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: Aysha Anwar, M.B.B.S[2], Anmol Pitliya, M.B.B.S. M.D.[3]
Overview
The symptoms of primary biliary cirrhosis usually develop in the fourth and fifth decade of life and start with symptoms such as fatigue and pruritis. If left untreated, patients with primary biliary cirrhosis may progress to develop an advanced stage of liver fibrosis and its subsequent complications such as portal hypertension and liver failure. Patients with untreated primary biliary cirrhosis have an increased the incidence of hepatocellular carcinoma. Prognosis of the disease is generally good with the mild disease and early treatment with ursodeoxycholic acid.
Natural History, Complications, and Prognosis
Natural History
- The symptoms of primary biliary cirrhosis usually develop in the fourth and fifth decade of life and start with symptoms such as fatigue and pruritis.[1][2][3]
- If left untreated, patients with primary biliary cirrhosis may progress to develop advanced stage of liver fibrosis and its subsequent complications such as portal hypertension and liver failure.
- Patients with untreated primary biliary cirrhosis have increased the incidence of hepatocellular carcinoma.
Complications
Prognosis
Prognosis is generally good with the mild disease and early treatment with ursodeoxycholic acid. Factors associated with poor prognosis include: [8][9][10][2][11][12][13]
- 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
- ↑ 1.0 1.1 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 2.2 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.
- ↑ 3.0 3.1 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.
- ↑ Parés A, Guañabens N (2008). "Osteoporosis in primary biliary cirrhosis: pathogenesis and treatment". Clin Liver Dis. 12 (2): 407–24, x. doi:10.1016/j.cld.2008.02.005. PMID 18456188.
- ↑ Phillips JR, Angulo P, Petterson T, Lindor KD (2001). "Fat-soluble vitamin levels in patients with primary biliary cirrhosis". Am J Gastroenterol. 96 (9): 2745–50. doi:10.1111/j.1572-0241.2001.04134.x. PMID 11569705.
- ↑ Lindor KD, Gershwin ME, Poupon R, Kaplan M, Bergasa NV, Heathcote EJ; et al. (2009). "Primary biliary cirrhosis". Hepatology. 50 (1): 291–308. doi:10.1002/hep.22906. PMID 19554543.
- ↑ Blachar, Arye; Federle, Michael P.; Brancatelli, Giuseppe (2001). "Primary Biliary Cirrhosis: Clinical, Pathologic, and Helical CT Findings in 53 Patients". Radiology. 220 (2): 329–336. doi:10.1148/radiology.220.2.r01au36329. ISSN 0033-8419.
- ↑ 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.