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.

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

“…In patients with inborn errors of immunity, it should be remembered that there is a possibility of obtaining false negative results more often than in the population of immunocompetent people. [17] It appears that false-negative results may be related to the very low SARS CoV2 load of the nasopharyngeal mucosa in some patients, especially in the early stages of infection. [18] The tests should be repeated several times if the results are negative, but epidemiological and clinical data indicate the possibility of COVID-19.…”

“…e new vaccines containing adjuvants (Novavax) are also introduced, the actual e ectiveness of which in relation to patients with IEI will be assessed in the studies. [16,17,18] Table 1 presents the latest vaccination schedules recommended by the CDC (Centre for Disease Control) and the current Polish recommendations for people with moderate or severe dysfunctions of the immune system. [19, 20,21] IEI patients with severe humoral de ciency, undergoing human immunoglobulin replacement therapy, should be vaccinated with an additional dose in the primary series and booster doses, according to current national recommendations.…”