Abstract
Primary immunodeficiencies (PIDs) are heterogeneous, rare disorders that increase susceptibility to infection and/or immune dysregulation. Individuals with certain PIDs are at high risk of severe or fatal outcomes from SARS-CoV-2 infections (the causative agent of COVID-19), either due to the underlying PID and/or due to the presence of comorbidities such as severe lung and liver disease. Vaccination remains the primary strategy to protect individuals with PID from COVID-19. However, populations with PID exhibit variable vaccine seroresponse rates, antibody titers, and neutralization activity depending on the type of PID and/or COVID-19 vaccine, and consequently, are at an elevated risk of severe disease. In this article, we review the COVID-19 burden in patients with PIDs and focus in-depth on findings from patients with predominantly antibody deficiencies or combined immunodeficiencies. We conclude by providing COVID-19 vaccination recommendations for this population.