Clinical Course and Characteristics of COVID-19 in Patients With Inborn Errors of Immunity: A Retrospective Multicenter Experience From Iran

Abstract Purpose This study aimed to assess the effect of COVID-19 on patients with IEIs, the potentially at-risk population, regarding the clinical course, complications, severity, and outcomes. Methods This two-phase study was conducted on patients from three referral immunodeficiency centers in Iran. At phase one, 98 IEI patients with COVID-19 infection were evaluated by telephone follow-up (TFU). At phase two, the demographic, clinical, and laboratory records of clinically confirmed 33 IEI patients with COVID-19 infection were collected and analyzed. Results At phase one, 16.3% represented COVID-19 infection without any report of pediatric intensive care unit (PICU) admission or death. During the second phase, combined immunodeficiency (CID) (42.4%) and predominantly antibody deficiencies (PADs) (33.3%) were the predominant immune defects. Atopy (27.3%) and lung disorders (27.3%) were the frequent pre-existing comorbidities. Organomegaly (p=0.030) and renal disorders (p=0.033) were significantly associated with the development of respiratory insufficiency. Cyanosis, tachypnea, intercostal retraction, and seizure were the chief complaints of patients who were more likely to progress respiratory insufficiency (p<0.05), being admitted to the PICU (p<0.05), and/or deceased (p<0.05). Laboratory evaluation revealed a marked positive correlation between D-Dimer (p=0.045), prothrombin time (p=0.045), C-reactive protein (p=0.041), proteinuria (p=0.013), ferritin (p=0.020), metabolic acidosis (p=0.003), and troponin (p=0.049) level with mortality. We detected a significant association between the chest X-ray pattern of COVID-19 infection with PICU admission (p=0.023) and death (p=0.046). Conclusion In the current study, patients with CID and PAD were introduced as patients at high risk of COVID-19 infection, who may need extra protective and therapeutic measurements.