Quality of Life in Patients with Predominant Antibody Deficiency

Abstract

RationaleQuality of life (QOL) is a multidimensional concept that encompasses physical, psychological, and social aspects of well-being. Patients with inborn errors of immunity are at risk for higher morbidity and mortality, and the effect of these complications on QOL requires additional study. MethodsPatients with predominant antibody deficiency (PAD) were asked to voluntarily complete the Centers for Disease Control (CDC) Health-Related Quality of Life (HRQOL)-14 Healthy Days Measure questionnaire at routine visits or at intermittent timepoints, per an IRB-approved protocol. This data was compared to the CDC-initiated Behavioral Risk Factor Surveillance System (BRFSS) (a cross-sectional standardized questionnaire including questions from the CDC-HRQOL-14), using a comparator group from the Boston-area in 2021 (n = 801,582). PAD was defined as mild (hypogammaglobulinemia, IgG subclass deficiency, specific antibody deficiency [SAD]), moderate (CVID), severe (CVID/SAD with autoinflammatory features), or secondary (e.g. B-cell depletion). Mean/median values were used to describe the QOL scores (higher scores associated with lower QOL). Two-proportion Z-test, t-tests, and ANOVA were used for comparisons; with statistical significance set at p < 0.05. Results46 patients with PAD completed the survey between 2020–2022. There were 36 (78%) female respondents, with a mean age of 54 years (standard deviation 14). Patients were subclassified as mild (n = 10), moderate (n = 9), severe (n = 23), or secondary PAD (n = 4). The mean HRQOL-14 score for mild PAD was 106.7 vs. 154 (moderate PAD) vs.149 (severe PAD); p = 0.58. Compared to the CDC-BRFSS data, there was significantly worse “fair or poor” self-rated health status in PAD patients (54.4% vs. 12%; PAD patients vs. controls; p < 0.0001), “mental health not good” for > = 14 days/month (45.7% vs. 14.7%; PAD patients vs. controls; p < 0.0001), and “physical health not good” for >= 14 days/month (26.1% vs. 8% PAD patients vs. controls;p < 0.0001). ConclusionsQOL in patients with PAD was significantly decreased compared to the CDC-BRFSS survey respondents from a similar geographical region. While mean HRQOL-14 scores for mild PAD were lower than moderate/severe PAD, this did not reach statistical significance, likely due to being underpowered. Additional research is needed to evaluate the impact of immune deficiency on QOL and identify areas for intervention to improve patient care.