Intrahepatic Cholestasis of Pregnancy (ICP)

The aim of this leaflet

This leaflet is designed to tell you more about Intrahepatic Cholestasis of Pregnancy (ICP). It tells you what the condition is, what causes it, what can be done about it and where to find out more about it.

What is Intrahepatic Cholestasis of Pregnancy?

Intrahepatic Cholestasis of Pregnancy refers to a liver condition in which the normal flow of bile is impaired resulting in severe itching in the mother and a risk for stillbirth and prematurity (delivery before term) in the baby. ICP usually develops during the last third of pregnancy, when hormone levels are highest. ICP is also referred to as obstetric cholestasis, cholestasis of pregnancy, and pruritus/prurigo gravidarum.

What causes Intrahepatic Cholestasis of Pregnancy?

There is a defect in the excretion of bile from the liver resulting in rising of bile acids in the blood. The exact mechanism is still not fully understood, but current research suggests genetic, hormonal and environmental factors. Recently a particular gene mutation (change in a specific gene) has been detected in some ICP patients. ICP typically develops when there are high hormone levels in late pregnancy and in women carrying twins or triplets. It may also occur in women on the oral contraceptive pill. In Europe about 0.2-2.4% of pregnant women will get the condition; in Scandinavia and South America ICP is more common. 

Does Intrahepatic Cholestasis of Pregnancy run in families?

Yes – mothers and sisters of patients with ICP are at higher risk of developing ICP, because there seems to be a change in one of your genes, which may be present in other family members. ICP also tends to recur in subsequent / following pregnancies (60-90%). 

What are the features of Intrahepatic Cholestasis of Pregnancy and what does it look like?

The main feature is a sudden onset of severe itch that will typically increase in severity until you are either treated for it or delivery takes place. It often starts on the palms and soles, but then quickly involves the whole body. The longer the itch persists, the more skin changes due to scratching may be present. These vary from fine scratch marks (excoriations) to 5-10mm raised lumps (so-called prurigo nodules) typically involving the shins, arms and buttocks. Apart from these changes, there is usually no rash associated with ICP. Loss of sleep, loss of appetite, and an inability to perform normal daily tasks can be a result of the intense itching.
Less common symptoms (<10% of patients) include dark urine and/or pale stools (greyish in colour), jaundice (the white of your eyes and sometimes the skin becomes yellowish), pain in your belly, and nausea.  

How will Intrahepatic Cholestasis of Pregnancy be diagnosed?

The best available test for ICP is the “serum bile acid test”. A diagnosis of ICP is certain when bile acid levels are elevated above the normal range. As bile acids are not part of a routine liver function test (LFT), this test must be specifically requested by your doctor. However, there are only few laboratories that have the equipment necessary to perform this test, because of this the diagnosis may be delayed.
A routine liver function test (LFT) that measures liver enzymes in the blood should also be done when checking for ICP. However, this test may be normal in ICP, meaning you may still
have ICP, because “serum bile acid levels” typically rise before liver enzymes increase. If “serum bile acid levels” are normal but LFTs are found to be increased, your doctor will search for other liver diseases for example hepatitis, other tests are required to diagnose this.  

Will the baby be affected?

Yes, this may happen. ICP harbours an increased risk for infant stillbirth (intrauterine death of the baby), premature (early) labour, and foetal distress (being unwell). If LFTs are abnormal in patients with ICP, in particular an elevated bilirubin (your eyes and skin may turn yellow). There is an increased risk for bleeding (haemorrhage) in both mother and child, because vitamin K, which is essential for blood clotting, is not absorbed properly.  

Can Intrahepatic Cholestasis of Pregnancy be cured?

No. It can not be cured, because it is, at least partly, determined by your genes. However, treatment for ICP will help both the mother’s and baby’s symptoms. Without treatment, the itch usually stops within days to weeks after delivery when hormone levels return to normal. However, a recurrence in following pregnancies or being on the contraceptive pill is common (see above).  

How can Intrahepatic Cholestasis of Pregnancy be treated?

Two important steps may be taken:
(1) Reducing the bile acids in your bloodstream: Ursodeoxycholic acid (UDCA - a naturally occurring bile acid) is currently the best treatment of ICP. UCDA improves the liver function, helps to reduce the toxic (bad) bile acid concentration in the bloodstream and improves bile acid transport across the placenta (takes it away from the baby). However, UDCA is not licensed for use in pregnancy. It may be prescribed on an individual base, with what is called ‘informed consent’. You have to discuss this with your doctor.
It is the only treatment that has been shown to reduce foetal risks in ICP. UDCA tablets, 15mg/kg/day or simply 1g daily are given either as a single dose or divided into 2-3 doses and continued until delivery then treatment can usually be stopped.
Other treatments seem not to be beneficial. Cholestyramine and other cholesterol lowering drugs should be avoided because they may increase the risk of bleeding.
(2) Delivering the mother as early as possible. However, the development of the baby’s lungs is important, and usually delivery may be after they are fully developed at weeks 36 or 37 of your pregnancy.  

Is the treatment safe for the baby and mother? Is any special monitoring required?

UDCA is safe for the mother and the baby and it is the only treatment that will reduce the baby’s risk. The only adverse effect may be mild diarrhoea in the mother.
Close monitoring of the baby is important; your doctor may prefer to deliver the baby early (between weeks 37 and 38 of your pregnancy) in order to keep the risk for your baby as low as possible. 
Certain weekly check-ups upon diagnosis are suggested; they include a “biophysical profile” (test that uses the non-stress test and ultrasound to examine foetal movements, heart rate, and amniotic fluid amounts), cardiotocography (evaluates foetal heart rate), Doppler flow study (a type of ultrasound which uses sound waves to measure the blood flow). These tests need to be discussed with a specialist.  

Is normal delivery possible?

Yes.  

Can women with Intrahepatic Cholestasis of Pregnancy still breastfeed?

Yes. Neither ICP nor treatment with UDCA influences breastfeeding negatively.  

Where can I get more information about Intrahepatic Cholestasis of Pregnancy?

Web links to detailed leaflets:

www.itchymoms.com
www.ocsupport.org.uk
 
Books:

Black, Edwards, Lynch, Ambros-Rudolph. Obstetric and Gynecologic Dermatology. 3rd edition, 2008 (UK)
Schaefer, Spielmann, Vetter. Arzneiverordnung in Schwangerschaft und Stillzeit. 8th edition, 2012 (Germany)
Schaefer, Peters, Miller: Drugs during pregnancy and lactation. 2nd edition 2007 (English)
Briggs, Freeman, Yaffe. Drugs in pregnancy and lactation. 7th edition, 2005 (USA)
 
While every effort has been made to ensure that the information given in this leaflet is accurate, not every treatment will be suitable or effective for every person. Your own doctor will be able to advice in greater detail. The style and general information of this leaflet is adapted from the BAD and Itchy Moms patient information leaflets. 
 
This leaflet has been prepared by the EADV task force “skin disease in pregnancy”, it does not necessarily reflect the official opinion of the EADV.
 
Updated 15.06.2013