Adaptive Immune System Evaluation in Familial Mediterranean Fever: Clinical and Immunological Analysis

Abstract

Background: Familial Mediterranean Fever is a common genetic autoinflammatory disease prevalent in the Mediterranean region. The clinical course of the disease is characterized by fever and serositis attacks. While defects in the innate immune system are known to play a role in the pathogenesis of the disease, the impact of the adaptive immune system remains unclear. Therefore, the main objective of this study is to analyze the adaptive immune system cells in FMF patients and investigate their relationship with the disease. Methods: Our study includes 88 FMF patients with confirmed MEFV gene mutations. The demographic characteristics, clinical symptoms, genetic profiles, treatment methods, and any accompanying diseases of the patients were thoroughly examined. Additionally, lymphocyte subpopulations were analyzed using flow cytometry, and inflammatory markers and immunoglobulin levels were evaluated. Results: Significant differences were observed in the distribution of adaptive immune system cells in FMF patients compared to the healthy reference group. In the analysis of lymphocyte subgroups, levels of CD3, CD4, CD19, CD16+56+, CD3CD4CD45RACD31, CD4+CD45RA+, CD8+CD45RA+, CD19+CD27+IgD+IgM+, CD19+CD27+IgD-IgM-, and CD19+CD38+CD21 were found to be lower compared to healthy individuals. Additionally, CD8, CD19+CD27-IgD+, and CD3/ CD8/TCRGD cells were found to be higher. Moreover, in FMF patients with accompanying diseases, CD3, CD4, and CD19 values were statistically lower (p<0.001). Conclusion: This study reveals that adaptive immune system cells are affected in FMF patients, suggesting their significant role in the disease's pathophysiology. Immunological evaluations should be prioritized in the management of FMF, enabling personalized treatment plans for more effective outcomes. (c) 2025 Codon Publications. Published by Codon Publications.