The Latest on Intrahepatic Cholestasis of Pregnancy – Update 2026

Abstract

Intrahepatic cholestasis of pregnancy (ICP) is the most common liver disorder specific to pregnancy. The condition is characterised by raised serum bile acids and aminotransferases and typically presents with pruritus as its defining symptom. Ursodeoxycholic acid remains the first line therapeutic option. Clinical management — including decisions regarding the timing of delivery — is guided principally by serum bile acid concentrations, with the aim of reducing perinatal complications. This review offers a clear and integrated overview of current diagnostic criteria, management strategies, and the evidence underpinning contemporary therapeutic recommendations.

Introduction

Intrahepatic cholestasis of pregnancy (ICP) is the most common pregnancy-related liver disorder 1 2 . It results in impaired secretion of substances that are normally excreted in the bile, which usually leads to elevated levels of bile acids and transaminases in the serum. Clinically, this condition is associated with skin that is initially free of lesions from the second half of pregnancy onwards. Intense scratching can lead to secondary skin changes. Reversibility of the clinical picture is a characteristic feature. The itching subsides shortly after delivery, and the elevated transaminase levels and cholestasis parameters should return to normal within a few weeks. Off-label treatment with ursodeoxycholic acid is the first-line treatment, which both alleviates pruritus and reduces bile acid levels. ICP is associated with a range of peripartum complications. There are currently no guidelines on ICP in Germany; however, the Working Group on Obstetrics and Prenatal Medicine (AGG) has issued recommendations on its management 3 .

Epidemiology

Across Europe, approximately 0.7–1% of pregnant women are affected, with significant geographical and ethnic variations. Scandinavian countries have the highest incidence rates in Europe. Globally speaking, incidence rates are in some cases significantly higher in South America and the Asian region 4 5 6 7 8 9 10 . A seasonal increase in incidence during the winter months has been described in the literature 11 .

Aetiopathogenesis

The aetiopathogenesis is multifactorial and has not yet been fully elucidated. In addition to hormonal 12 and genetic 13 14 15 factors, environmental factors 11 also play an important role. Due to hormonal stimulation caused by the accumulation of progesterone metabolites, the secretion of bile acids and other choleretic substances from hepatocytes is disrupted. Certain sulphated progesterone metabolites, such as PM2DiS, PM3S and PM3Dis, appear to play a significant role in the pathogenesis of ICP and pruritus 16 .

The main symptom of the condition is pruritus. This usually occurs in the third trimester, rarely before then. At this stage, physiological levels of oestrogen and progesterone reach their peak during pregnancy. This leads to impaired function of the two key transport proteins, BSEP (bile salt export pump) and MDR3 (multidrug resistance-associated protein 3, phospholipid transporter) 17 .

During pregnancy, an increase in bile acids and a change in their composition are physiological. The ratio of cholic acid (CA) to chenodeoxycholic acid (CDCA) is the same in healthy pregnant women, although there tends to be a slight predominance of CDCA. If ICP is present, a foeto-maternal concentration gradient of bile acids develops due to the proportional increase in CA 18 . Due to the increased presence of taurine-conjugated bile acids, the glycine-to-taurine ratio is reduced 19 . There is also ongoing discussion that a maternal immunological imbalance is associated with ICP.

Similarly, molecular genetic factors influence the pathogenesis and development of ICP, affecting the mechanisms of the bile acid receptor and bile acid transport. Genetic variations are found in 10–15% of cases 20 . At approximately 16%, these are the most common variants in the ABCB4 gene, located on chromosome 7q21.1. This gene encodes MDR3 (multidrug resistance-associated protein 3), which is responsible for the transmembrane transport of phospholipids into bile 13 20 . Furthermore, variants are found in approximately 5% of cases in the ABCB11 gene, which encodes the hepatocyte-specific protein BSEP (bile salt export pump). If a genetic variant is present, the transport of bile acids from the hepatocytes into the bile may be impaired. Consequently, the accumulation of toxic bile acids can lead to liver cell damage 20 21 . Certain genetic variants are also associated with an increased risk of hepatocellular carcinoma 20 .

