Risk Factors and Prediction of Long-term Outcome in Primary Biliary Cirrhosis

Abstract

The natural history of the disease varies greatly among individual patients with primary biliary cirrhosis (PBC). Some patients live long without any symptoms while other patients present jaundice and develop hepatic failure in early phases of the disease. Previous studies showed that the natural course of PBC is altered by the use of ursodeoxy cholic acid (UDCA). In this review we discuss variation in the natural course of the disease and it's alteration by UDCA, and risk factors that predict disease progression. Based on clinical observations, there are three types of clinical evolution in PBC: 1) minimal to slow progression over several years; 2) rapid progression to jaundice and hepatic failure, and 3) progression to portal hypertension without developing deep jaundice. Notably, based on our analyses accelerated progression to jaundice and liver failure are reflected by a sustained serologic presence of anti-gp210 antibodies whereas patients with portal hypertension in the absence of jaundice have anti-centromere autoantibodies. These observations highlight the clinical importance of antinuclear antibody analysis in patients with PBC.

Diagnostic and clinical significance of anti-centromere antibodies in primary biliary cirrhosis

“…In line with the previous study, the authors reported that more ACA positive patients developed portal hypertension compared to ACA negative PBC patients (10.5% versus 1.2%, P < 0.05), while anti-gp210 positivity was associated with higher Mayo risk scores and lower serum albumin levels [175]. Considered together, these two studies suggest that ACA positivity may be a risk factor for development of portal hypertension, but not to progression to hepatic failure in patients with long-standing PBC [176]. Also noteworthy is the fact that ACA can be detected before the onset of PBC, and that their titres do not appear to change from positive to negative during the course of the disease, suggesting a role for ACA production in the pathogenesis and progression of the disease [177].…”

The long-term mortality of spontaneous bacterial peritonitis in cirrhotic patients: A 3-year nationwide cohort study

“…When the patients had other diagnostic coding for secondary peritonitis, like appendicitis, hollow organ or biliary tract perforation, ischemic bowel disease, peritoneal dialysis catheter-related peritonitis, as well as those having an additional procedure code for abdominal surgery, they were not included. Because primary biliary cirrhosis is a chronic autoimmune cholestatic liver disorder and is a liver disease of a different mechanism, cases with biliary liver cirrhosis were not included in our study (5,6). In this study, we defined ascites as a patient with the ICD-9-CM diagnosis code 789.5 or received the procedure of paracentesis in hospitalization.…”

Ursodeoxycholic acid: A therapeutic niche in an internist’s practice

“…Никакого статистически значимого влия-ния УДХК на смертность и потребность в трансплантации, а так-же на морфологическое строение печени и выраженность кожно-го зуда обнаружено не было, а действие УДХК было сведено толь-ко к улучшению показателей некоторых неспецифических био-химических маркеров -билирубина, щелочной фосфатазы, трансаминаз [30]. В настоящее время установлено, что эффек-тивность терапии УДХК зависит от клинического фенотипа ПБЦ [31,32]:…”