Lymphocyte alterations in patients with Common Variable Immunodeficiency (CVID) and autoimmune manifestations.

Clin Immunol

Paediatrics Clinic and Institute for Molecular Medicine A. Nocivelli, Department of Clinical and Experimental Sciences, University of Brescia, ASST- Spedali Civili of Brescia, Brescia, Italy. Electronic address:

Published: August 2022

AI Article Synopsis

  • Autoimmunity is prevalent in Common Variable Immune Deficiency (CVID) patients, but the exact mechanisms behind its development remain unclear.
  • Data from a study involving 122 CVID patients distinguished between those with and without autoimmune conditions, revealing that 45.1% showed signs of autoimmunity, with immune thrombocytopenia being the most frequent manifestation.
  • The study found unique immune profiles in patients with autoimmune cytopenias, indicating potential biomarkers or phenotypic features that differentiate them from other CVID patients.

Article Abstract

Introduction: Autoimmunity is a common feature in CVID patients. To date the mechanisms leading to the development of such complications are not fully elucidated.

Materials And Methods: Data from 122 CVID patients subdivided in three groups based on the absence of autoimmunity (n-AI) or the presence of hematologic autoimmune phenomena (Cy-AI) or non-hematologic autoimmune phenomena (n-Cy-AI) were evaluated.

Results: We identified a total of 128 autoimmune manifestations in 55/122 patients (45.1%). 30/122 (24.6%) patients presented hematologic autoimmune phenomena while 29/122 (23.8%) presented gastrointestinal autoimmune involvement. Immune thrombocytopenia was the most common manifestation (27/122; 22.1%), followed by autoimmune hemolytic anemia (18/122; 14.8%) and autoimmune enteropathy (17/122; 13.9%). Cy-AI patients displayed higher CD4 effector memory and terminally differentiated CD8 cells with lower percentages of naïve and recent thymic emigrants (RTEs) CD4 cells and a significant expansion of the CD19CD21 population.

Conclusions: CVID patients developing autoimmune cytopenias display characteristic immune phenotypic features.

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Source
http://dx.doi.org/10.1016/j.clim.2022.109077DOI Listing

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