Pregnancy in primary biliary cirrhosis complicated by portal hypertension: report of a case and review of the literature

Case report A 39 year old woman came to the antenatal clinic for booking at the 12 th week of her fifth pregnancy. She complained of pruritis, particularly over her hands and feet. The pruritis preceded her pregnancy by five months. There was no change in urine or stool colour and her appetite was good. Physical examination showed no abnormality. She had no rash, took no medication, and there was no family history of liver disease. She drank less than 15 units of alcohol per week before her pregnancy and had then become abstinent. She had four previous pregnancies, the first pregnancy being complicated by pre-eclampsia at term, the second and third ending in miscarriages in the first trimester, and the fourth being an uneventful full term pregnancy. Five years previously she had attended her general practitioner with tiredness, nausea, headache, reduced appetite and her stool looking ‘different’. Liver function tests revealed an elevated gamma-glutamyl transferase level at 112 iu/L. Ultrasound examination of her liver was normal. Liver function tests repeated one week later showed no change. As her symptoms had improved by the second consultation, no follow up was thought necessary. Her past medical history included infection with hepatitis A virus, and depression after her second miscarriage. In this pregnancy liver function tests were abnormal (alkaline phosphatase 888 iu/L, alamine aminotransferase 101 iu/L, bilirubin 27 mmol/L). The differential diagnosis included cholestasis of pregnancy, primary biliary cirrhosis, cholelithiasis, primary sclerosing cholangitis and infiltrating diseases of the liver. She had IgG antibodies to the mitochondrial pyruvate dehydrogenase complex. A diagnosis of primary biliary cirrhosis was made. We prescribed ursodeoxycholic acid 250 mg three times daily and cholestyramine 4 g twice daily from twenty weeks of gestation. Her liver function tests during her pregnancy are shown in Fig. 1. Her pruritus persisted, despite the improvement in her liver function tests. Chlorpheniramine 4 mg at night was added, without much benefit. An ultrasound scan of the upper abdomen carried out at 30 weeks of gestation showed splenic varices, suggestive of portal hypertension. In view of the risk of rupture of oesophageal varices during delivery, we advised an elective caesarean section. Her coagulation screen was normal throughout her pregnancy. Vitamin K 10 mg per day was prescribed from the 26 th week of gestation. One week before to her delivery, she received two intramuscular injections of bethamethasone 12 mg, twelve hours apart 1 , and an elective caesarean section was carried out in the 37 th week of pregnancy. A female infant weighing 2.97 kg was born in good condition. She was able to breastfeed normally. There was no change in the woman’s symptoms following her delivery. At the sixth week postpartum her liver function test had deteriorated, and she is receiving continuing care in the department of hepatology.