Noncirrhotic portal fibrosis: a rare cause of end-stage liver disease requiring liver transplantation

Abstract

Non-cirrhotic portal fibrosis (NCPF) is a disease of uncertain etiology characterized by periportal fibrosis and involvement of small and medium branches of the portal vein, resulting in the development of portal hypertension [1]. This has been reported from different parts of the world under a variety of names, such as idiopathic portal hypertension, hepatoportal sclerosis, and noncirrhotic intrahepatic portal hypertension, etc. In India, NCPF formed an important group of patients with portal hypertension, and accounted for 15–30% of all such patients in early nineties [2]. However, its incidence has considerably decreased over time and it is now believed to be a vanishing disease in the subcontinent [1]. Improvement in sanitary conditions and decrease in bacterial infections has lead to a decline in the incidence of the disease. Similar drop in the incidence of new cases has been reported from Japan and other countries [1, 3]. How NCPF differs from cirrhosis of liver has generated immense interest over the years, although at times it may not be possible to differentiate NCPF from Child A cirrhosis of liver. Majority of these patients present with features of portal hypertension and well-tolerated variceal bleeding and have good long-term prognosis [1, 4]. A long-term followup study of 59 patients with noncirrhotic intrahepatic portal hypertension in one hospital over 30-year period showed a 5-year survival of 90% and a 30-year survival of 55% [5]. However, a small proportion of patients do behave like endstage liver disease and develop features of decompensation in the form of ascites, jaundice and hepatic encephalopathy. This subgroup of NCPF with bad liver functions has been recognized and constitutes\10% of the patients, who have mild jaundice, mild ascites and low serum albumin [4, 6]. Nodular transformation of the liver with extensive subhepatic and portal fibrosis has been reported in these patients and the condition constitutes a late manifestation of NCPF. Incomplete septal cirrhosis and nodular regenerative hyperplasia may also be associated with NCPF or idiopathic portal hypertension and may be the late manifestation of this disorder [7–10]. These severe histological changes and abnormal liver function have been infrequently reported and require longer follow-up studies to look into this aspect. There are no large studies that have looked at the subgroup of patients who undergo decompensation; however, in some patients with NCPH, the complications of portal hypertension are sufficiently severe to warrant liver transplantation [7, 11–16]. The study in this issue of Hepatology International shows that small fraction of patients (*5%) do develop ascites, jaundice, and hepatic encephalopathy making it difficult to differentiate it from cirrhosis of liver [17]. Another interesting observation of this study is the overlap NCPF group which has features of both NCPF and cirrhosis and was not reported earlier. This was seen in ten patients in which cause of chronic liver disease was hepatitis C in five, hepatitis B in one and nonalcoholic fatty liver disease (NAFLD) in four patients. All ten patients in this group had characteristic portal vein changes. This may be in continuum with the spectrum of NCPF not defined earlier due to absence of serological markers of hepatitis B and C and recent identification of NAFLD as a cause of significant liver disease; although nodular regenerative hyperplasia and incomplete septal cirrhosis have been reported earlier. The existence of two diseases in a single S. Taneja Y. Chawla R. K. Dhiman (&) Department of Hepatology, Postgraduate Institute of Medical Education and Research, Chandigarh 160012, India e-mail: rkpsdhiman@hotmail.com