Outcome of SARS-CoV-2 Infection in 121 Patients with Inborn Errors of Immunity: A Cross-Sectional Study

Abstract

Purpose There is still scarce data on SARS-CoV-2 infection in patients with Inborn Errors of Immunity (IEI) and many unresolved questions. We aimed to describe the clinical outcome of SARS-CoV-2 infection in Brazilian IEI patients and identify factors influencing the infection. Methods We did a cross-sectional, multicenter study that included patients of any age affected by IEI and SARS-CoV-2 infection. The variables studied were sex, age, type of IEI, comorbidities (number and type), treatment in use for IEI, clinical manifestations and severity of SARS-CoV-2 infection. Results 121 patients were included: 55.4% female, ages from six months to 74 yo (median age = 25.1 yo). Most patients had predominantly antibody deficiency (n = 53). The infection was mostly asymptomatic (n = 21) and mild (n = 66), and one child had multisystem inflammatory syndrome (MIS-C). We could not observe sex-related susceptibility, and there was a weak correlation between age and severity of infection. The number of comorbidities was higher in severe cases, particularly bronchiectasis and cardiopathy. There were no severe cases in hereditary angioedema patients. Six patients aged 2 to 74 years died, three of them with antibody deficiency. Conclusion The outcome was mild in most patients, but the Case Fatality Ratio was higher than in the general population. However, the type of IEI was not a determining factor for severity, except for complement deficiencies linked to milder COVID-19. The severity of SARS-CoV-2 infection seems to be more related to older age, a higher number of comorbidities and type of comorbidities (bronchiectasis and cardiopathy).

Risk Factors for Severe COVID-19 and Hospital Admission in Patients With Inborn Errors of Immunity - Results From a Multicenter Nationwide Study

“…An asymptomatic course of SARS-CoV-2 infection was reported in only 11% of the patients with IEI ( 16 ). Based on these results, we assumed an important role of IEI itself, which is also supported by other studies ( 15 , 17 , 21 ) and meta-analyses ( 22 ). Additionally, anti-spike SARS-Cov2 monoclonal antibodies and convalescent plasma seem to be safe and possibly effective treatment options for patients with IEI.…”

“…With regard to patients with HAE, they showed neither SARS-CoV-2-associated hospital admission nor death despite previously proposed role of the bradykinin overproduction in the kallikrein-kinin cascade as a possible co-mediator of COVID-19-related pulmonary complications ( 24 – 26 ). However, three cohorts of patients with HAE who contracted COVID-19 did not confirm this hypothesis ( 17 , 27 ). Thus, our observations support these findings.…”

“…Interestingly, children who underwent hematopoietic stem cell transplantation showed a similar risk ratio for severe COVID-19 compared to the general population (12). Despite the progress in understanding how COVID-19 may impact patients with IEI, studies focused on the clinical presentation and outcome of COVID-19 in these patients remain limited, and present ambiguous results (13)(14)(15)(16)(17) that may reflect interpopulation differences. Therefore, we initiated a retrospective multicenter study focused on the clinical presentation and outcome of COVID-19 in patients with IEI.…”

Diagnosis of APS-1 in Two Siblings Following Life-Threatening COVID-19 Pneumonia

“…At the beginning of the COVID-19 pandemic, patients with IEIs were considered to be at risk for severe COVID-19. Recent studies however report that most patients with IEIs had silent infection with SARS-CoV-2 or mild COVID-19, as seen in the general population [ 1 , 2 ]. However, the COVID-19-related mortality rates among IEI patients in global and Brazilian studies were higher than those of the general population [ 1 , 2 ].…”

Vaccination against COVID-19 for patients with primary immunodeficiency and hereditary angioedema: the position paper of the Russian Association of Allergology, Clinical Immunology, and the National Association of Experts in Primary Immunodeficiencies

“…Вакцинации против COVID-19 отводится ключевая роль в прекращении пандемии [5], однако на сегодняшний день нет клинических исследований, позволяющих прогнозировать эффективность/безопасность вакцинации против COVID-19 у пациентов с ПИД. Принимая во внимание повышенный риск тяжёлого течения COVID-19 при некоторых формах ПИД [6], а также накопленный опыт вакцинации пациентов с ПИД от других инфекций [7][8][9][10][11], международные сообщества сформулировали совместное заявление по поводу вакцинации пациентов с ПИД [12].…”