Persistent pruritus as a rare and potentially serious manifestation of liver involvement in autosomal dominant polycystic kidney disease.

Abstract

Polycystic liver disease (PCLD) is the most frequent extrarenal manifestation of autosomal dominant polycystic kidney disease (ADPKD), usually asymptomatic. We present a patient with ADPKD who developed cholestatic pruritus due to bile duct compression of multiple liver cysts. Because of severity of pruritus she received treatment with lanreotide to reduce liver volume and she was studied to be included in the liver transplant waiting list. She evolved favourably with medical treatment and she is now asymptomatic. In conclusion, persistent pruritus is a rare but potentially serious manifestation of PCLD, so it should be taken into account in patients with ADPKD.