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Inicio Medicina Clínica (English Edition) Portal hypertension in familial Mediterranean fever patients
Journal Information
Vol. 159. Issue 9.
Pages 405-410 (November 2022)
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Vol. 159. Issue 9.
Pages 405-410 (November 2022)
Original article
Portal hypertension in familial Mediterranean fever patients
Hipertensión portal en pacientes con fiebre mediterránea familiar
Bahri Oztasa, Hatice Erenb, Ali Yagiz Aylab, Ali Kocc, Kemal Erold, Adile Irfana, Erkan Caglare, Serdal Ugurlub,
Corresponding author
serdalugurlu@gmail.com

Corresponding author.
a Department of Internal Medicine, Kayseri Training and Education Hospital, Kayseri, Turkey
b Division of Rheumatology, Department of Internal Medicine, Cerrahpasa Medical Faculty, Istanbul, Turkey
c Department of Radiology, Kayseri Training and Education Hospital, Kayseri, Turkey
d Division of Rheumatology, Department of Internal Medicine, Kayseri Training and Education Hospital, Kayseri, Turkey
e Division of Gastroenterology, Department of Internal Medicine, Balikesir Medical Faculty, Balikesir, Turkey
Article information
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Abstract
Introduction

Familial 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.

Methods

We 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.

Results

Spleen 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).

Conclusion

The 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.

Keywords:
Familial Mediterranean fever
Portal hypertension
Fibrosis
TGF-β
Resumen
Introducción

La fiebre mediterránea familiar (FMF) es una enfermedad autoinflamatoria caracterizada por poliserositis y ataques de artritis que están mediados por niveles plasmáticos elevados de citocinas. Nuestra hipótesis fue que el aumento de citocinas específicas también puede conducir a hipertensión portal, incluso en ausencia de esteatosis hepática manifiesta.

Métodos

Diseñamos un estudio transversal comparativo con 41 pacientes con FMF y 30 individuos sanos para mostrar si existe una relación entre la hipertensión portal y la FMF. La ecografía en modo B y la ecografía Doppler se utilizaron para evaluar la ecogenicidad hepática, el diámetro de la vena porta, la velocidad máxima del flujo sanguíneo portal y la dirección del flujo de la vena porta, que son criterios de diagnóstico importantes para la hipertensión portal.

Resultados

El tamaño del bazo y del hígado y los diámetros de la vena porta de los pacientes con FMF y los controles sanos no fueron significativamente diferentes. Las imágenes de 4 pacientes revelaron manguito periportal y un paciente con manguito periportal también tenía un aspecto granular fino del hígado. La velocidad máxima del flujo sanguíneo portal de los pacientes con FMF fue menor que en el grupo control (p<0,007).

Conclusión

El grupo de FMF tuvo una velocidad máxima del flujo sanguíneo portal significativamente menor que el grupo control, lo que indica la existencia de hipertensión portal. Sin embargo, las diferencias entre los otros hallazgos que se correlacionan con la hipertensión portal no fueron significativas.

Palabras clave:
Fiebre mediterránea familiar
Hipertensión portal
Fibrosis
TGF-β

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