#PAGE_PARAMS# #ADS_HEAD_SCRIPTS# #MICRODATA#

Chronic cholestatic liver diseases – Primary biliary cholangitis and Primary sclerosing cholangitis


Authors: Tomáš Fejfar 1;  Tomáš Vaňásek 1;  Petr Hůlek 1,2
Authors‘ workplace: II. interní gastroenterologická klinika LF UK a FN Hradec Králové 1;  Katedra interních oborů LF OU Ostrava 2
Published in: Vnitř Lék 2020; 66(5): 287-300
Category:

Overview

Cholestasis is defined as hepatocyte and cholangiocyte bile excretion failure or failure of bile transport to the duodenum. Primary biliary cholangitis (PBC) and primary sclerosing cholangitis as chronic progressive cholestatic diseases are the common reasons of chronic cholestasis. Altogether with cholestatic laboratory picture the pruritus, liver osteodystrophy and fatigue are associated symptoms in both diseases. All associated symptoms and complications are needed to be diagnosed and treated early. In case of liver cirrhosis complicatons of accompanied portal hypertension should be treated and liver transplantation must be considered in all those patients. Diagnosis of PBC is based on cholestatic laboratory features, animitochondrial antibody positivity or typical histological patern. Most patients are asymptomatic in time of diagnosis. First line therapy is ursodeoxycholic acid. In case of first line therapy failure, the prognosis is unfavourable. In this case, second line therapy must be considered. In case of PSC the diagnosis is based on MRCP finding mainly, laboratory test and liver biopsy in some cases. Progressive inflamatory and fibrosing impairment affecting intrahepatic and extrahepatict biliary ducts and strong association with inflamatory bowel disease, especially ulcerative colitis is typical for PSC. Endoscopic therapy with dilatation of dominant structure is crucial. The effect of pharmacotherapy is still being discussed and ursodeoxycholic acid could be used. During follow up patients are in the risk of bacterial cholangitis and malignant tumor development (cholangiogenic and colorectal carcinoma mainly). In PSC patients the severe pruritus and reccurent bacterial cholangitis could be an indication for the liver transplantation.

Keywords:

diagnostics – biliary cholangitis – cholangitis – cholestasis – sclerosing cholangitis – treatment


Sources

1. Jirsa M, Mareček P, Vítek L. Cholestáza. In: Hůlek P, Urbánek P (eds.) Hepatologie. 3 vydání. Praha: Grada 2018, 166–178.

2. Hirschfield GM, Beuers U, Corpechot C, et al. European Association for the Study of the Liver. EASL Clinical Practice Guidelines: The diagnosis and management of patients with primary biliary cholangitis. J Hepatol 2017; pii: S0168–8278(17)30186–1.

3. Fejfar T, Vaňásek T, Hůlek P, et al. Primární biliární cholangitida – doporučený postup České hepatologické společnosti ČLS JEP pro diagnostiku a léčbu. Gastroenterol Hepatol 2018; 72: 109–118.

4. A Name Change for PBC: Cholangitis replacing Cirrhosis, AASLD (online). (Cit. 20. 3. 2018). Dostupné z: http://www.aasld.org/name‑change‑pbc‑cholangitis‑replacing‑cirrhosis# sthash. hrd6kN6F.dpuf

5. Invernizzi P, Lleo A, Podda M. Interpreting serological tests in diagnosing autoimmune liver diseases. Semin Liver Dis 2007; 27: 161–172.

6. Vergani D, Alvarez F, Bianchi FB, et al. Liver autoimmune serology: a consensus statement from the committee for autoimmune serology of the International Autoimmune Hepatitis Group. J Hepatol 2004; 41: 677–683.

7. Dahlqvist G, Gaouar F, Carrat F, et al. Large‑scale characterization study of patients with antimitochondrial antibodies but nonestablished primary biliary cholangitis. Hepatology 2017; 65: 152–163.

8. Hirschfield GM, Heathcote EJ. Antimitochondrial antibody‑negative primary biliary cirrhosis. Clin Liver Dis 2008; 12: 323–331.

9. Kakuda Y, Harada K, Sawada‑Kitamura S, et al. Evaluation of a new histologic staging and grading system for primary biliary cirrhosis in comparison with classical systems. Hum Pathol 2013; 44: 1107–1117.

10. Carbone M, Mells GF, Pells G, et al. Sex and age are determinants of the clinical phenotype of primary biliary cirrhosis and response to ursodeoxycholic Acid. Gastroenterology 2013; 144: 560–569.

11. Mayo PBC model (online). (Cit.28. 10. 2018). Dostupné z: https://www.mayoclinic.org/ medical‑professionals/ model‑end‑stage‑liver‑disease/ updated‑natural‑history‑model‑for‑primary‑biliary‑cirrhosis

12. Angulo P, Lindor KD, Therneau TM, et al. Utilization of the Mayo risk score in patients with primary biliary cirrhosis receiving ursodeoxycholic acid. Liver 1999; 19: 115–121.

