Natural history of primary biliary cirrhosis

Abstract

The natural history of primary biliary cirrhosis (PBC) has improved significantly over the last two decades. Most patients are diagnosed with asymptomatic PBC (a-PBC). The prognosis of a-PBC is usually better than that of symptomatic PBC (s-PBC). Among a-PBC patients, some remain asymptomatic, whereas others progress to s-PBC. The prognosis of s-PBC is still poor and the main cause of death in PBC is liver failure. Other complications, such as esophageal varices and hepatocellular carcinoma, also affect the prognosis of PBC patients. Ursodeoxycholic acid treatment improves the prognosis of PBC patients in the early stage. There seems to be several types of PBC progression.

Ulcerative Colitis Complicated with Primary Biliary Cirrhosis

“…PBC is an autoimmune liver disease characterized by chronic inflammatory destruction and obliteration of the intrahepatic bile ducts, and the infiltration of lymphocytes and plasma cells into the portal tract (3,4). PBC is associated with several extrahepatic autoimmune diseases including Sjögren syndrome, chronic thyroiditis, and rheumatoid arthritis (3,4).…”

“…PBC is associated with several extrahepatic autoimmune diseases including Sjögren syndrome, chronic thyroiditis, and rheumatoid arthritis (3,4). However, an association with inflammatory bowel diseases, including UC or Crohn's disease, is not widely recognized.…”

Early biochemical response to ursodeoxycholic acid predicts symptom development in patients with asymptomatic primary biliary cirrhosis

“…Over the last few decades, the awareness of PBC has increased due to the use of multiphasic biochemical screening as a part of routine examination. More patients with asymptomatic-PBC (a-PBC) are now diagnosed at earlier stages [1,2].…”

A case of primary biliary cirrhosis that complicated with combined hepatocellular and cholangiocellular carcinoma

“…However, in recent years, detection of HCC in patients with PBC has increased and the reported incidence is 0.7-3.6% [1]. There is a high risk of carcinogenesis in cases with histological progression to fibrosis, as with viral liver diseases, but the pathogenesis is unclear.…”