Non-cirrhotic portal fibrosis related end stage liver disease in adults: evaluation from a study on living donor liver transplant recipients

Abstract

That non-cirrhotic portal fibrosis (NCPF) can lead to end stage chronic liver disease (CLD) has been convincingly demonstrated only recently after the study of explant livers from clinically cirrhosis cases. This study attempted to determine the frequency of NCPF among adults transplanted for end stage CLD and to identify parameters for a pre-transplant diagnosis of NCPF. Several parameters were analyzed in three categories of cases: pure NCPF (n=10), overlap NCPF (n=10), and NAFLD cirrhosis controls (n=44). Morphologic features of NCPF were looked for in explant livers of all these. Explant livers in the pure NCPF group were non-cirrhotic and showed histologic features of NCPF. These features were also present in all cases of overlap NCPF in the background of established cirrhosis of other etiologies but absent in the NAFLD cirrhosis controls. Values of seven objective and two subjective parameters showed significant differences between pure NCPF and NAFLD control groups. Compared to NAFLD controls, the model for end stage liver disease (MELD) score, body mass index (BMI), bilirubin, albumin, aspartate amino transferase (AST), and international normalized ratio (INR) were significantly less, whereas variceal grade was higher in the pure NCPF group. The study concludes that in our population, NCPF constitutes about 5% of the subset of end stage CLD considered eligible for liver transplantation (LT), presenting mostly as cryptogenic cirrhosis (CC). A diagnosis of NCPF should be considered when patients presumed to have cryptogenic or other cirrhosis become eligible for LT even in the presence of relatively well-preserved liver function and low MELD scores. End stage CLD manifests at earlier age, when cirrhosis of another etiology supervenes on pre-existent NCPF.