Autoimmunity and Freiburg classification in common variable immunodeficiency
DOI:
https://doi.org/10.5281/Keywords:
Freiburg, Primary Immunodeficiencies, Autoimmunity, Autoimmune Diseases, Common Variable ImmunodeficiencyAbstract
Introduction: Up to 25% of patients with common variable immunodeficiency (CVID) debut with autoimmunity, which is related to the Freiburg classification, which is based on flow cytometry.
Objective: to determine the frequency and type of autoimmune diseases and their association with the Freiburg classification in adults with CVID.
Material and methods: A cross-sectional, analytical and observational study was carried out with 33 patients belonging to the Primary Immunodeficiency Clinic of a third level hospital, with a diagnosis of CVID. They were divided into 3 phenotypes according to the Freiburg classification.
Results: Of the 33 patients studied, 66.6% presented autoimmune diseases, 19 of them (86.3%) had cytopenia; 42.1% belonged to Freiburg group Ia, 36.8% to Ib and 21% to phenotype II. In 36.6% of the patients, autoimmune cytopenia were the first manifestation of CVID; and up to 70% of them belong to the Freiburg phenotype Ia (p = 0.086). Patients with autoimmune cytopenia had a lower percentage of isotype-switched memory B cells (p = 0.018), no higher percentage of CD21low B cells (p = 0.226).
Conclusions: Classification by CVID phenotypes allows the identification of the patient’s profile according to the percentage of memory B cells with isotype change, which is useful to intentionally search for non-infectious complications of the disease.
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