Non-cirrhotic portal fibrosis/idiopathic portal hypertension: APASL recommendations for diagnosis and management

Abstract

Since the Asian Pacific Association for the Study of the Liver (APASL) published guidelines on non-cirrhotic portal fibrosis/idiopathic portal hypertension in 2007, there has been a surge in new information, especially with the introduction of the term porto-sinusoidal vascular disorder (PSVD). Non-cirrhotic intra-hepatic causes of portal hypertension include disorders with a clearly identifiable etiology, such as schistosomiasis, as well as disorders with an unclear etiology such as non-cirrhotic portal fibrosis (NCPF), also termed idiopathic portal hypertension (IPH). This entity is being increasingly recognized as being associated with systemic disease and drug therapy, especially cancer therapy. An international working group with extensive expertise in portal hypertension was assigned with formulating consensus guidelines to clarify the definition, diagnosis, histological features, natural history, and management of NCPF/IPH, especially in the context of PSVD. The guidelines were prepared based on evidence from existing published literature. Whenever there was paucity of evidence, expert opinion was included after detailed deliberation. The goal of this manuscript, therefore, is to enhance the current understanding and help create global consensus on the issues surrounding NCPF/IPH.

Keywords: APASL consensus; INCPH; Idiopathic non-cirrhotic portal hypertension; Incomplete septal cirrhosis; NCPF; NCPH; Nodular regenerative hyperplasia; Non-cirrhotic portal hypertension; Obliterative portal venopathy; Portal hypertension; Porto-sinusoidal vascular disease; Variceal bleed.