Systemic disease associated with noncirrhotic portal hypertension

Development of portal hypertension most often occurs in the context of liver cirrhosis. However, noncirrhotic portal hypertension (NCPH) can be a complication of different systemic, primarily extrahepatic, diseases. NCPH is defined as a syndrome with a portal‐caval pressure gradient exceeding 5 mm Hg in the absence of cirrhosis.1 This review will focus on NCPH in systemic diseases along with idiopathic NCPH (INCPH). NCPH associated with extrahepatic portal vein obstruction (EHPVO) or portal vein thrombosis will be discussed in accompanying articles.2, 3