Systemic Disease and Portal Hypertension

Purpose of Review: The development of portal hypertension is typically a consequence of liver cirrhosis due mainly to primary liver disorders, whereas non-cirrhotic portal hypertension (NCPH) can be a complication of systemic, primarily extrahepatic diseases. Our purpose was to review the various systemic disorders leading to portal hypertension and provide a pathway for diagnosis and management. Recent Findings: Non-cirrhotic portal hypertension is a heterogeneous group of liver disorders primarily of vascular origin that may manifest as portal hypertension. The diagnosis of NCPH in the setting of systemic diseases is challenging and a liver biopsy may be required to confirm the diagnosis. Etiologies include those of vascular origin, autoimmune disorders, drug exposures, and infections. Summary: Complications of portal hypertension in the setting of systemic diseases are similar to patients having cirrhosis and should be addressed similarly while addressing the underlying systemic disorder if possible © The Author(s), under exclusive licence to Springer Science+Business Media, LLC, part of Springer Nature 2024.