Primary biliary cirrhosis natural history, complications and prognosis: Difference between revisions
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{{Primary biliary cirrhosis}} | {{Primary biliary cirrhosis}} | ||
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==Overview== | |||
==Natural History, Complications and Prognosis== | ==Natural History, Complications and Prognosis== | ||
===Natural History=== | ===Natural History=== | ||
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===Complications=== | ===Complications=== | ||
Patients with primary biliary cirrhosis have an increased risk of [[hepatocellular carcinoma]]. | Patients with primary biliary cirrhosis have an increased risk of [[hepatocellular carcinoma]]. | ||
===Prognosis=== | ===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.<ref>[http://emedicine.medscape.com/article/171117-followup eMedicine > Primary Biliary Cirrhosis: Follow-up] Author: Nikolaos T Pyrsopoulos. Coauthor: K Rajender Reddy. Updated: Dec 23, 2009</ref> | 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.<ref>[http://emedicine.medscape.com/article/171117-followup eMedicine > Primary Biliary Cirrhosis: Follow-up] Author: Nikolaos T Pyrsopoulos. Coauthor: K Rajender Reddy. Updated: Dec 23, 2009</ref> | ||
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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.<ref name="k&c-p429">Killenberg & Clavien (2006), p. 429.</ref> | 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.<ref name="k&c-p429">Killenberg & Clavien (2006), p. 429.</ref> | ||
==References== | ==References== | ||
{{reflist|2}} | {{reflist|2}} | ||
[[Category:Gastroenterology]] | [[Category:Gastroenterology]] | ||
[[Category:Hepatology]] | [[Category:Hepatology]] | ||
[[Category:Disease]] | [[Category:Disease]] | ||
[[Category:Needs overview]] | [[Category:Needs overview]] | ||
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Revision as of 18:29, 15 July 2016
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief:
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Overview
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.