Primary biliary cirrhosis natural history, complications and prognosis
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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 Primary biliary cirrhosis include:[1][2][3]
- Cirrhosis
- Hepatocellular carcinoma
- Metabolic bone disease
- Malabsorption
Prognosis
Prognosis is generally good with the mild disease and early treatment with Ursodeoxycholic acid. Factors associated with poor prognosis include: [4] [5][6][7][2][8][9][10]
- Advanced disease
- Co existing autoimmune disorders such as Sicca syndrome, Thyroiditis, and Scleroderma
- Symptomatic disease
- Increased levels of alkaline phosphatase 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
- ↑ Selmi C, Bowlus CL, Gershwin ME, Coppel RL (2011). "Primary biliary cirrhosis". Lancet. 377 (9777): 1600–9. doi:10.1016/S0140-6736(10)61965-4. PMID 21529926.
- ↑ 2.0 2.1 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.
- ↑ Liang Y, Yang Z, Zhong R (2012). "Primary biliary cirrhosis and cancer risk: a systematic review and meta-analysis". Hepatology. 56 (4): 1409–17. doi:10.1002/hep.25788. PMID 22504852.
- ↑ 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.
- ↑ 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.