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PW01-018 – Circulating endothelial biomarkers in FMF


Familial Mediterranean fever (FMF) is a hereditary autoinflammatory disease that affects the populations with certain ethnic backgrounds. It is characterized by self-limiting febrile attacks of polyserositis. In recent years, some studies reported that FMF patients had increased vascular wall alterations and damage which may be another clinical phenotype of the disease.


In the present study, we extensively evaluated biomarkers related with endothelial damage in regularly treated and attack-free FMF patients.


Forty FMF patients and eighteen healthy controls with no known cardiovascular risk factors were included. All patients were receiving regular colchicine treatment and examinations were performed during attack-free periods. Serum samples were used for the determination of high sensitive C-reactive protein (hs-CRP), tissue factor (TF), tissue plasminogen activator (t-PA) and osteoprotegerin (OPG). Plasma samples were used for the determination of asymmetric dimethylarginine (ADMA) and thrombomodulin (TM).


There were 40 FMF patients (21 M and 19 F, 31 [15-58] years) and18 healthy subjects (11 M and 7 F, 35.5 [19-46] years). The median disease duration was 15 (0.6-45) years. Age, sex distribution, waist circumference, body mass index, smoking status and serum lipids were similar between the patients and controls (P > 0.05). The concentrations of high sensitive C-reactive protein (hs-CRP) was significantly higher in FMF patients compared to controls (hs-CRP: 0.78 [0.03-20.2] vs. 0.15 [0.02-4.71], µg/ml, P = 0.03). Asymmetric dimethylarginine (ADMA), osteoprotegerin (OPG) and thrombomodulin (TM) concentrations were significantly lower in the patients’ group compared to those of controls (ADMA: 2.56 [0.84-4.07] vs. 3.26 [0.88-3.63], umol/l, P = 0.04; OPG: 361.5 [50.5-1232] vs. 548.9 [193-1181], pg/ml, P = 0.01; TM: 2.69 [0.92-7.26] vs. 3.59 [2.8-8.3], ng/ml, P = 0.001 respectively). However, von Willebrand factor (vWF), tissue factor (TF) and tissue plasminogen activator (t-PA) levels were similar between the groups (P > 0.05).


In this study we showed that markers related with endothelial injury including ADMA, OPG and TM were significantly down-regulated in FMF patients who were on regular colchicine treatment during attack-free disease state.

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Sari, I., Pamuk, B., Selcuk, S. et al. PW01-018 – Circulating endothelial biomarkers in FMF. Pediatr Rheumatol 11 (Suppl 1), A71 (2013).

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