COVID-19 in patients with primary and secondary immunodeficiency: The United Kingdom experience

Abstract

Background: As of November 2020, severe acute respiratory syndrome coronavirus 2 has resulted in 55 million infections worldwide and more than 1.3 million deaths from coronavirus disease 2019 (COVID-19). Outcomes following severe acute respiratory syndrome coronavirus 2 infection in individuals with primary immunodeficiency (PID) or symptomatic secondary immunodeficiency (SID) remain uncertain.

Objectives: We sought to document the outcomes of individuals with PID or symptomatic SID following COVID-19 in the United Kingdom.

Methods: At the start of the COVID-19 pandemic, the United Kingdom Primary Immunodeficiency Network established a registry of cases to collate the nationwide outcomes of COVID-19 in individuals with PID or symptomatic SID and determine risk factors associated with morbidity and mortality from COVID-19 in these patient groups.

Results: A total of 100 patients had been enrolled by July 1, 2020, 60 with PID, 7 with other inborn errors of immunity including autoinflammatory diseases and C1 inhibitor deficiency, and 33 with symptomatic SID. In individuals with PID, 53.3% (32 of 60) were hospitalized, the infection-fatality ratio was 20.0% (12 of 60), the case-fatality ratio was 31.6% (12 of 38), and the inpatient mortality was 37.5% (12 of 32). Individuals with SID had worse outcomes than those with PID; 75.8% (25 of 33) were hospitalized, the infection-fatality ratio was 33.3% (11 of 33), the case-fatality ratio was 39.2% (11 of 28), and inpatient mortality was 44.0% (11 of 25).

Conclusions: In comparison to the general population, adult patients with PID and symptomatic SID display greater morbidity and mortality from COVID-19. This increased risk must be reflected in public health guidelines to adequately protect vulnerable patients from exposure to the virus.

Keywords: COVID-19; SARS-CoV-2; primary immunodeficiency; secondary immunodeficiency.

Fig 1

Prevalence of common comorbidities in patients with PID hospitalized with COVID-19 (red bars) compared with the general population (blue bars) based on data from the ISARIC study. Data represent the percentage of individuals within each age bracket with comorbidity. Binomial CIs were calculated by Wilson’s method. The overall prevalence of each comorbidity in the general population and in the PID cohort is presented as horizontal dotted lines. Proportions were compared using Fisher exact test. For the purposes of this analysis, the ISARIC categories of “Mild Liver Disease” and “Moderate to Severe Liver Disease” were combined into a single “Liver Disease” and “Diabetes without complications” was combined with “Diabetes with complications” into a single “Diabetes mellitus” category. ISARIC, International Severe Acute Respiratory and emerging Infections Consortium; NS, not significant. ∗P < .05, Fisher exact test.