ABSTRACT
Background: Inborn Errors of Immunity (IEI) are a heterogeneous group of diseases which immune defect may be related to a complications by COVID 19. Although there are few studies on the evolution and the real clinical impact of infection by SARS-CoV 2 in this group of patients. The aim of the study was to describe the clinical evolution of COVID-19 in patients with different IEI, in a reference center in Brazil. Method: Retrospective and longitudinal study, with analysis of electronic medical charts of patients with IEI and diagnosis of suspected/ confirmed COVID-19 from March 2020 until now. Results: Eighteen patients (61% male), with a median age of 26.6 years (range 12,3-53,4 y), were included in the study. Four X-linked agammaglobulinemia (XLA), 7 common variable immunodeficiency (CVID), 1 specific antibody deficiency, one Good Syndrome-GS, 2 STAT1-GOF, 2 MSMD and one AT. Ten patients (55,5%) had mild flu syndrome and only one patient was asymptomatic. Hospitalization was necessary for 7 patients (38,8%) due to respiratory complications and 3 (16.6%) deceased (2 XLA and 1 GS). Two patients were reinfected (STAT1-GOF and MSMD), with no need for hospitalizations or long-term complications. One of the XLA patients remains hospitalized, with fever for more than 90 days. Five patients experience coughing and tiredness after more than three months of the disease, one also persists with anosmia. Conclusion: Almost 40% of our sample required hospitalization and 16% died. This rate is worrying and reveals how much immunological competence is required by SARS-CoV-2 as well as the fact that Brazil has increasing death rates from COVID-19. Post-acute COVID-19 syndrome (PASC) has presented in more than 25% of IEI patients infected with SARS-CoV-2.