Vetter M, Kremer A (2018)
Publication Type: Journal article
Publication year: 2018
Book Volume: 59
Pages Range: 544-550
Journal Issue: 6
DOI: 10.1007/s00108-018-0427-0
BACKGROUND: Patients with primary biliary cholangitis (PBC, formerly primary biliary cirrhosis) and insufficient treatment response or risk factors exhibit a remarkably increased risk for disease progression and associated complications. Furthermore, extrahepatic manifestations may considerably reduce quality of life in affected patients. OBJECTIVES: This article presents an overview on standard therapy with ursodeoxycholic acid (UDCA) and further therapeutic options in patients with insufficient treatment response. In addition, symptom-orientated therapies will be presented in a practical and compact way. METHODS: The current European and German guidelines from 2017 in addition to several research papers and expert opinions are the basis for this review. RESULTS: Every PBC patient should be treated with UDCA life-long. In case of insufficient response to UDCA, obeticholic acid (OCA) has been approved as second line therapy since 2016. Fibrates and budesonide present off-label options for certain patient subpopulations. Pruritus should initially be treated with colestyramine. In case of insufficient efficacy or intolerance, rifampicin represents the most effective off-label option. If fatigue is present, differential diagnoses shall be excluded and coping strategies combined with regular physical activity can have a positive effect. CONCLUSION: UDCA and OCA are effective and approved drugs for treating PBC. Patients with insufficient treatment response or risk factors have to be treated consequently. Due to the improved anti-cholestatic treatment options, therapies to reduce fatigue and pruritus are increasingly important.
APA:
Vetter, M., & Kremer, A. (2018). Primary biliary cholangitis-established and novel therapies. Internist, 59(6), 544-550. https://doi.org/10.1007/s00108-018-0427-0
MLA:
Vetter, Marcel, and Andreas Kremer. "Primary biliary cholangitis-established and novel therapies." Internist 59.6 (2018): 544-550.
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