Abstract
Coronavirus disease 2019 (COVID-19) has spread rapidly and become a pandemic. Caused by a novel human coronavirus called severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2), severe COVID-19 is characterized by cytokine storm syndromes due to innate immune activation. Primary immunodeficiency (PID) cases represent a special patient population whose impaired immune system might make them susceptible to severe infections, posing a higher risk to COVID-19, but this could also lead to suppressed inflammatory responses and cytokine storm. It remains an open question as to whether the impaired immune system constitutes a predisposing or protective factor for PID patients when facing SARS-CoV-2 infection. After literature review, it was found that, similar to other patient populations with different comorbidities, PID patients may be susceptible to SARS-CoV-2 infection. Their varied immune status, however, may lead to different disease severity and outcomes after SARS-CoV-2 infection. PID patients with deficiency in antiviral innate immune signaling [eg, Toll-like receptor (TLR)3, TLR7, or interferon regulatory factor 7 (IRF7)] or interferon signaling (IFNAR2) may be linked to severe COVID-19. Because of its anti-infection, anti-inflammatory, and immunomodulatory effects, routine intravenous immunoglobulin therapy may provide some protective effects to the PID patients.
Keywords: COVID-19; IVIG; SARS-CoV-2; cytokine release syndrome; innate immunity; primary immunodeficiency.
【저자키워드】 COVID-19, SARS-CoV-2, Innate immunity, Primary Immunodeficiency, IVIG, Cytokine release syndrome, 【초록키워드】 Coronavirus disease 2019, Intravenous immunoglobulin, Cytokine storm, coronavirus, pandemic, therapy, severe COVID-19, Anti-inflammatory, Antiviral, SARS-COV-2 infection, disease severity, Comorbidities, TLR7, immunodeficiency, Primary Immunodeficiency, Cytokine storm syndrome, immune system, immunomodulatory effects, outcome, Spread, Patient, interferon signaling, receptor, patients, protective effect, Immune status, Signaling, innate immune, IFNAR2, Inflammatory response, literature review, deficiency, acute respiratory syndrome, higher risk, interferon regulatory factor, protective factor, severe infections, cytokine release, patient population, innate immune activation, susceptible, characterized, question, suppressed, anti-infection, PID, 【제목키워드】 review, response, role,