Systemic Disease and Portal Hypertension

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. 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. 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