EVALUATING BEHAVIORAL PROBLEMS IN PATIENTS WITH PRIMARY IMMUNODEFICIENCY DISORDERS WITHIN THE US IMMUNODEFICIENCY NETWORK REGISTRY

Abstract

Introduction Primary immunodeficiency (PID) encapsulates various disorders with diverse phenotypes. Its burden of disease has the potential to impact the emotional well-being of patients. The purpose of this study is to evaluate the psychosocial impact of PID using data from the US Immunodeficiency Network (USIDNET) national registry. Methods We analyzed 600 patients with PID disorders and categorized them based on the phenotypic classification of the International Union of Immunological Societies (IUIS). Demographics, PID characteristics, and behavioral problems were summarized using median (IQR) or mean (SD) for continuous measures and n (%) for categorical measures. Linear regression, Fisher's Exact test, and logistic regression were done to examine unadjusted and adjusted associations of selected demographics and PID categories with behavioral problems. Results Most patients were male (63.3%) and non-Hispanic white (63.3%). The most common PID was primarily antibody deficiency (49.1%). The mean number of behavioral problems per patient was 0.12 (SD: 0.47) before/at time of PID diagnosis and 0.30 (SD: 0.66) after diagnosis. Approximately 8.5% of patients had at least one behavioral problem before/at time of PID diagnosis, while 20.7% had a behavioral problem after diagnosis. A one-sided upper-tail t-test showed that the average change in number of behavioral problems was significantly greater than zero (p Conclusions There was a significant average increase in the number of behavioral problems after PID diagnosis, illustrating the importance of evaluation and treatment of psychological disorders in the PID population.