Abstract

BackgroundFamilial Mediterranean fever is a genetic autoinflammatory disease most commonly affecting the ethnic groups originating from around the Mediterranean Sea. Apoptosis plays an important role in down-regulation of the inflammatory response by reducing the lifespan of activated immunocompetent cells. Thus, increased apoptosis may be associated with pathogenesis of familial Mediterranean fever.MethodsIn the present study we determined the serum levels of apoptotic marker, Annexin A5, in familial Mediterranean fever patients, within an attack and attack-free, in comparison to healthy subjects and assessed the influence of colchicine treatment on this parameter. In addition, in all study subjects serum levels of C-reactive protein and interleukine-1β, and the total leukocyte count were also determined.ResultsOur results demonstrated that pathogenesis of familial Mediterranean fever is characterized by the increased levels of circulating Annexin A5, which is higher in patients within the attack and which associate with the increased levels of C-reactive protein and interleukine-1β and total leukocyte count.ConclusionsThe results obtained indicate elevated rates of apoptosis of subpopulations of leukocytes involved in autoinflammation and recurrent episodes of fever in familial Mediterranean fever. It was also revealed that regular colchicine treatment sufficiently decreases the rate of apoptosis in familial Mediterranean fever patients by affecting the intensity of autoinflammatory reactions.

Highlights

  • Familial Mediterranean fever (FMF; MIM 294100) is a genetic autoinflammatory disease most commonly affecting the ethnic groups originating from around the Mediterranean Sea, including Armenians, North African Arabs, Sephardic Jews, and Turks

  • According to the results obtained significantly increased serum levels of ANX-V were detected in all FMF patients as compared to controls (M ± SD: 7.88 ± 3.23 vs 1.83 ± 0.53, p < 0.0001), with higher levels in the attack group compared to attack free (M ± SD: 10.64 ± 2.72 vs 5.598 ± 1.11, p < 0.0001)

  • Circulating ANX-V can be released from the cells of the vascular wall, from secretor cells of the spleen and liver or from apoptotic particles derived from injured tissue, monocytes, lymphocytes, etc

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Summary

Introduction

Familial Mediterranean fever (FMF; MIM 294100) is a genetic autoinflammatory disease most commonly affecting the ethnic groups originating from around the Mediterranean Sea, including Armenians, North African Arabs, Sephardic Jews, and Turks. It is the most prevalent hereditary periodic fever worldwide characterized by self-limited recurrent episodes of fever (FMF attack) and is often complicated by amyloidosis. Familial Mediterranean fever is a genetic autoinflammatory disease most commonly affecting the ethnic groups originating from around the Mediterranean Sea. Apoptosis plays an important role in downregulation of the inflammatory response by reducing the lifespan of activated immunocompetent cells. Increased apoptosis may be associated with pathogenesis of familial Mediterranean fever

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