Other genes described as playing a role in ICP include ATP8B1, TJP2, ABCC2, NR1H4, FGF19 and SLC4A2 20 22 23 24 . However, genetic testing following prior counselling should only be considered on a case-by-case basis after a pregnancy.

Women with a genetic predisposition often have altered oestrogen and progesterone levels during pregnancy, as well as pre-existing elevated bile acid levels, which contribute to an increased incidence of the condition 4 .

Risk Factors

Elevated levels of oestrogen and progesterone appear to play a significant role in the development of ICP. In particular, elevated oestrogen levels 25 are found in twin pregnancies 26 and following hormonal hyperstimulation 27 28 in early pregnancy as part of assisted reproduction. In addition to advanced maternal age (over 35 years), other factors associated with an increased risk of ICP are summarised in Table 1 .

Table 1 Maternal risk factors for ICP.

Maternal risk factors
Multiple pregnancy 7 8 26 29 30 31 32
Maternal age > 35 years
Low selenium levels 22 33
Low vitamin D levels 34 35
Chronic hepatitis C virus infection 36 37
Chronic hepatitis B virus infection 38
Cholelithiasis 39
ICP in the medical history 15
Reproductive medical procedures 40
Substitution with oral or vaginal progesterone 41 42 43
Polymorphisms in bile transporters (e.g., ABCB4, ABCB11, ATPB1, etc.) 44

There are reports of an increased risk of ICP occurring in women during and after treatment with azathioprine 45 46 47 48 .

Clinical Picture

The condition manifests itself in 65% of cases, predominantly in the third trimester, and rarely earlier 49 50 . Pronounced, newly developed cholestatic pruritus is the main symptom; often, it is also the only symptom. Usually starting in the limbs, pruritus can spread to the entire body and be extremely distressing for those affected 22 . This applies in particular to severe night-time itching 51 , which can lead to serious sleep disturbances. The palms and soles are typically the areas most affected. It is possible for the initial pruritus to occur weeks before any laboratory abnormalities are detected 52 . This is thought to be due to the direct pruritic effect of bile acids on the skin 11 , although the level of bile acids does not correlate with the severity of pruritus 53 54 . The individual experience of pruritus can vary greatly. The link between pruritus and laboratory findings therefore remains unclear, as these changes can occur both before and after the onset of pruritus 22 . Pruritus during pregnancy is not an uncommon symptom; it affects around 25% of pregnant women, with 9% of those experiencing pruritus having ICP 15 55 56 57 . Pain in the upper right abdomen caused by stretching of the liver capsule, as well as nausea or loss of appetite, are other possible non-specific symptoms. There are no primary skin lesions, but secondary changes may develop as a result of scratching. In rare cases, steatorrhoea and vitamin K deficiency may occur as secondary manifestations of hepatobiliary disease. General vitamin K prophylaxis is not currently recommended.

Pregnant women with ICP are also more likely to have associated conditions such as dyslipidaemia, gestational diabetes, foetal macrosomia and pre-eclampsia 29 34 58 59 60 61 62 63 64 . In one study, the earlier ICP manifested, the stronger the association with pre-eclampsia was. The relative risk of pre-eclampsia is 3.74 on average (95% CI 12.0–7.02), and is more than five times higher in cases of severe ICP 60 .

Both the laboratory findings and the clinical changes must resolve within a short time after birth and cease completely after several weeks. If elevated transaminases or cholestasis parameters persist, a more detailed hepatological assessment is essential to rule out hepatobiliary diseases such as autoimmune hepatitis (AIH), primary sclerosing cholangitis (PSC), primary biliary cholangitis (PBC) or hereditary cholestasis syndromes, as these conditions can also mimic ICP clinically and in laboratory tests.

