Common variable immunodeficiency disorder (CVID) is one of the most frequent inborn errors of immunity characterized by decreased immunoglobulin production, impaired specific antibody response, and higher susceptibility to infections along with immune system dysregulation and higher prevalence of non-infectious complications. Despite the markedly impaired antibody production, diseases hallmarked by the presence of autoantibodies, such as autoimmune hemolytic anemia (AIHA) or immune thrombocytopenic purpura (ITP), are among the most commonly diagnosed autoimmune complications in CVID patients.
The mainstay of CVID management is a regular, long-term immunoglobulin replacement therapy (IRT). Importantly, the immunoglobulin solutions used for IRT were shown to contain various specific antibodies and may even be responsible for IRT-associated adverse events, such as self-limiting acute hemolysis triggered by passivelly transmitted antierythrocyte alloantibodies.
Therefore, we initiated a prospective observational trial to determine the prevalence, clinical significance, and origin of the spectrum of autoantibodies in CVID patients on IRT.