectasis; Forced expiratory volume in one second; St George Respiratory Questionnaire; Common variable immunodeficiency disorders
• ISSN: 0271-9142; 1573-2592
• DOI: 10.1007/s10875-022-01206-8

Bronchiectasis is a frequent complication of common variable immunodeficiency disorders (CVID). In a cohort of patients with CVID, we sought to identify predictors of bronchiectasis. Secondly, we sought to describe the impact of bronchiectasis on lung function, infection risk, and quality of life. We conducted an observational cohort study of 110 patients with CVID and an available pulmonary computed tomography scan. The prevalence of bronchiectasis was 53%, with most of these patients (54%) having mild disease. Patients with bronchiectasis had lower median serum immunoglobulin (Ig) concentrations, especially long-term IgM (0 vs 0.25 g/l; p  < 0.01) and pre-treatment IgG (1.3 vs 3.7 g/l; p  < 0.01). CVID patients with bronchiectasis had worse forced expiratory volume in one second (2.10 vs 2.99 l; p  < 0.01) and an annual decline in forced expiratory volume in one second of 25 ml/year (vs 8 ml/year in patients without bronchiectasis; p  = 0.01). Patients with bronchiectasis also reported more annual respiratory tract infections (1.77 vs 1.25 infections/year, p  = 0.04) and a poorer quality of life (26 vs 14 points in the St George’s Respiratory Questionnaire; p  = 0.02). Low serum immunoglobulin M concentration identifies patients at risk for bronchiectasis in CVID and may play a role in pathogenesis. Bronchiectasis is relevant because it is associated with frequent respiratory tract infections, poorer lung function, a greater rate of lung function decline, and a lower quality of life.