Clinically oriented look into diagnostics and therapy of primary sclerosing cholangitis. Review of literature
DOI:
https://doi.org/10.12775/QS.2024.24.54844Keywords
Primary Sclerosing Cholangitis, Bile Duct Disease, Pharmacotherapy, Hepatic FailureAbstract
Introduction: Primary sclerosing cholangitis (PSC) is a complex liver disorder marked by multifocal bile duct strictures and dilatations, leading to cholestasis and liver fibrosis. Although the exact cause is unknown, autoimmune mechanisms are strongly suspected.
Aim of the Study: This paper reviews current knowledge on PSC, covering its etiology, prevalence, treatment options, and practical management strategies.
Current State of Knowledge: PSC management focuses on symptom relief and liver transplantation, with limited success in pharmacological treatments. New therapies, including immune modulators and anti-fibrotic agents, show promise but require further research. Liver transplantation remains the most effective treatment, though recurrence risks necessitate ongoing monitoring.
Materials and Methods: This article reviews the current understanding of PSC, including its pathophysiology, diagnostic approaches, clinical features, and management strategies. A comprehensive literature search was conducted using PubMed and Google Scholar, with articles selected based on relevant keywords and evaluated for inclusion in this review.
Conclusions: Effective management of PSC necessitates a multidisciplinary approach involving hepatologists, gastroenterologists, radiologists, and transplant surgeons. Ongoing efforts to better understand PSC’s pathogenesis, refine diagnostic methods, and develop more effective therapies are crucial for improving patient outcomes.
References
Prokopič M, Beuers U. Management of primary sclerosing cholangitis and its complications: an algorithmic approach. Hepatol Int. 2021;15(1):6-20. doi:10.1007/s12072-020-10118-x
Sarcognato S, Sacchi D, Grillo F, et al. Autoimmune biliary diseases: Primary biliary cholangitis and primary sclerosing cholangitis. Pathologica. 2021;113(3):170-184. doi:10.32074/1591-951X-245
Little R, Wine E, Kamath BM, Griffiths AM, Ricciuto A. Gut microbiome in primary sclerosing cholangitis: A review. World J Gastroenterol. 2020;26(21):2768-2780. doi:10.3748/WJG.V26.I21.2768
Tanaka A. IgG4-related sclerosing cholangitis and primary sclerosing cholangitis. Gut Liver. 2019;13(3):300-307. doi:10.5009/gnl18085
Kunovsky L, Dite P, Hornakova L, et al. Differentiating primary sclerosing cholangitis from similar diseases of autoimmune origin. Journal of Gastrointestinal and Liver Diseases. 2021;30(3):398-403. doi:10.15403/jgld-3849
Beheshti-Maal A, Tamimi A, Iravani S, et al. PSC associated inflammatory bowel disease: a distinct entity. Expert Rev Gastroenterol Hepatol. 2022;16(2):129-139. doi:10.1080/17474124.2022.2031979
Rabiee A, Silveira MG. Primary sclerosing cholangitis. Transl Gastroenterol Hepatol. 2021;6. doi:10.21037/TGH-20-266
Karlsen TH, Folseraas T, Thorburn D, Vesterhus M. Primary sclerosing cholangitis – a comprehensive review. J Hepatol. 2017;67(6):1298-1323. doi:10.1016/j.jhep.2017.07.022
Eaton JE, McCauley BM, Atkinson EJ, et al. Variations in primary sclerosing cholangitis across the age spectrum. Journal of Gastroenterology and Hepatology (Australia). 2017;32(10):1763-1768. doi:10.1111/jgh.13774
Sarcognato S, Sacchi D, Grillo F, et al. Autoimmune biliary diseases: Primary biliary cholangitis and primary sclerosing cholangitis. Pathologica. 2021;113(3):170-184. doi:10.32074/1591-951X-245
Wunsch E, Stadnik A, Kruk B, et al. Chronic Fatigue Persists in a Significant Proportion of Female Patients After Transplantation for Primary Sclerosing Cholangitis. Liver Transplantation. 2021;27(7):1032-1040. doi:10.1002/lt.26033
Dyson JK, Beuers U, Jones DEJ, Lohse AW, Hudson M. Primary sclerosing cholangitis. The Lancet. 2018;391(10139):2547-2559. doi:10.1016/S0140-6736(18)30300-3
Da Cunha T, Vaziri H, Wu GY. Primary Sclerosing Cholangitis and Inflammatory Bowel Disease: A Review. J Clin Transl Hepatol. 2022;10(3):531-542. doi:10.14218/JCTH.2021.00344
Rupp C, Mummelthei A, Sauer P, et al. Non-IBD immunological diseases are a risk factor for reduced survival in PSC. Liver International. 2013;33(1):86-93. doi:10.1111/liv.12028
Morgan MA, Khot R, Sundaram KM, et al. Primary sclerosing cholangitis: review for radiologists. Abdominal Radiology. 2023;48(1):136-150. doi:10.1007/s00261-022-03655-6
Ringe KI, Bergquist A, Lenzen H, et al. Clinical features and MRI progression of small duct primary sclerosing cholangitis (PSC). Eur J Radiol. 2020;129. doi:10.1016/j.ejrad.2020.109101
Kozaka K, Sheedy SP, Eaton JE, Venkatesh SK, Heiken JP. Magnetic resonance imaging features of small-duct primary sclerosing cholangitis. Abdominal Radiology. 2020;45(8):2388-2399. doi:10.1007/s00261-020-02572-w
Gohlke F, Lohse AW, Dienes HP, et al. Evidence for an overlap syndrome of autoimmune hepatitis and primary sclerosing cholangitis. J Hepatol. 1996;24:699-705.
Mago S, Wu GY. Primary sclerosing cholangitis and primary biliary cirrhosis overlap syndrome: A review. J Clin Transl Hepatol. 2020;8(3):336-346. doi:10.14218/JCTH.2020.00036
Fung BM, Lindor KD, Tabibian JH. Cancer risk in primary sclerosing cholangitis: Epidemiology, prevention, and surveillance strategies. World J Gastroenterol. 2019;25(6):659-671. doi:10.3748/wjg.v25.i6.659
Ludwig DR, Anderson MA, Itani M, Sharbidre KG, Lalwani N, Paspulati RM. Secondary sclerosing cholangitis: mimics of primary sclerosing cholangitis. Abdominal Radiology. 2023;48(1):151-165. doi:10.1007/s00261-022-03551-z
Lazaridis KN, Larusso NF. The cholangiopathies. Mayo Clin Proc. 2015;90(6):791-800. doi:10.1016/j.mayocp.2015.03.017
Björnsson ES, Kalaitzakis E. Recent advances in the treatment of primary sclerosing cholangitis. Expert Rev Gastroenterol Hepatol. 2021;15(4):413-425. doi:10.1080/17474124.2021.1860751
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Copyright (c) 2024 Tymoteusz Ślósarz, Rafał Kaczorowski, Tomasz Forenc, Aleksandra Pelczarska, Paulina Maria Krupa, Maciej Witkowski, Patryk Mejza, Anna Kaczorowska
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