title: Idiopathic Non-Cirrhotic Intrahepatic Portal Hypertension (NCIPH)-Newer Insights into Pathogenesis and Emerging Newer Treatment Options.
abstract: Chronic microangiopathy of portal venules results in idiopathic non-cirrhotic intrahepatic portal hypertension (NCIPH). Recent data suggest a role for vasoactive factors of portal venous origin in the pathogenesis of this 'pure' vasculopathy of the liver. Enteropathies (often silent), are an important 'driver' of this disease. NCIPH is under-recognized and often mis-labeled as cryptogenic cirrhosis. Liver biopsy is needed to prove the diagnosis of NCIPH. In these patients, with advancing disease and increased porto-systemic shunting, the portal venous vasoactive factors bypass the liver filter and contribute to the development of pulmonary vascular endothelial disorders-porto-pulmonary hypertension and hepato-pulmonary syndrome as well as mesangiocapillary glomerulonephritis. Prognosis in NCIPH patients is determined by presence, recognition and management of associated disorders. With better understanding of the pathogenesis of NCIPH, newer treatment options are being explored. Imbalance in ADAMTS 13 (a disintegrin and metalloprotease with thrombospondin type 1 motif, member 13): vWF (von-Willebrand factor) ratio is documented in NCIPH patients and may have a pathogenic role. Therapeutic interventions to correct this imbalance may prove to be important in the management of NCIPH.
authors.affiliation: Department of Hepatology, Christian Medical College, Vellore, Tamil Nadu, India.
authors.forename: Ashish
authors.initials: A
authors.lastname: Goel
authors.affiliation: Addenbrooke's Hospital, Cambridge, UK.
authors.forename: Joshua E
authors.initials: JE
authors.lastname: Elias
authors.affiliation: Department of Hepatology, Christian Medical College, Vellore, Tamil Nadu, India.
authors.forename: Chundamannil E
authors.initials: CE
authors.lastname: Eapen
authors.affiliation: Department of Pathology, Christian Medical College, Vellore, Tamil Nadu, India.
authors.forename: Banumathi
authors.initials: B
authors.lastname: Ramakrishna
authors.affiliation: Department of Hepatology, Christian Medical College, Vellore, Tamil Nadu, India ; Liver Unit, University Hospital Birmingham, Birmingham, UK.
authors.forename: Elwyn
authors.initials: E
authors.lastname: Elias
keywords: ADAMTS 13; CVID, common variable immunodeficiency; HPS, hepato-pulmonary syndrome; HVPG, hepatic venous pressure gradient; IBD, inflammatory bowel disease; NCIPH, non-cirrhotic intrahepatic portal hypertension; NRH, nodular regenerative hyperplasia; OPV, obliterative portal venopathy; PPH, porto-pulmonary hypertension; PVT, portal vein thrombosis; SOS, sinusoidal obstruction syndrome; endothelial dysfunction; primary haemostasis; tTG, Tissue transglutaminase; von-Willebrand factor (vWF)