Primary biliary cirrhosis natural history, complications and prognosis
Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]
|
Primary Biliary Cirrhosis Microchapters |
|
Differentiating Primary Biliary Cirrhosis from other Diseases |
|---|
|
Diagnosis |
|
Treatment |
|
Case Studies |
|
Primary biliary cirrhosis natural history, complications and prognosis On the Web |
|
American Roentgen Ray Society Images of Primary biliary cirrhosis natural history, complications and prognosis |
|
FDA on Primary biliary cirrhosis natural history, complications and prognosis |
|
CDC on Primary biliary cirrhosis natural history, complications and prognosis |
|
Primary biliary cirrhosis natural history, complications and prognosis in the news |
|
Blogs on Primary biliary cirrhosis natural history, complications and prognosis |
Natural History, Complications and Prognosis
Natural History
Stages of primary biliary cirrhosis are as follows:
- Stage 1 - Portal Stage: Normal sized triads; portal inflammation, subtle bile duct damage. Granulomas are often detected in this stage.
- Stage 2 - Periportal Stage: Enlarged triads; periportal fibrosis and/or inflammation. Typically characterized by the finding of a proliferation of small bile ducts.
- Stage 3 - Septal Stage: Active and/or passive fibrous septa.
- Stage 4 - Biliary Cirrhosis: Nodules present; garland or jigsaw pattern.
Complications
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]
References
- ↑ eMedicine > Primary Biliary Cirrhosis: Follow-up Author: Nikolaos T Pyrsopoulos. Coauthor: K Rajender Reddy. Updated: Dec 23, 2009
- ↑ 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.
- ↑ 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.
- ↑ Killenberg & Clavien (2006), p. 429.