RESUMO
Pruritus is a common, troublesome symptom in patients with cholestatic liver diseases, especially frequent in intrahepatic cholestasis of pregnancy (ICP) and in primary biliary cholangitis (PBC). Cholestatic associated pruritus can have profound effects on the quality of life. The underlying mechanism is still poorly understood. Severe potential pruritogens have been discussed, such as bile salts, opioids, steroid and lysophosphatidic acid (LPA), but none of these are considered as key mediators. Because of this unraveling pathophysiology the treatment of hepatogenic pruritus often represents a clinical challenge. The EASL guidelines have suggested a step-wise approach, starting with elimination of pruritogens by bile acid sequestrants (cholestyramine), in second line managing the metabolism of pruritogens (rifampicin) and in third-line and fourth- line by modifying the itch perception with µ-opioid antagonist or selective serotonin reuptake inhibitors (SSRI). In treatment-refractory pruritus interruption of the enterohepatic cycle by molecular absorbent recirculating system (MARS), nasobiliairy drainage or experimental therapy such as Ultraviolet B light therapy can be considered. Liver transplantation may be reserved for intractable pruritus. Clinical trials with novel agents are ongoing, potentially providing efficacious options in the future.