Recurrences occur in up to 70% of subsequent pregnancies 39 . According to a large Swedish study, there is an increased lifetime risk of hepatobiliary diseases 39 , such as gallstones or primary biliary cholangitis 65 , as well as cardiovascular or immunological conditions, such as diabetes mellitus, hypothyroidism, Crohn’s disease or psoriasis 59 .

Diagnostics

ICP is a diagnosis of exclusion. Any onset of pruritus should prompt immediate laboratory testing 66 . Pruritus combined with elevated bile acids confirms the suspected diagnosis. Bile acids play a key role in the emulsification and absorption of dietary fats. The main components are the primary bile acids cholic acid (CA) and chenodeoxycholic acid (CDCA). Conjugated with taurine and glycine, they are secreted into the duodenum, converted by bacterial enzymes in the intestine into secondary or tertiary bile acids, and then largely reabsorbed in the terminal ileum. CA and CDCA exist in a hydrophobic conformation and may therefore play a clinically significant role as toxic metabolites in the pathophysiology of cholestasis 67 . Bile acid levels of up to 10 or 11 µmol/l are considered normal in healthy, fasting pregnant women, whereas levels of up to 14 µmol/l are normal when not fasting 19 .

New maximum peak values for defining the severity of ICP have recently been evaluated. In each case, the postprandial, non-fasting peak values were taken into account 68 . This enables clinicians to improve prediction of poor perinatal outcomes. It was established that, if bile acid levels were measured in a fasting state, 9 out of 10 pregnant women would not be diagnosed with ICP (with levels of ≥ 40 µmol or ≥ 100 µmol/l). The highest levels are measured 30–120 minutes after eating, which is also when the bile acid test should be carried out.

Elevated transaminase levels do not correlate with bile acid levels 69 and are not associated with pregnancy or perinatal outcomes 4 . Other laboratory abnormalities include a possible prolonged partial thromboplastin time (pTT) due to vitamin K deficiency 11 ; mild hyperbilirubinaemia may also be present in fewer than 10% of cases. The enzyme autotaxin has been described as a highly sensitive marker for intrahepatic cholestasis of pregnancy; however, it is not available in routine clinical practice 54 .

Intrahepatic ultrasound can be used to rule out post-hepatic biliary obstructions, particularly as the incidence of ICP is higher in patients with cholelithiasis. No specific ultrasound findings are observed, nor are there any specific histological changes. For this reason, a liver biopsy is not indicated.

As certain genetic causes of ICP are also associated with an increased risk of gallstones, cholecystitis or cholangitis, liver fibrosis and malignant liver diseases, affected women – particularly those who have experienced a severe, fulminant course of the disease – should be offered genetic counselling and, where appropriate, genetic testing 20 .

Terminology

In a study by Mitchell et al., reference values of bile acid were evaluated in 560 non-fasting pregnant women. In this context, values up to 18 µmol/l were defined as normal 68 . Table 2 shows the classification of the severity of ICP based on peak bile acid concentrations during pregnancy.

Table 2 New ICP classification according to 70 .

Diagnosis	Clinical characteristics
Pregnancy pruritus	Pruritus and peak bile acid concentration < 19 µmol/l
Mild ICP	Pruritus and peak bile acid concentration 19–39 µmol/l
Moderate ICP	Pruritus and peak bile acid concentration 40–99 µmol/l
Severe ICP	Pruritus and peak bile acid concentration > 100 µmol/l

Differential Diagnosis

In the differential diagnosis, conditions associated with pruritus – such as polymorphic erythema of pregnancy (PEP), atopic erythema of pregnancy (AEP) or pemphigoid gestationis (PG) 71 – should be ruled out, alongside urticaria and allergic or drug-induced reactions. Furthermore, conditions associated with elevated transaminases and/or cholestasis parameters should be ruled out, such as acute or chronic viral hepatitis (A–E), cholestatic liver diseases (PSC, PBC), obstructive jaundice, acute fatty liver of pregnancy, HELLP syndrome and pre-eclampsia.

