Pruritus (itch) is an important symptom of
primary biliary cirrhosis (PBC), an archetypal cholestatic
liver disease. Cholestatic
pruritus can be a debilitating symptom causing significant deterioration in patients' quality of life. Effective management of
pruritus in PBC involves awareness among clinicians to adequately assess its severity, and treatment with specific
drug therapies in line with current practice guidelines. In PBC,
antipruritic drugs are not universally effective and/or have significant side effects, and despite best efforts with various combinations of drugs, some patients remain significantly symptomatic, eventually opting for invasive or experimental treatments. Therefore, there is a clear unmet need for better alternative treatments for patients with refractory or intractable cholestatic
pruritus. Recent advances in the understanding of pathogenesis of cholestatic
pruritus and
bile acid physiology have raised hopes for novel
therapies, some of which are currently under trial. In this review, we aim to provide a practical guide to the management of this important and complex problem, discussing current knowledge and recent advances in the pathogenesis, summarise the evidence base for available therapeutic approaches and update potential novel future
therapies for the management of
pruritus in PBC.