Pulmonary Computed Tomography Screening Frequency in Primary Antibody Deficiency

The aim of the study was to identify patients with primary antibody deficiency (PAD) at greatest risk for pulmonary complications, which would warrant regular screening with CT.This study included 91 patients (both children and adults) with primary antibody deficiency at a single European center who were routinely screened for pulmonary disease between 2008 and 2021.This was a retrospective, longitudinal cohort study of 91 patients with primary antibody deficiency (eg, X-linked agammaglobulinemia, common variable immunodeficiency [CVID], immunoglobulin subclass deficiency, and/or specific antibody deficiency). Clinical, laboratory, and CT modified Brody-II scores were collected. Univariate analysis and multivariable logistic regressions were used to identify potential risk factors.The top 3 risk factors for progression of airway disease in patients with primary antibody deficiency were (1) a diagnosis of X-linked agammaglobulinemia (XLA), (2) 2.5 or more recurrent airway infections a year, and (3) the presence of airway disease at baseline. Almost 50% of patients with XLA had progression of airway disease, whereas only 21% of other PADs had progression. Higher airway disease scores were associated with chronic cough, decreased forced expiratory volume in 1 second, and median B cell counts ≤ 205. CVID and increased CD4 effector or memory cells were risk factors for the development of interstitial lung disease (ILD). Granulomatous lymphocytic interstitial lung disease was reported only in CVID patients. ILD scores were higher in patients with CVID.Patients with primary antibody deficiency are at increased risk for pulmonary disease. Patients with risk factors should have a routine screening chest CT at least every 5 years, and those deemed low-risk (without risk factors) can be screened every 10 years.Primary antibody deficiency increases risk for recurrent respiratory infections and pulmonary disease. This study identified patients with PAD at greatest risk for pulmonary complications: (1) those with XLA, (2) those with 2.5 or more recurrent airway infections a year, and (3) those with baseline presence of airway disease. The authors suggest that all of these patients should undergo routine screening with chest CT at least every 5 years. Patients with XLA were more likely to have severe airway disease with progression, whereas patients with CVID were more likely to develop ILD. Patients without risk factors can be screened less frequently, probably at least once every 10 years. The greatest limitation to this study was selection bias, as patients with more severe pulmonary disease are likely to be seen and scanned more frequently. The study was also limited by a small sample size (n = 91) with CVID overrepresentation. General pediatricians and subspecialists need to be cognizant of the need for routine pulmonary disease surveillance in all patients (eg, every 5–10 years) with PAD, and frequency can be determined based on the presence of risk factors.