Treatment

The primary aim of treatment is to reduce pruritus and improve laboratory test results in order to minimise foetal complications. Ursodeoxycholic acid (UDCA), a naturally occurring, tertiary, hydrophilic bile acid, is the treatment of choice. When taken orally, the unconjugated form is passively absorbed in the jejunum and ileum, whilst the conjugated form is actively absorbed in the distal ileum after passing through the enterohepatic circulation 72 . UDCA protects both hepatocytes and cholangiocytes from bile acid-induced damage 73 . According to the current S2k guideline ‘Diagnosis and Treatment of Chronic Pruritus’, the recommended dose is 10–20 mg/kg body weight per day 74 . The starting dose is usually 3 × 250 mg or 2 × 500 mg, with a maximum daily dose of 2 g/day 3 . In a 2012 meta-analysis, the use of UDCA resulted in a significant reduction in pruritus, transaminases and bile acids compared with placebo, and thus presumably improved the perinatal outcome 75 .

UDCA also reduces bile acid concentrations in amniotic fluid and umbilical cord blood 18 50 . The medicine is safe throughout the entire duration of pregnancy. Mild side effects, such as changes in bowel habits, may occur. However, UDCA is not approved for use during pregnancy and is used off-label. As a result, it requires specific guidance. It should be noted that various analytical laboratory methods are used to determine bile acids. Unlike liquid chromatography and mass spectrometry, this enzymatic method – which is well established and highly reproducible in many laboratories – is unable to distinguish between unconjugated and conjugated bile acids.

The largest prospective, randomised, double-blind PITCHES study to date, conducted in 2019, included 605 pregnant women with mostly mild ICP (mean bile acid levels of 28 μmol/l). Only 25% of patients had a severe ICP ≥ 40 µmol/l. In this group, which had only a low risk of complications, UDCA at a moderate dose (2 × 500 mg) did not improve maternal or neonatal outcomes compared with placebo. The risk of intrauterine foetal death in the placebo group was just 1%, and the risk of preterm birth was 20%; UDCA therapy was unable to reduce these risks significantly 76 . In the secondary analysis conducted in 2021, no subgroup could be identified in which the use of UDCA led to a clinically significant reduction in bile acid scores or pruritus severity 77 .

The updated Cochrane review from 2020 demonstrated a benefit in terms of reducing pruritus and only a trend towards a lower rate of IUFT (RR 0.33, 95% CI 0.08–1.37), a lower rate of transfers to the neonatal unit (RR 0.77, 95% CI 0.55–1.08) and a lower rate of both spontaneous (RR 0.78, 95% CI 0.40–1.23) and iatrogenically induced (RR 0.60, 95% CI 0.37–0.97) preterm births 78 .

Reduced rates of preterm birth have also been demonstrated in other publications 79 80 81 . A study found that bile acid levels of 40 µmol/l or higher were associated with a significant increase in the rate of spontaneous preterm births, whereas this was not the case for levels of < 40 µmol/l 81 . A positive effect of UDCA on foetal lipid metabolism has been reported 82 .

Rifampicin, a bactericidal antibiotic used to treat tuberculosis, also has anti-cholestatic effects. It may be considered in combination with UDCA in cases of persistent pruritus during monotherapy with UDCA 3 . The dose is 150–300 mg/day. This can reduce both the severity of pruritus and the cholestasis parameters 83 . However, there is limited experience during pregnancy. The Australian TURRIFIC study is expected to provide further insights into this 84 . The current S2k guideline ‘Diagnosis and Treatment of Chronic Pruritus’ lists rifampicin (off-label use) as the third-line treatment during pregnancy 74 .

