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Portal hypertension in familial Mediterranean fever patients

Bahri Oztas1, Hatice Eren2, Ali Yagiz Ayla2

  • 1Department of Internal Medicine, Kayseri Training and Education Hospital, Kayseri, Turkey.

Medicina Clinica
|March 20, 2022
PubMed
Abstract

Insights

Familial Mediterranean Fever (FMF) patients exhibited lower peak portal blood flow velocity, suggesting portal hypertension. This study explored the link between FMF and portal hypertension, even without fatty liver disease.

Area of Science:

  • Autoinflammatory diseases
  • Gastroenterology
  • Vascular medicine

Background:

  • Familial Mediterranean Fever (FMF) is an autoinflammatory condition causing polyserositis and arthritis via elevated cytokines.
  • The study hypothesizes that elevated cytokines in FMF may induce portal hypertension, independent of hepatic steatosis.

Purpose of the Study:

  • To investigate the relationship between portal hypertension and FMF.
  • To assess diagnostic criteria for portal hypertension in FMF patients.

Main Methods:

  • A comparative cross-sectional study included 41 FMF patients and 30 healthy controls.
  • B-mode and Doppler ultrasound assessed liver echogenicity, portal vein diameter, peak portal blood flow velocity, and flow direction.

Main Results:

  • No significant differences in spleen, liver sizes, or portal vein diameters between groups.
  • Periportal cuffing observed in 4 FMF patients; one also showed fine granular liver appearance.
  • FMF patients had significantly lower peak portal blood flow velocity compared to controls (p<0.007).

Conclusions:

  • Lower peak portal blood flow velocity in FMF patients indicates the presence of portal hypertension.
  • While portal hypertension is suggested, other ultrasound findings related to it were not significantly different between groups.
Keywords:
Familial Mediterranean feverFibrosisFiebre mediterránea familiarHipertensión portalPortal hypertensionTGF-β

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