Recurrent familial prolonged intrahepatic cholestasis of pregnancy associated with chronic liver disease

Four Puerto Rican sisters had recurrent prolonged cholestasis of pregnancy without preexisting or intercurrent hepatic disorders. Available information was reviewed on the course, mechanism, and sequelae of prolonged recurrent cholestasis after 14 pregnancies in the 4 sisters. Etiologic, clinical, laboratory, radiological, and morphological studies of the liver and biliary tract were assessed. Each sister had contraceptive pill-induced pruritus. Prolonged recurrent cholestasis in the eldest sister was followed by cirrhosis and death. The second and third sisters had biopsy evidence of portal triaditis and fibrosis after five and three pregnancies, respectively. Intrahepatic cholestatic cirrhosis was present after three pregnancies in the youngest sister, necessitating an orthotopic liver transplantation; a posttransplantation pregnancy was also associated with prolonged cholestasis. Recurrent prolonged intrahepatic cholestasis of pregnancy was followed by periportal fibrosis or cirrhosis in 4 sisters. This finding suggests that patients with prolonged cholestasis after pregnancy should be followed up for evidence of ongoing liver disease, should be counseled on the potential of recurrence and disease progression in future pregnancies, and should alert family members at risk of possible occurrence of the syndrome.