Pregnancy in PSC and after liver transplantation

Although PSC can occur at any age, it does affect young people of child-bearing age. Most women with PSC can undergo an uncomplicated and successful pregnancy ¹.

However, factors that may complicate a pregnancy in women with PSC include the presence of cirrhosis and portal hypertension (high pressure system around the liver). Women with advanced liver disease are deemed to have ‘high-risk’ pregnancies, however, this is not the case for women with mild liver disease. Importantly, complications in those with advanced disease can be avoided with careful monitoring during pregnancy. It is therefore important to discuss any plans to start a family with your hepatologist (liver doctor) or obstetrician as early as possible.

Complicated cases of PSC may require a more formal discussion with a team of experts in the field (e.g. specialist obstetrician, hepatologist and midwife, etc) before conception. This forum facilitates the team to understand what the potential risks are in each individual pregnancy, as well as addressing any concerns. There are assessment tools and scores which can help evaluate risk, so that the team can make an individualised plan about medications and scans.

The main risks that can occur during pregnancy in PSC patients are:

Pre-term birth

One study from Sweden and one study from the UK demonstrated increased rates of pre-term birth (delivery before 37 weeks gestation) in PSC pregnancies. However, there was no increased risk of poorer outcomes (e.g. death) for both mother and baby ^2,3.

Intrahepatic cholestasis of pregnancy

Intrahepatic cholestasis of pregnancy (ICP), also known as obstetric cholestasis, is a pregnancy specific disorder which is caused by a change in the way in which bile is processed through the liver and can lead to a build-up of bile acids. The main symptoms include itching, jaundice (yellowing of the skin/eyes) or pale stools. A small study from Germany reported on 2/21 mothers (≈10%) with PSC who developed ICP during their pregnancy ⁴. ICP can sometimes be difficult to distinguish from a flare of PSC. Treatment of ICP includes ursodeoxycholic acid (UDCA) and in some cases expedited delivery of the baby. You may already be on UDCA, but this may need to be increased during pregnancy. Alternative drugs such as cholestyramine and rifampicin can be used to relieve severe itch during pregnancy.

PSC related complications

The typical problems that occur in PSC outside of pregnancy can also develop during pregnancy including itching, jaundice and cholangitis (infection of the bile ducts) secondary to gallstones or bile duct narrowing. It’s always important to let your doctor know about any new or worsening symptoms and this is also the case during pregnancy. Ultrasound imaging is safe during pregnancy. Magnetic resonance imaging (MRI) is also safe during the 2nd and 3rd trimesters of pregnancy. If imaging during pregnancy reveals stones or a significant narrowing of the bile ducts, an endoscopic procedure called an ERCP (endoscopic-retrograde-cholangiopancreatography) may be recommended, although risks of sedation and radiation requires consideration.

It can sometimes be difficult for women with cirrhosis to get pregnant, because they tend to have higher rates of infertility due to hormonal changes that occur with advanced liver disease. Irregular and infrequent periods are also common.

Pregnant women with cirrhosis are at a slightly higher risk of death (≈0-14%) compared to pregnant women without liver disease, however, your hepatologist and obstetrician will monitor you closely during pregnancy in order to identify any possible complications early.

Existing portal hypertension tends to worsen during pregnancy, which increases the risk of internal bleeding from varices (enlarged veins in the gullet). Pre-emptive screening for varices with an endoscopy should be performed during the second trimester. This procedure is generally safe during pregnancy.

Other complications, such as deterioration in liver function during pregnancy, can also be predicted to a certain extent ⁵.

Patients with cirrhosis are recommended to have additional scans of the baby in the 3rd trimester.

PSC alone should not complicate delivery for patients without cirrhosis. Concerns over internal bleeding from varices (in patients who have portal hypertension) during labour often makes caesarean section the preferred mode of delivery in patients with cirrhosis (although this is controversial).

However, there is a changing school of thought where vaginal deliveries are preferable where possible in these patients.  If a woman is keen on a vaginal delivery, this should be discussed with your obstetrician. However, it is important to remember that each case is different, and a vaginal birth may not be a suitable option in all cases. There is an increased risk of bleeding after delivery in patients with cirrhosis.

Fertility tends to improve in women after liver transplantation. Women should wait at least one year after transplantation before attempting to become pregnant. It’s important that the liver transplant and kidneys are working well.

Pregnancy after liver transplantation should be considered ‘high risk’ and include additional scans of the baby. Medications which suppress your immune system should be continued throughout pregnancy (see table below).

Rejection of the liver during pregnancy is rare. There is an increased risk of pre-term delivery, low birth weight of the baby and pregnancy related blood pressure and diabetes problems in the mother ⁶, so you should have extra monitoring throughout your pregnancy.