Differentiation of Common Variable Immunodeficiency from Igg Deficiency

Publication date: Available online 14 December 2018

Source: The Journal of Allergy and Clinical Immunology: In Practice

Author(s): Charles A. Filion, Sarah Taylor-Black, Paul J. Maglione, Lin Radigan, Charlotte Cunningham-Rundles

Abstract

Background

Common variable immunodeficiency (CVID) and IgG deficiency are two of the more prevalent primary humoral immune defects. Whereas the former is defined by consensus with criteria for quantitative and qualitative antibody defects, the latter is used to describe patients with reduced IgG, who commonly have recurrent sinopulmonary infections but do not fulfill CVID criteria. However, these patients are often given this diagnosis.

Objective

We compared immunological findings and clinical manifestations of two large cohorts of patients with CVID or IgG deficient to better delineate differences between those syndromes.

Methods

We extracted clinical and laboratory data from electronic medical records of patients at our institution who had received International Classification of Disease codes for either CVID, or IgG deficiency. We gathered immunoglobulin levels, lymphocyte sub-population counts, and serological vaccine responses. In some patients, we performed flow cytometry to determine percentages of memory and switched-memory B cells. We compiled and statistically compared clinical data related to infectious manifestations, bronchiectasis, autoimmune diseases, infiltrative inflammatory processes and lymphoid malignancies.

Results

In contrast to IgG deficient patients, we found that CVID patients had lower IgG levels, greater unresponsiveness to most vaccines, lower percentages of memory and isotype switched-memory B cells, and lower CD4 T cell counts. Clinically, CVID patients presented similar rates of sinusitis and pneumonias, but a significantly higher prevalence of bronchiectasis and especially non-infectious complications.

Conclusion

CVID and IgG deficiency do not share the same disease spectrum, the former being associated with immunodysregulative manifestations and markers of a more severe immune defect. These data may allow clinicians to distinguish these conditions and the management differences that these patients pose.

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