13. Parés A, Caballería L, Rodés J. Excellent long‑term survival in patients with primary biliary cirrhosis and biochemical response to ursodeoxycholic Acid. Gastroenterology 2006; 130: 715–720.

14. Corpechot C, Abenavoli L, Rabahi N, et al. Biochemical response to ursodeoxycholic acid and long‑ term prognosis in primary biliary cirrhosis. Hepatology 2008; 48: 871–877.

15. Kuiper EM, Hansen BE, de Vries RA, et al. Dutch PBC Study Group. Improved prognosis of patients with primary biliary cirrhosis that have a biochemical response to ursodeoxycholic acid. Gastroenterology 2009; 136: 1281–1287.

16. Kumagi T, Guindi M, Fischer S, at al. Baseline Ductopenia and Treatment Response Predict Long‑Term Histological Progression in Primary Biliary Cirrhosis. Am J Gastroenterol 2010; 105: 2186–2194.

17. Lammert C, Juran BD, Schlicht E, et al. Biochemical response to ursodeoxycholic acid predicts survival in a North American cohort of primary biliary cirrhosis patients.J Gastroenterol 2014; 49: 1414–1420.

18. Corpechot C, Chazouillères O, Poupon R. Early primary biliary cirrhosis: biochemical response to treatment and prediction of long‑term outcome. J Hepatol 2011; 55: 1361–1367.

19. Azemoto N, Abe M, Murata Y, et al. Early biochemical response to ursodeoxycholic acid predicts symptom development in patients with asymptomatic primary biliary cirrhosis. J Gastroenterol 2009; 44: 630–634.

20. Azemoto N, Kumagi T, Abe M, et al. Biochemical response to ursodeoxycholic acid predicts long‑term outcome in Japanese patients with primary biliary cirrhosis. Hepatol Res 2011; 41: 310–317.

21. Momah N, Silveira MG, Jorgensen R, et al. Optimizing biochemical markers as endpoints for clinical trials in primary biliary cirrhosis. Liver Int 2012; 32: 790–795.

22. Carbone M, Sharp SJ, Flack S, et al. The UKPBC risk scores: Derivation and validation of a scoring system for long‑term prediction of end‑stage liver disease in primary biliary cirrhosis. Hepatology 2016; 63: 930–950.

23. Lammers WJ, Hirschfield GM, Corpechot C. Global PBC Study Group. Development and validation of a scoring system to predict outcomes of patients with primary biliary cirrhosis receiving ursodeoxycholic acid therapy. Gastroenterology 2015; 149: 1804–1812. GLOBE kalkulátor http://www.globalpbc.com/globe

24. Leuschner U, Fischer H, Kurtz W, et al. Ursodeoxycholic acid in primary biliary cirrhosis: results of a controlled double‑blind trial. Gastroenterology 1989; 97: 1268–1274.

25. Juřica J. Ursodeoxycholová kyselina. Remedia 2016; 26: 529–535.

26. Corpechot C, Carrat F, Bonnand AM, at al. The effect of ursodeoxycholic acid therapy on liver fibrosis progression in primary biliary cirrhosis. Hepatology 2000; 32: 1196–1199.

27. Poupon RE, Lindor KD, Cauch‑Dudek K, et al. Combined analysis of randomized controlled trials of ursodeoxycholic acid in primary biliary cirrhosis. Gastroenterology 1997; 113: 884–890.

28. Lindor KD, Dickson ER, Baldus WP, et al. Ursodeoxycholic acid in the treatment of primary biliary cirrhosis. Gastroenterology 1994; 106: 1284–1290.

29. Shi J, Wu C, Lin Y, et al. Long‑term effects of mid‑dose ursodeoxycholic acid in primary biliary cirrhosis: a meta‑analysis of randomized controlled trials. Am J Gastroenterol 2006; 101: 1529–1538.

30. Lammers WJ, van Buuren HR, Hirschfield, et al. Levels of alkaline phosphate and bilirubin are surrogate end points of outcomes of patients with primary biliary cirrhosis: an international follow up study. Gastroenterology 2014; 147: 1338–1349.

31. Hempfling W, Dilger K, Beuers U. Systematic review: ursodeoxycholic acid‑adverse effects and drug interactions. Aliment Pharmacol Ther 2003; 18: 963–972.

32. EASL‑ALEH Clinical Practice Guidelines: Non‑invasive tests for evaluation of liver disease severity and prognosis. J Hepatol 2015; 63: 237–264.

Labels
Diabetology Endocrinology Internal medicine

Article was published in

Internal Medicine

Issue 5

2020 Issue 5

Most read in this issue
Topics Journals
Login
Forgotten password

Enter the email address that you registered with. We will send you instructions on how to set a new password.

Login

Don‘t have an account?  Create new account

#ADS_BOTTOM_SCRIPTS#