Abstract

The evaluation of the response to vaccination in patients with inborn errors of immunity is a tool to evaluate T-dependent and T-independent antibody residual function of B lymphocytes and it is part of the diagnostic definition for Common Variable Immune Deficiencies. Currently used classifications for Common Variable Immune Deficiencies patients are based on the frequency of B cell subsets, and have been proven as a valid instrument for identification of patients at higher risk of infectious and non-infectious complications. This 6-years period observational study delineated the measurement of specific IgA antibodies induced by a 23-valent pneumococcal polysaccharides vaccine by a standardized ELISA for the quantification of IgA antibodies to all 23 pneumococcal serotypes as an additional prognostic marker in 74 CVID patients. The inability to mount an IgA-mediated response against the pneumococcal polysaccharide antigens or the inability to maintain the antibody response over time identified poor IgA CVID responders with severe immunological impairment, great risk of co-morbidities, and poor prognosis. The division of CVID patient into specific IgA-non responders and IgA-responders discriminated better than other CVID classifications for infectious risk, while it overlapped for non-infectious complications. Our study suggested to add the evaluation of the antibody response by the 23-valent IgA assay in the clinical monitoring of CVID patients.

Highlights

  • Immunoglobulin serum levels and antigen-specific antibodies are included as diagnostic criteria in the assessment of patient with inborn defects of immunity [1]

  • The kinetics of the IgA response to Pneumovax R showed a peak at 3–4 weeks after vaccination with an increase in pneumococcal serotypes (PnPS)-IgA antibody concentration of 10–12 times

  • The evaluation of the vaccination response in patients with inborn errors of immunity has a further informative role, being a tool to evaluate the residual function of B lymphocytes

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Summary

Introduction

Immunoglobulin serum levels and antigen-specific antibodies are included as diagnostic criteria in the assessment of patient with inborn defects of immunity [1]. Used classifications for CVID patients are based on the frequency of B cell subsets These immunephenotypic classifications, and in particular the studies done by EUROclass group [8] and by the Freiburg group [9], take into consideration the frequency of peripheral B cells, classswitched memory B cells, and of CD21low B cells. These classifications have been proven as a valid instrument for identification of CVID patients at higher risk of infectious and non-infectious complications

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