Effects of the COVID-19 Pandemic on A Group of Patients with Pathogenic Variant of Cytotoxic T-Lymphocyte Associated Protein 4 (CTLA-4) in a Tertiary Center in Florida

Immune dysregulation and tendency for inflammation are risk factors for severe COVID-19 as described in several autoimmune disorders. Similarly, the clinical course of patients with immune dysregulation secondary to inborn errors of immunity in COVID could also be severe as shown in NFKB1 and NFKB2 deficient patients. However, data is limited on clinical and laboratory features of patients with COVID-19 and/or SARS-CoV-2 immunization and cytotoxic T-lymphocyte associated protein 4 (CTLA-4) deficiency, which is the focus of our study. We performed retrospective chart review and telephone interviews of genetically confirmed CTLA4 deficiency patients for demographic information, clinical history including acute infection, and infectious exposure mitigation, immune phenotype and immune response after SARS-CoV-2 immunization. Antibody response to SARS-CoV-2 were measured by ELISA and photonics ring resonance assay. Our patient cohort included 2 children and 6 adults (median age 23.5 years, range 15-51). All adults have clinical disease and required treatment for hypogammaglobulinemia and immune dysregulation. Patients demonstrated varying levels of exposure mitigation. So far, only one patient was infected—a young adult who had benign course of disease. All adults, and one out of 2 vaccine eligible children, are fully immunized. SARS-CoV2 antibody levels of 2 patients tested thus far (1 post-vaccination, 1 post natural infection) were < 6.25% that of vaccinated healthy controls. During this pandemic, most of our CTLA-4 patients have not experienced COVID-19. Antibody response to the SARS-CoV-2 vaccine and/or infection were suboptimal which can increase their vulnerability. T cell studies are needed to further understand immune responses.