Intrahepatic cholestasis of pregnancy is the most common pregnancy-related liver disease, manifesting typically during the third trimester of pregnancy with pruritus and elevated serum bile acids. This condition is associated with increased fetal morbidity and mortality, and its pathogenesis is still incompletely understood, but is most likely multifactorial, involving ethnicity, genetics, hormones and environmental factors. Available evidence covering the pathophysiology of both maternal and fetal manifestations, and potential new areas of interest such as microbiota and the environment, have been reviewed, as well as available biomarkers that can be used particularly with regard to genetics, multiomics and the possible use of machine learning algorithms to predict intrahepatic cholestasis of pregnancy. Ursodeoxycholic acid is still the mainstay of therapy with limited alternative options; however, a new class of drugs inhibiting intestinal bile acid transport might be on the horizon. Intrahepatic cholestasis of pregnancy is still not completely understood, warranting a critical appraisal of its epidemiology, pathogenesis, diagnosis and management.
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