Investigation of serum SCD40L level in children with famillian mediterranean fever

Objective: Familial Mediterranean Fever (FMF) is a chronic autoinflammatory disease. One of the long-term effects of inflammation is vascular endothelial dysfunction. We aimed to determine soluble CD40 ligand (sCD40L), which is one of the endothelial damage markers, may be a marker in evaluating possible cardiovascular disease risks in FMF. We investigated children with FMF without any comorbidity in order to distinguish the role of sCD40L alone in FMF inflammation. Material and Method: Our study consists 68 children with FMF diagnosed according to the Tel Hashomer criteria were defined as the case group and 65 healthy children were defined as the control group of children who applied to Düzce University Faculty of Medicine Pediatrics Polyclinics, aged 2-18 years without comorbidity. Gender, age, height, weight, BMI, BMI percentile, blood pressure and systemic examinations of all children were evaluated, and hemogram and biochemical parameters were studied in the laboratory of our Hospital and sCD40L levels were investigated by Sandwich ELISA method. The clinical features of the patients, disease severity scores, complete and partial responses to colchicine, subclinical inflammations and MEFV mutation characteristics were also evaluated. Results: There was no statistical difference between hemoglobin, leukocyte, neutrophil, lymphocyte, platelet counts, biochemistry parameters, lipid profiles and atherogenic indexes of the Case and Control Groups, which were statistically similar in terms of gender, age, BMI, BMI percentile. (p>0.05) The mean sCD40L of the Case Group was 250.7 ± 194.9 pg/ml and the Control Group was 155.5 ± 137.7 pg/ml, the sCD40L elevation of the Case Group was statistically significant compared to the Control Group. (p<0.005) The relationship between sCD40L and gender, age, BMI, BMI percentile, age at diagnosis, duration of diagnosis, disease severity scores in the Case Group was investigated, no statistically significant difference was found. (p>0.05) Positive correlation was found in both parameters in the relationship between sCD40L with platelet and platelet lymphocyte ratio (PLR). (p<0.05) The mean sCD40L of patients with complete response to colchicine was found to be 219.7 ± 180.9 pg/ml and with partial response as 320.1 ± 211.3 pg/ml, but there was no statistically significant difference. (p>0.05) The mean sCD40L of FMF patients without subclinical inflammation was found to be 218.0 ± 182.0 pg/ml and with subclinical inflammation as 307 ± 207.0 pg/ml, but there was no statistically significant difference between them. (p>0.05) There was no statistically significant difference in sCD40L between homozygous, compound heterozygous and heterozygous groups according to MEFV mutations of the patients. (p>0.05) While the mean sCD40L of patients with M694V mutation was found to be 336.6 ± 220.1 pg/ml and without M694V mutation as 209.6 ± 169.2 pg/ml, the sCD40L level of patients with M694V mutation was statistically higher than without M694V mutation. (p<0.05) Conclusion: The increase of sCD40L, which is a marker of endothelial dysfunction, in children with Familial Mediterranean Fever and especially in with M694V mutation, causes endothelial dysfunction from early childhood and increases the risk of future cardiovascular disease in FMF patients. Keywords: Familial Mediterranean Fever, Children, Soluble CD40 Ligad, Subclinical Inflammation, Colchicine Response, M694V Mutation, Endothelial Dysfunction