Primary biliary cirrhosis: A 2010 update

Abstract

Primary biliary cirrhosis (PBC) is a chronic inflammatory autoimmune disease that mainly targets the cholangiocytes of the interlobular bile ducts in the liver. The condition primarily affects middle-aged women. Without treatment, PBC generally progresses to cirrhosis and eventually liver failure over a period of 10-20 years. PBC is a rare disease with prevalence of less than 1/2000. PBC is thought to result from a combination of multiple genetic factors and superimposed environmental triggers. The contribution of the genetic predisposition is evidenced by the familial clustering. Several risk factors, including exposure to infectious agents and chemical xenobiotics, have been suggested. Ursodeoxycholic acid (UDCA) is currently the only FDA-approved medical treatment for PBC. When administered at doses of 13-15 mg/kg/day, a majority of patients with PBC have a normal life expectancy without additional therapeutic measures. One out of three patients does not adequately respond to UDCA therapy and may need additional medical therapy and/or liver transplantation. This review summarises current knowledge on the epidemiology, ethiopathogenesis, clinical, and therapeutic aspects of PBC.

Brazilian society of hepatology recommendations for the diagnosis and management of autoimmune diseases of the liver

“…They can undergo experimental therapies such as oral ondansetron (4-24 mg/ day), oral phenobarbital (2-5 mg/kg/day), propofol (10-15 mg iv bolus, followed by 1 mg/kg/hour), lidocaine (100 mg/ day), phototherapy UVB, extracorporeal albumin dialysis, plasmapheresis, nasobiliary drainage and biliary diversion. LT can be regarded as the last step, when all available interventions have been proved to be ineffective (121,128,192,196,198) . LT raises issues of organ allocation priority.…”

“…It is estimated that 40%-80% of the patients with PBC would have fatigue. Half of them will complain of fatigue as their leading symptom of liver disease (128,197,198) . Some authors consider PBC as a systemic disorder, inasmuch as the physiopathology of fatigue was shown to involve degenerative changes in the central nervous system, in domains critical for sleep and autonomic function, and mitochondrial dysfunction leading to increased anaerobic muscle activity.…”

“…Thus, the ANA pattern can both aid detection of AMA and assist in the diagnosis of AMA negative PBC cases. Moreover, these autoantibodies can provide prognostic in formation, since presence of antigp210 and anticentromere centromere has been associated with a more rapid progression to liver failure and portal hypertension (123,128).…”

The Role of the Acute-Phase Proteins in the Development and Progression of Liver Diseases

“…Clinical, biochemical, histopathological, and cholangiographic criteria are used for differential diagnosis (Table 4). AIH is characterized by severe liver damage with a modest or low elevation of alkaline phosphatase level (Oo, 2010 In PBC (Poupon, 2010), inflammation is associated with similar rates of apoptosis and proliferation of biliary cells. Cytolytic T cells are attracted to autoantibody-marked cells by cytokines and chemokines, which also induce the killing of cells via TNF-, CD-40, and Fas receptors.…”

Geoepidemiology and changing mortality in primary biliary cholangitis

“…Primary biliary cholangitis (PBC), formerly called primary biliary cirrhosis, is a chronic cholestatic liver disease, of autoimmune origin, predominantly affecting middle-aged women and that may progress to end-stage liver disease [1]. The first description was reported by Addison and Gull [2] in 1857 in a 42-year-old women who reported having had ''jaundice for 2 years and patches of a light opaque color in the surface of eyelids; after 4 years from the beginning of jaundice the general surface deepened to a mahogany brown.''…”