Primary biliary cirrhosis natural history, complications and prognosis

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Natural History, Complications and Prognosis

Complicatons

Patients with primary biliary cirrhosis have an increased risk of hepatocellular carcinoma.

Prognosis

The serum bilirubin level is an indicator of the prognosis of primary biliary cirrhosis, with levels of 2–6 mg/dL having a mean survival time of 4.1 years, 6–10 mg/dL having 2.1 years and those above 10 mg/dL having a mean survival time of 1.4 years.[1]

Anti-nuclear antibodies appear to be prognostic agents in PBC. Anti-glycoprotein-210 antibodies, and to a lessor degree anti-p62 antibodies correlate with progression toward end stage liver failure. Anti-centromere antibodies correlate with developing portal hypertension.[2] Anti-np62[3] and anti-sp100 are also found in association with PBC.

After liver transplant, the recurrence rate may be as high as 18% at 5 years, and up to 30% at 10 years. There is no consensus on risk factors for recurrence of the disease.[4]

  1. eMedicine > Primary Biliary Cirrhosis: Follow-up Author: Nikolaos T Pyrsopoulos. Coauthor: K Rajender Reddy. Updated: Dec 23, 2009
  2. Nakamura M, Kondo H, Mori T; et al. (2007). "Anti-gp210 and anti-centromere antibodies are different risk factors for the progression of primary biliary cirrhosis". Hepatology. 45 (1): 118–27. doi:10.1002/hep.21472. PMID 17187436.
  3. Nesher G, Margalit R, Ashkenazi YJ (2001). "Anti-nuclear envelope antibodies: Clinical associations". Semin. Arthritis Rheum. 30 (5): 313–20. doi:10.1053/sarh.2001.20266. PMID 11303304.
  4. Killenberg & Clavien (2006), p. 429.