S-adenosylmethionine (SAM, off-label use) at a dose of 1000 mg/day is recommended as a second-line treatment, particularly in cases of persistent pruritus 74 . SAM increases the hepatic expression of the MRP2 transporter and thereby enhances the hepatic excretion of bilirubin glucuronides. As SAM has not been shown in previous publications, with the exception of two older, small-scale studies 85 86 , to be more effective than UDCA in terms of pruritus, bile acids, preterm birth or perinatal outcomes 87 88 89 , the Maternal Diseases Section of the Working Group on Obstetrics and Prenatal Medicine (AGG) cannot currently recommend treatment with SAM 3 .

Similarly, according to the AGG’s recommendation, ICP should not be treated with colestyramine 3 . In a randomised trial, colestyramine was found to be inferior to treatment with UDCA 90 . Due to reduced fat absorption and the potential development of a vitamin K deficiency, this may lead to an increased risk of bleeding in both mother and baby. Intravenous vitamin K reduces the risk of peripartum haemorrhage in severe cases 8 . Similarly, dexamethasone should not be included in the treatment of ICP 3 .

Supportive treatment using moisturising and cooling menthol-based topical preparations can relieve pruritus 22 without affecting laboratory parameters or perinatal outcomes 78 . Antihistamines may also be considered to alleviate symptoms, although their benefit is limited to a placebo effect 3 .

The current guidelines from the European Association for the Study of the Liver (EASL) state that, although evidence is currently limited, rifampicin, colestyramine, guar gum or activated charcoal may be considered as additional treatments to alleviate maternal pruritus (strong recommendation, strong consensus) 91 .

The ileal bile acid transporters (IBAT) inhibitors maralixibat and odevixibat are non-absorbable substances that inhibit the reabsorption of bile acids in the terminal ileum. They are approved for the treatment of pruritus in progressive familial intrahepatic cholestasis (PFIC) and Alagille syndrome for use from infancy onwards. In small case series, they have demonstrated promising results in the treatment of intrahepatic pregnancy-related cholestasis. These medications can be considered for use in pregnant patients in serious cases as an alternative and causal treatment option 92 93 . Table 3 presents various therapeutic drug classes for the treatment of pregnant women with ICP.

Table 3 Overview of therapeutic drug classes for the treatment of women with ICP during pregnancy, adapted from 74 .

Drug class*	Dosage/d	AGG recommendation	Notes from 74
* all for off-label use
Ursodeoxycholic acid (UDCA)	10–20 mg/kg body weight	First line	Antipruritic effect confirmed in controlled trials
Rifampicin in combination with UDCA	150–300 mg	This may be considered on a case-by-case basis Particularly in cases of severe, treatment-resistant pruritus	Antipruritic effect in case reports
S-adenosylmethionine (SAM)	max. 1000 mg	No recommendation	Contradictory results in controlled trials
Colestyramine	8 g	No recommendation	No effect in controlled trials
IBAT inhibitors (Maralixibat, odevixibat)	Odevixibat (40–120 μg/kg body weight)/ Maralixibat (190–570 μg/kg body weight)	No recommendation	Antipruritic effect in case reports

Management

In 2021, the Maternal Diseases Section of the Working Group on Obstetrics and Prenatal Medicine (AGG) published recommendations on the management of ICP 3 . Similarly, in 2021, the American Society for Maternal-Fetal Medicine (SMFM) published its recommendations on this condition 94 . The following year, the Royal College of Obstetricians and Gynaecologists (RCOG) published the revised Green-top Guideline on ICP. In particular, as a change from the previous guideline dating from 2011, the terminology and diagnostic accuracy were redefined using bile acid peak values in the non-fasting state 70 . However, there are still no standardised recommendations 66 .

