Pediatric and Adult Inborn Errors of Immunity and COVID-19: A Comparative Study

Abstract

COVID-19 has worse clinical outcomes in inborn errors of immunity (IEI) patients. We aimed to determine COVID-19-related hospitalization/ICU admission/mortality risk in patients with IEI. We included 62 COVID-19 (29 children and 33 adults) in a referral center. F/M ratio was 0.94 with median age, 19 (8 months-64 years) years. 53.2% had primary antibody deficiency. Hospitalization rate was 11/29 in children, 21/33 in adults and 7/11 in patients with combined immunodeficiency diseases. Myalgia was more common in adults compared to children (p = 0.013). Inpatients have more cough compared to outpatients (p = 0.002). D-dimer and ferritin levels were higher in inpatients (p = 0.033 and p = 0.046, respectively). Cough (OR: 6.05; [95% CI: 1.76–20.74], p = 0.004) and immunoglobulin replacement therapy use in IEI (OR: 5.15; [95% CI: 1.46–18.11], p = 0.010) were related to hospitalization risk. Inpatients with intensive care unit (ICU) admission had higher ferritin levels (p = 0.02). 77.4% had at least one comorbidity like pulmonary (45.2%), autoimmune (38.7%), and gastrointestinal diseases (32.3%). ICU admission was high in agammaglobulinemia (40%) and immune dyseregulation (ID) (16.6%). An LRBA deficiency patient experienced MIS-C (Multisystem Inflammatory Syndrome in Children) and another died. Eight patients, four in the present center, received convalescent plasma (X-linked agammaglobulinemia [XLA] [n = 3], autosomal recessive agammaglobulinemia [n = 1], LRBA deficiency [n = 1], and CTLA4 deficiency [n = 1], CVID [n = 1], and STAT1 deficiency [n = 1]). Overall mortality was 6.5%, high in ID (16.6%), none in children. Higher D-dimer and ferritin levels is associated with a higher hospitalization ratio- twice in adults compared to children. Overall mortality (6.5%) was about six times the general population with no mortality in children. A high ICU ratio in agammaglobulinemia, suggesting the importance of mucosal IgA in COVID-19 defense. Convalescent plasma helps shorten hospitalization period in agammaglobulinemia.