Contraception and Pregnancy in people with primary biliary cholangitis (PBC)

Pregnancy in liver disease

Description

Primary biliary cholangitis (PBC) belongs to the group of autoimmune liver diseases. In PBC the immune system causes “irritation” of the bile ducts in the liver which can lead to scar tissue in the liver and rarely poorer liver function.
Most often the PBC disease can be treated well with ursodeoxycholic acid, in some cases combination with another drug is needed (fibrate or obeticholic acid). If you have PBC, you should get regular check-ups from a liver expert. Please seek advice from your liver doctor before planning your pregnancy.

Conception and contraception in PBC
Menstrual cycle irregularities can occur in patients with autoimmune liver diseases. Well-controlled PBC is the best prerequisite if you want to become pregnant. So far, no lower fertility rates have been noted in women with PCB.

In PBC, contraception is possible using hormones or copper-containing or hormone containing intrauterine device. Hormonal contraception should be used with caution once you have advanced cirrhosis (scarring in the liver).

Please talk to your liver doctor or a gynaecologist about the optimal contraceptive options available to you.

Diagnosis

General information about pregnancy

If you have PBC, you may experience itching during pregnancy (so do some healthy women as well), but the liver function usually remains normal. If you experience start of itching or worsening of itching, you should discuss this with your liver doctor and have liver blood tests to check the level of bile acids in your blood. Mostly, liver blood tests stay stable or improve during pregnancy, but may worsen after pregnancy (in women with PBC). Preterm birth occurs more commonly, and live birth rates are slightly reduced. During pregnancy and after birth liver tests and bile acids will be monitored. Bile acids are associated with reduced gestation length if they rise to higher than 40µmol/L. The unborn baby should be evaluated regularly by antenatal ultrasound.
In some cases, it may be relevant that your liver doctor and obstetrician communicate about pregnancy related issues.

If you have advanced impairment of liver function (cirrhosis)
If you have impaired liver function, it is of extra importance to seek advice from your liver doctor before planning your pregnancy. In case of PBC and especially if you have cirrhosis, ideally, pregnancy should be planned, since complications may be anticipated and prevented with appropriate counselling. People with advanced liver function impairment and its associated complications (such as abdominal fluid (ascites), jaundice and varices of the esophagus) should not be generally advised against pregnancy, but it is vital that you speak to your liver doctor when thinking about starting a family so that you can have the safest pregnancy possible. Advanced liver function impairment (cirrhosis) needs careful assessment and risk stratification. Please ask your liver doctor if he/she recommends additional test such as a gastroscopy prior to pregnancy.

Management

Which medications are possible during pregnancy and breastfeeding?
Most often the ongoing therapy at the onset of pregnancy should be continued. Avoid changing medications if you are pregnant and discuss changes in medication with your liver doctor.

Ursodeoxycholic acid should be continued during pregnancy in primary biliary cholangitis. It is safe in pregnancy and breastfeeding.

Obeticholic acid use is currently not recommended in pregnancy or during lactation in women with primary biliary due to a lack of safety data.

Fibrates may be used after the first trimester if the clinical team believes that the benefits outweigh the risks, however, fibrates are generally not recommended.

For severe itch, the treatments include rifampicin or cholestyramine. Both drugs can be used during pregnancy and lactation.

The safety of drugs used for liver disease in pregnancy and lactation is summarized in the European Association for the Study of the Liver (EASL) Clinical Practice Guidelines on Management of Liver Disease in Pregnancy
(DOI: 10.1016/j.jhep.2023.03.006).

Men with PBC
Men with PBC are allowed to continue their medication during conception and no changes are needed.

Birth
In general, vaginal delivery is preferred where possible and caesarean section should be performed only for obstetric indications.

Authors:
Leona Dold
Catherine Williamson
Henriette Ytting
Willy Visser
Maria Antonella Burza
Wiebke Papenthin and Martine Walmsley, ePAG advocates
On behalf of the ERN RARE-LIVER transversal working group on liver disease in pregnancy

Reference: EASL Clinical Practice Guidelines on the management of liver diseases in pregnancy, Journal of Hepatology. 2023, https://doi.org/10.1016/j.jhep.2023.03.006