When managing the situation, a complex balancing act must be achieved. The severity of the mother’s symptoms and the resulting impairment, as well as the risks to the baby arising from iatrogenic preterm birth and its consequences for the newborn in the event of a proactive approach must be considered against the risk of intrauterine foetal death (IUFT) in the third trimester if a wait-and-see approach is adopted. In this context, bile acid concentration is expected to play the most important role in decision-making, as it acts as a concentration-dependent (≥ 40 µmol/l or ≥ 100 µmol/l) predictive marker for poorer perinatal outcomes and IUFT. In the case of ICP, laboratory tests should be tailored to the individual and carried out within the clinical context. To ensure that any potential short-term progression of ICP is not overlooked, the RCOG 70 recommends monitoring liver function parameters, bilirubin and bile acid levels in the laboratory up to once a week. If necessary, in the event that outpatient treatment proves unsuccessful, hospitalisation and close foetal-maternal monitoring – with a possible adjustment to the treatment – must also be considered.

In the analysis by Ovadia et al., which included more than 5200 singleton pregnancies with ICP, the rate of IUFT increased significantly from 34+0 weeks’ gestation onwards when the bile acid concentration was ≥ 100 µmol/l. This represented a 30-fold higher risk compared with women with bile acid levels of < 40 µmol/l 95 .

According to the AWMF S2k guideline on labour induction, induction may be recommended as early as between 34+0 and 36+6 weeks of gestation in cases of ICP and a bile acid concentration of ≥ 100 µmol/l. For values < 100 µmol/l, induction of labour should be considered from 37+0 weeks’ gestation, and from 38+0 weeks it is recommended. These recommendations are based on expert consensus with a consensus strength of +++ 96 .

In its Green-top Guideline No. 43, the RCOG recommends that, for women with mild ICP (bile acid values 19–39 µmol/l) and no other risk factors, delivery may be considered as late as 40 weeks’ gestation without increasing the risk of preterm birth (Level of evidence 1+, Grade of recommendation A). For women with moderate ICP (bile acid levels of 40–99 µmol/l), delivery is recommended at 38 weeks’ gestation (evidence level 1+, recommendation grade A) 70 . If comorbidities are present (gestational diabetes, pre-eclampsia), these may be associated with an increased risk of IUFT 31 . In cases of severe ICP (bile acid values ≥ 100 µmol), the aim should be to deliver the baby at 35–36 weeks’ gestation (evidence level 1+, recommendation grade A), as the risk of intrauterine foetal death subsequently increases significantly compared with the general risk of intrauterine foetal death 70 . This was also confirmed as the optimal approach in a statistical decision analysis of pre- and post-partum decision-making factors (evidence level 1+) 96 .

The approach should always be tailored to the individual. There are still no reliable predictors for forecasting intrauterine foetal death, which is the most serious complication and can, of course, occur unexpectedly and suddenly. For this reason, the level of bile acid concentration remains the most important factor in the decision-making process. In any case, one must be familiar with the exact laboratory method used to determine bile acids in order to interpret the measured levels of bile acids.

The decision to adopt a watch-and-wait approach rather than actively terminating the pregnancy can only be made by taking into account the pregnant woman’s individual risk profile, based on the severity of her pruritus, laboratory results and their progression, additional risks (gestational diabetes, pre-eclampsia, multiple pregnancy, etc.) and foetal condition diagnostics (CTG, biometry, Doppler ultrasound), and may require interdisciplinary consultation and care for the pregnant woman by obstetricians, neonatologists and hepatologists 97 . The pregnant woman should be informed, during a consultation, about the monitoring protocol, and that none of the tests can definitively rule out sudden foetal death.

Pregnant women with ICP do not have higher rates of assisted vaginal deliveries or cesarean sections; recommendations regarding the mode of delivery should therefore be based on established obstetric criteria 3 98 . Fig. 1 illustrates a possible approach for pregnant women with ICP and elevated bile acid levels.

Fig. 1.

Recommendation on the management of elevated GS levels. * according to 70 , ** according to 96 .

