COVID-19 Vaccination in Patients With Inborn Errors of Immunity Reduces Hospitalization and Critical Care Needs Related to COVID-19: A USIDNET Report

The purpose of this study was to evaluate coronavirus disease 2019 (COVID-19) vaccination frequency, vaccination safety, and examine its effect on outcomes in patients with inborn errors of immunity (IEI).This study included 1245 patients (both children and adults) with IEI from 24 countries.A secure COVID-19 specific registry database was created in conjunction with Cincinnati Children’s Hospital, the US Immunodeficiency Network, and the Clinical Immunology Society. A REDCap database was used to collect the data. Physicians entered clinical, laboratory, and vaccination data on their patients with IEI from January to August 2022. Patients were eligible if they carried an IEI diagnosis and had either or both COVID-19 infection and COVID-19 vaccination.The majority (63.7%) of IEI patients in the registry database had antibody deficiencies with combined immunodeficiency, and syndrome-associated immunodeficiency were also frequent. Almost two-thirds (64.7%) of the patients received at least 1 COVID-19 vaccination, and of the vaccinated patients, 26.7% of them were vaccinated before developing COVID-19 infection. Ninety percent of the vaccinated patients received 2 or more vaccinations. The majority of patients (84%) received mRNA-based vaccines. Two percent of vaccinated patients sought medical care for a vaccine-related complication. Nearly two-thirds (66.1%) of IEI patients were infected by COVID-19 with a variety of outcomes: 19% required hospitalization, 5.7% required ICU care, and 3.4% died. Most deceased patients had multiple comorbidities. Patients with innate immune defects had the highest rates of hospitalization (44%), ICU admission (22%), and death (11%). Patients who received COVID-19 vaccination before infection had lower rates of hospitalization (9.3% versus 24.4%, P < .001), ICU admission (2.8% versus 7.6%, P = .013), and death (2.3% versus 4.3%, ns, P = .20). Odds of hospitalization and ICU admission were significantly increased in patients who did not receive at least 1 COVID-19 vaccination.COVID-19 vaccination is safe, well tolerated and reduces COVID-19 severity in patients with IEI.This study is the largest registry report of outcomes from COVID-19 vaccination and infection in patients with IEI to date. The registry not only includes a large cohort (n = 1245) but it represents a wide variety of IEIs from around the world. The majority of patients had COVID-19 infections and manifested mild symptoms, but 19% required hospitalization, 5.7% required ICU admission, and 3.4% died. The observed COVID-19 death rate in this cohort was higher than the US COVID-19 death rate for the general population (3.4% versus 1.1% - from Johns Hopkins Coronavirus Resource Center). COVID-19 vaccination before infection yielded lower rates of hospitalization (9.3% versus 24.4%, P < .001), ICU admission (2.8% versus 7.6%, P = .013), and death (2.3% versus 4.3%, ns, P = .20). Unvaccinated patients had an increased odds ratio (>3.5) of hospitalization and ICU admission when compared with those with at least 1 vaccination. COVID-19 vaccination was well tolerated by most patients, although 2% of vaccinated patients required medical care for vaccine-related complications. Given the low risk of vaccine-related complications and reduced severity in patients with IEI who received vaccination, COVID-19 vaccination is effective in decreasing serious outcomes and should be considered in all patients with IEI. This study was limited by one point in time data entry without ability to update entries later, so it could underreport on repeat COVID-19 infection and outcomes.