Ailevi Akdeniz Ateşi Hastalarında Otoenflamasyonun İncelenmesi

Abstract

Familial Mediterranean Fever (FMF) is the most common autoinflammatory disease. The aim of this study is to determine the changes of GSDMD, IL-1B, and IL-18 in disease attacks of FMF and to compare their levels in the attack-free periods, healthy controls, and febrile controls without a chronic disease. Patients with a diagnosis of FMF were included in the study during attack and attack-free periods. Healthy children as the control group and febrile children without a chronic disease as the febrile control group were added. Gasdermin-D gene expression was measured by the RT-PCR. Interleukin- 1B and IL-18 levels were quantified by the ELISA. A total of 16 FMF patients, 10 healthy children, and eight children with a febrile infection were included in the study. All but one patients with FMF were carriers of an exon 10 mutation in the MEFV gene. It was found that GSDMD gene expression increased significantly during disease attacks compared to the other groups. The levels of IL-1B and IL-18 were found to be high and at similar levels both at the attack and attack-free periods. Gasdermin-D gene expression showed a significant correlation with acute phase reactants at the disease attack. After colchicine, a significant decrease was found in IL-1B and IL-18 levels. No significant difference was detected in these molecules according to the MEFV genotype. An increase in GSDMD levels was observed in the febrile control group, with viral infections being higher than bacterial infections. Gasdermin-D is critical in the pathogenesis of disease attacks in FMF. Interleukin-1B and IL-18 are high at the attack and between the attacks that they might be an indicator of chronic inflammation. There is no significant relationship between these molecules and the MEFV genotype.