Complications and Prognosis

ICP is associated with increased foetal risks. This includes the dreaded IUFT, preterm birth, meconium-stained amniotic fluid with respiratory distress syndrome (RDS), and the need for neonatal intensive care 3 15 31 58 95 99 100 101 102 . The risk of foetal morbidity and mortality correlates with the level of bile acids 31 95 , which accumulate in the foetal compartment 19 and can cause direct harm to the foetus. It is thought that an accumulation of toxic taurine conjugates leads to foetal arrhythmias 103 and pathological, vasoconstrictive mechanisms in the foeto-placental vessels 104 105 . Furthermore, uterine contractility can be increased by the enhanced bile acid-induced expression of oxytocin receptors in the myometrium 106 107 , which may lead to premature labour.

In a meta-analysis by Ovadia et al., the following rates of IUFT were found depending on bile acid concentration: in cases of mild ICP, 0.13% [95% CI 0.02–0.38] (n = 3/2310), with moderate ICP in 0.28% [95% CI 0.08–0.72] (n = 4/1412) and with severe ICP in 3.44% [95% CI 2.05–5.37] (n = 18/524) with a slightly increased overall IUFT rate (OR 1.46, 95% CI 0.73–2.89). The IUFT rate increased significantly after 34 weeks of gestation when bile acid levels were ≥ 100 µmol 95 . The same study confirmed that women with ICP have a significantly increased risk of both spontaneous (OR 3.47, 95% CI 3.06–3.95) and iatrogenically induced (OR 3.65, 95% CI 1.94–6.85) preterm birth. In addition, meconium-stained amniotic fluid was observed more frequently (OR 2.60, 95% CI 1.62–4.16), and the newborns required neonatal intensive care more often (OR 2.12, 95% CI 1.48–3.03).

A significantly higher rate of intrauterine foetal death was also found in a systematic review based on more than 1200 singleton pregnancies where bile acid values were ≥ 100 µmol. In addition, a higher rate of preterm birth and a significantly increased incidence of meconium in the amniotic fluid were observed 108 , which can lead to foetal meconium aspiration syndrome 109 .

A Swedish registry study involving more than 1.2 million singleton pregnancies highlighted an increased risk of preterm birth among late preterm infants born between 32 and 37 weeks’ gestation (OR 3.30, 95% CI 3.00–3.63). Below 32+0 weeks’ gestation, the preterm birth rate was lower than in pregnant women without ICP 5 .

In a French cohort, women with ICP were found to have a significantly increased risk of neonatal respiratory distress syndrome (RDS) (17.1% vs. 4.6%, p < 0.001) compared with pregnant women without ICP 100 .

Women with bile acid concentrations of ≥ 40 µmol/l therefore have an increased risk of spontaneous preterm birth, meconium in the amniotic fluid and foetal hypoxia 31 58 . If pregnancy-related comorbidities (gestational diabetes, pre-eclampsia, twin pregnancy) are present, they can increase the risk of IUFT even in women with low bile acid concentrations 76 95 .

Children of pregnant women with ICP are at increased risk of dyslipidaemia and obesity later in life 110 .

The maternal peripartum prognosis is considered favourable. If oral contraceptives with a high oestrogen content are prescribed to women following an ICP, cholesterol levels may rise and pruritus may occur 58 . The WHO therefore recommends the use of progestogen-only or progestogen-releasing preparations, progestogen-containing IUDs or etonorgestrel implants. Combined oral contraceptives should be subject to a rigorous risk-benefit assessment 111 . All women should be made aware that they are at increased risk of developing complications. In this respect, regular follow-up care is recommended.

Summary

ICP is a liver condition that occurs during pregnancy and can be associated with potentially serious perinatal complications. The main symptom is pruritus associated with elevated serum levels of bile acids, which are considered a predictor of poor perinatal outcomes, including foetal death in utero. Symptom improvement can be achieved using UDCA, the first-line therapy established in clinical practice. Pregnant women should be closely monitored in order to minimise increased perinatal morbidity and mortality.