This EASL Quiz was developed in collaboration with ERN.

 

A 41-year-old woman with a history of Sjögren syndrome is referred to the hepatology department for persistent thrombocytopenia (platelet count 98 × 10⁹/L). Abdominal ultrasound shows homogeneous liver parenchyma and splenomegaly (15 cm), with a patent 13 mm wide portal vein. Liver stiffness measurement is 6 kPa and spleen stiffness measurement is 30 kPa. Upper gastrointestinal endoscopy reveals small oesophageal varices.

A transjugular liver biopsy (non-fragmented 2 cm length) adequately excludes cirrhosis. Histological examination shows portal venule stenosis involving 35% of portal tracts and regenerative changes in the absence of nodular regenerative hyperplasia. No additional relevant histological abnormalities are identified.

Based on the findings presented, which of the following is the most appropriate diagnosis?