COVID-19 in patients with inborn errors of immunity - the current state of knowledge, diagnostic and therapeutic dilemmas

Abstract

Patients with inborn errors of immunity (IEI) are at risk of contracting the SARS CoV2 virus, as are all humans. The risk of a serious course of COVID-19 related to the type of IEI concerns patients with combined immunodeficiencies, immune dysregulation syndromes, and certain inborn immunity defects, including type I interferon defects. Adult patients with severe humoral immunodeficiency are also at the increased risk (for example CVID or agammaglobulinemia). The additional risk results from the frequent occurrence of many comorbidities and the treatment used. Vaccination is the most effective way to prevent a severe course of COVID-19. An additional dose is required in the selected patients with IEI. The vaccine response in selected IEI groups may be ineffective. Therefore, the pre-exposure prophylaxis with the use of long-acting monoclonal antibodies is justified. With regard to the Omicron variant, there are no recommendations for the post-exposure prophylaxis - no effective preparations. Treatment of COVID-19 in patients with IEI, especially those at risk of severe disease, should be started not later than 5-10 days after the infection. It is advisable to use in paralel,the antiviral drugs and monoclonal antibodies, effective for the currently dominant variants of the virus. It is important to continue treatment of the inbornerrors of immunity, especially human immunoglobulin replacement therapy.