Portal hypertension in familial Mediterranean fever patients

Abstract

IntroductionFamilial Mediterranean fever (FMF) is an autoinflammatory disease characterized by polyserositis and arthritis attacks, which are mediated by increased plasma levels of cytokines. Our hypothesis was that the increase in specific cytokines can also lead to portal hypertension, even in the absence of overt hepatic steatosis. MethodsWe designed a comparative cross-sectional study with 41 patients and 30 healthy individuals to show if there is a relationship between portal hypertension and FMF. B mode ultrasound and Doppler ultrasound were utilized to evaluate liver echogenicity, portal vein diameter, peak portal blood flow velocity, and portal vein flow direction, which are important diagnostic criteria for portal hypertension. ResultsSpleen and liver sizes and portal vein diameters of the FMF patients and the healthy controls were not significantly different. Imaging of 4 patients revealed periportal cuffing and one patient with periportal cuffing also had a fine granular appearance of the liver. The peak portal blood flow velocity of the FMF patients was lower than that of the control group (p<0.007). ConclusionThe FMF group had significantly lower peak portal blood flow velocity than the control group, indicating the existence of portal hypertension. However, the differences between the other findings that correlate with portal hypertension were not significant.