COVID-19 in complex common variable immunodeficiency patients affected by lung diseases

Abstract

Purpose of reviewIn the general population, the risk of severe COVID-19 is associated with old age, male sex, hypertension, obesity and chronic diseases. Chronic lung diseases are listed as additional risk factors for hospitalization and ICU admission. The purpose of this review is to define whether chronic lung diseases, such as bronchiectasis and interstitial diseases, represent a risk for a severe SARS-CoV-2 infection in patients affected by common variable immunodeficiency (CVID), the most common symptomatic primary antibody defect. Recent findingsCVID patients with SARS-CoV-2 infection have been reported since the beginning of the pandemic with a wide range of clinical presentations ranging from asymptomatic to mild/moderate and severe COVID-19. The meta-analysis of 88 CVID cases described in large cohorts and case reports demonstrated that CVID patients with chronic lung involvement have an increased risk for severe COVID-19 in comparison to CVID without lung diseases (50 vs. 28%, relative risk 1.75, 95% confidence interval 1.04-2.92, P ¼ 0.043). Differently from the general population, age and metabolic comorbidities did not represent a risk factor for severe course in this patient's population.

The Immune Response to SARS-CoV-2 Vaccination: Insights Learned From Adult Patients With Common Variable Immune Deficiency

“…Thus, CVID patients might be partially protected against the dangerous macrophage hyperactivation resulting in cytokine storm. On the contrary, CVID patients with an underlying inflammatory chronic lung disease have a worse COVID-19 prognosis ( 46 ). CVID-associated immune dysregulation is a Th1-mediated inflammatory process driven by the IFN-γ pathway ( 47 ) and by a persistent activation on innate immunity ( 48 ), possibly due to the activation of IFN-γ:STAT1:BAFF axis leading to a dysregulated B cell responses ( 49 ).…”

Short and Long-Term Impact of COVID-19 Infection on Previous Respiratory Diseases

“…This is probably due to a reduction in viral infections motivated by social distancing measures, which this population has taken to the extreme, both due to the fear of suffering severe COVID-19, and the stigmatization that chronic cough entails in many of these patients. 101 Finally, there are publications on patients with bronchiectasis generating diseases, such as common variable immunodeficiency 102 or ciliary dyskinesia, 103 which show a low incidence of COVID-19. In these diseases, the severity of COVID-19 is associated with the concomitant presence of respiratory diseases, such as bronchiectasis or interstitial lung disease.…”

Elevated CD21low B Cell Frequency Is a Marker of Poor Immunity to Pfizer-BioNTech BNT162b2 mRNA Vaccine Against SARS-CoV-2 in Patients with Common Variable Immunodeficiency

“…Patients with CVID exhibit a variable susceptibility to Coronavirus disease 2019 (COVID-19), where those with underlying lung diseases, such as bronchiectasis and interstitial lung disease, have the highest risk [ 8 ]. In contrast, there are several case series that report a more moderate risk [ 9 , 10 ].…”