Primary biliary cirrhosis natural history, complications and prognosis: Difference between revisions
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===Prognosis=== | ===Prognosis=== | ||
Prognosis is generally good with the mild disease and early treatment with Ursodeoxycholic acid. Factors associated with poor prognosis include: | Prognosis is generally good with the mild disease and early treatment with Ursodeoxycholic acid. Factors associated with poor prognosis include: <ref name="pmid7930469">{{cite journal| author=Mahl TC, Shockcor W, Boyer JL| title=Primary biliary cirrhosis: survival of a large cohort of symptomatic and asymptomatic patients followed for 24 years. | journal=J Hepatol | year= 1994 | volume= 20 | issue= 6 | pages= 707-13 | pmid=7930469 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=7930469 }} </ref> <ref name="pmid9934730">{{cite journal| author=Springer J, Cauch-Dudek K, O'Rourke K, Wanless IR, Heathcote EJ| title=Asymptomatic primary biliary cirrhosis: a study of its natural history and prognosis. | journal=Am J Gastroenterol | year= 1999 | volume= 94 | issue= 1 | pages= 47-53 | pmid=9934730 | doi=10.1111/j.1572-0241.1999.00770.x | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=9934730 }} </ref><ref name="pmid20800924">{{cite journal| author=Jones DE, Al-Rifai A, Frith J, Patanwala I, Newton JL| title=The independent effects of fatigue and UDCA therapy on mortality in primary biliary cirrhosis: results of a 9 year follow-up. | journal=J Hepatol | year= 2010 | volume= 53 | issue= 5 | pages= 911-7 | pmid=20800924 | doi=10.1016/j.jhep.2010.05.026 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=20800924 }} </ref><ref name="pmid25160979">{{cite journal| author=Lammers WJ, van Buuren HR, Hirschfield GM, Janssen HL, Invernizzi P, Mason AL et al.| title=Levels of alkaline phosphatase and bilirubin are surrogate end points of outcomes of patients with primary biliary cirrhosis: an international follow-up study. | journal=Gastroenterology | year= 2014 | volume= 147 | issue= 6 | pages= 1338-49.e5; quiz e15 | pmid=25160979 | doi=10.1053/j.gastro.2014.08.029 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=25160979 }} </ref><ref name="pmid9362353">{{cite journal| author=Jones DE, Metcalf JV, Collier JD, Bassendine MF, James OF| title=Hepatocellular carcinoma in primary biliary cirrhosis and its impact on outcomes. | journal=Hepatology | year= 1997 | volume= 26 | issue= 5 | pages= 1138-42 | pmid=9362353 | doi=10.1002/hep.510260508 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=9362353 }} </ref><ref name="pmid17187436">{{cite journal| author=Nakamura M, Kondo H, Mori T, Komori A, Matsuyama M, Ito M et al.| title=Anti-gp210 and anti-centromere antibodies are different risk factors for the progression of primary biliary cirrhosis. | journal=Hepatology | year= 2007 | volume= 45 | issue= 1 | pages= 118-27 | pmid=17187436 | doi=10.1002/hep.21472 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=17187436 }} </ref><ref name="pmid18930330">{{cite journal| author=Poupon R, Ping C, Chrétien Y, Corpechot C, Chazouillères O, Simon T et al.| title=Genetic factors of susceptibility and of severity in primary biliary cirrhosis. | journal=J Hepatol | year= 2008 | volume= 49 | issue= 6 | pages= 1038-45 | pmid=18930330 | doi=10.1016/j.jhep.2008.07.027 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=18930330 }} </ref><ref name="pmid21703179">{{cite journal| author=Corpechot C, Gaouar F, Chrétien Y, Johanet C, Chazouillères O, Poupon R| title=Smoking as an independent risk factor of liver fibrosis in primary biliary cirrhosis. | journal=J Hepatol | year= 2012 | volume= 56 | issue= 1 | pages= 218-24 | pmid=21703179 | doi=10.1016/j.jhep.2011.03.031 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=21703179 }} </ref> | ||
*Advanced disease | *Advanced disease | ||
*Co existing autoimmune disorders such as Sicca syndrome, thyroiditis, and scleroderma | *Co existing autoimmune disorders such as Sicca syndrome, thyroiditis, and scleroderma |
Revision as of 18:15, 20 October 2017
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief:
Overview
If left untreated, [#]% of patients with [disease name] may progress to develop [manifestation 1], [manifestation 2], and [manifestation 3].
