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How would you approach the diagnosis and management of a patient with features of portal hypertension but normal HVPG and no cirrhosis on biopsy, in the setting of possible but atypical primary biliary cholangitis, and how would you evaluate for alternative causes of presinusoidal or non-cirrhotic portal hypertension?

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Mednet Member

Hepatology · University of Texas at Austin Dell Medical School

PBC can sometimes present with non-cirrhotic portal hypertension, like nodular regenerative hyperplasia, but your patient's normal HVPG argues against this. Having said that, HVPG is not great for measuring presinusoidal portal hypertension. Given the "atypical" nature of your patient's PBC, I would...

How would you approach the diagnosis and management of a patient with features of portal hypertension but normal HVPG and no cirrhosis on biopsy, in the setting of possible but atypical primary biliary cholangitis, and how would you evaluate for alternative causes of presinusoidal or non-cirrhotic portal hypertension? | Mednet