Objective:

This study sought to evaluate the safety and tolerability of IVIg in individuals with DSRD receiving home-based infusions.

Background:

Down Syndrome Regression Disorder (DSRD) is a rare neuropsychiatric condition affecting otherwise healthy individuals with Down syndrome (DS). Multiple studies on DSRD have revealed that immunotherapy with IVIg is both safe and effective although site of infusion has never been studied.

Design/Methods:

 A single-center, case-control, retrospective chart review evaluating infusion reactions was performed for individuals meeting criteria for DSRD and having IVIg infusions between 2019 and 2024 was performed. Adverse events (AEs) were evaluated for severity and need for alterations in infusion plan. A cohort of individuals receiving home-based infusions was compared to a cohort of individuals receiving infusions at an academic medical center.

Results:

In total, 512 individuals were identified for inclusion but only 162 in the institutional infusion cohort and 153 in the home infusion cohort were met all inclusion criteria. There were no statistically significant differences between cohorts. Individuals receiving home infusions had the same rate of experiencing any adverse event during an infusion (p=0.14, 95%CI: 0.89-2.18) although did have a lower total number of adverse events (p<0.001, 95%CI: 1.18-1.49). Individuals receiving home infusions experienced a lower number of behavioral issues with infusions (p=0.03, 95%CI: 1.07-4.32) and had significantly lower discontinuations of infusions secondary to behavioral issues (p=0.04, 95%CI: 1.04-12.82).

Conclusions:

Rates of AEs and SAEs in those with DSRD were the same regardless of site of infusion. This data may demonstrate lower rates of behavioral issues associated with infusions when the site of administration is home-based.