OR
Common complications of [disease name] include [complication 1], [complication 2], and [complication 3].
OR
Prognosis is generally excellent/good/poor, and the 1/5/10-year mortality/survival rate of patients with [disease name] is approximately [#]%.
Natural History, Complications, and Prognosis
Natural History
- The symptoms of (disease name) usually develop in the first/ second/ third decade of life, and start with symptoms such as ___.
- The symptoms of (disease name) typically develop ___ years after exposure to ___.
- If left untreated, [#]% of patients with [disease name] may progress to develop [manifestation 1], [manifestation 2], and [manifestation 3].
Complications
- Common complications of [disease name] include:
- [complication 1]
- [complication 2]
- [complication 3]
Prognosis
Prognosis is generally good with the mild disease and early treatment with Ursodeoxycholic acid. Factors associated with poor prognosis include: [1] [2][3][4][5][6][7][8]
- Advanced disease
- Co existing autoimmune disorders such as Sicca syndrome, thyroiditis, and scleroderma
- Symptomatic disease
- Increased levels of Alkaline PO4ase and bilirubin levels at the time of diagnosis
- Stage III and IV disease on histological examination
- Presence of Antinuclear antibodies( antiGp210, antiSp100)
- Cigarette Smoking
- Genetic polymporphism
References
- ↑ Mahl TC, Shockcor W, Boyer JL (1994). "Primary biliary cirrhosis: survival of a large cohort of symptomatic and asymptomatic patients followed for 24 years". J Hepatol. 20 (6): 707–13. PMID 7930469.
- ↑ Springer J, Cauch-Dudek K, O'Rourke K, Wanless IR, Heathcote EJ (1999). "Asymptomatic primary biliary cirrhosis: a study of its natural history and prognosis". Am J Gastroenterol. 94 (1): 47–53. doi:10.1111/j.1572-0241.1999.00770.x. PMID 9934730.
- ↑ Jones DE, Al-Rifai A, Frith J, Patanwala I, Newton JL (2010). "The independent effects of fatigue and UDCA therapy on mortality in primary biliary cirrhosis: results of a 9 year follow-up". J Hepatol. 53 (5): 911–7. doi:10.1016/j.jhep.2010.05.026. PMID 20800924.
- ↑ Lammers WJ, van Buuren HR, Hirschfield GM, Janssen HL, Invernizzi P, Mason AL; et al. (2014). "Levels of alkaline phosphatase and bilirubin are surrogate end points of outcomes of patients with primary biliary cirrhosis: an international follow-up study". Gastroenterology. 147 (6): 1338–49.e5, quiz e15. doi:10.1053/j.gastro.2014.08.029. PMID 25160979.
- ↑ Jones DE, Metcalf JV, Collier JD, Bassendine MF, James OF (1997). "Hepatocellular carcinoma in primary biliary cirrhosis and its impact on outcomes". Hepatology. 26 (5): 1138–42. doi:10.1002/hep.510260508. PMID 9362353.
- ↑ Nakamura M, Kondo H, Mori T, Komori A, Matsuyama M, Ito M; 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.
- ↑ Poupon R, Ping C, Chrétien Y, Corpechot C, Chazouillères O, Simon T; et al. (2008). "Genetic factors of susceptibility and of severity in primary biliary cirrhosis". J Hepatol. 49 (6): 1038–45. doi:10.1016/j.jhep.2008.07.027. PMID 18930330.
- ↑ Corpechot C, Gaouar F, Chrétien Y, Johanet C, Chazouillères O, Poupon R (2012). "Smoking as an independent risk factor of liver fibrosis in primary biliary cirrhosis". J Hepatol. 56 (1): 218–24. doi:10.1016/j.jhep.2011.03.031. PMID 21703179.