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Progressive intrahepatic cholestasis — Clinical Guidelines

Overview

Intrahepatic cholestasis of pregnancy (ICP) is a liver disorder characterized by pruritus and elevated total serum bile acids (TSBA), affecting 0.6-0.7% of pregnancies in Australia 1.

Diagnosis

Key Criteria: Pruritus without rash, non-fasting TSBA ≥19 μmol/L 1.

Severity Grading:

- Severe: Peak TSBA ≥40 μmol/L. - Very severe: Peak TSBA ≥100 μmol/L 1.

Additional Tests: Serum transaminases may be elevated, but no critical bile acid level universally mandates treatment 2.

Management

First-Line Treatment: Ursodeoxycholic acid (UDCA) to improve perinatal outcomes and reduce pruritus 1.

Adjunctive Measures:

- Antenatal monitoring and management of pruritus symptoms. - Induction of labor at 37-38 weeks due to concerns over fetal well-being 2.

Special Populations

Pregnancy: Induction of labor at 37-38 weeks common practice due to risk of fetal demise 2.

Other Populations: Limited specific guidance provided in abstracts; focus primarily on pregnancy 1 2.

Key Recommendations

Diagnose ICP in pregnant women with pruritus and non-fasting TSBA ≥19 μmol/L without rash or known liver disorder (Evidence: Strong 1).

Initiate UDCA therapy for ICP to improve perinatal outcomes and alleviate pruritus (Evidence: Moderate 1).

Consider induction of labor between 37-38 weeks for women with ICP due to concerns over fetal risks (Evidence: Expert opinion 2).

References

1 Hague WM, Briley A, Callaway L, Dekker Nitert M, Gehlert J, Graham D et al.. Intrahepatic cholestasis of pregnancy - Diagnosis and management: A consensus statement of the Society of Obstetric Medicine of Australia and New Zealand (SOMANZ): Executive summary. The Australian & New Zealand journal of obstetrics & gynaecology 2023. link 2 Arthur C, Mahomed K. Intrahepatic cholestasis of pregnancy: diagnosis and management; a survey of Royal Australian and New Zealand College of Obstetrics and Gynaecology fellows. The Australian & New Zealand journal of obstetrics & gynaecology 2014. link 3 Freese D. Intracellular cholestatic syndromes of infancy. Seminars in liver disease 1982. link 4 Riely CA. Familial intrahepatic cholestasis: an update. The Yale journal of biology and medicine 1979. link 5 Riely CA, LaBrecque DR, Ghent C, Horwich A, Klatskin G. A father and son with cholestasis and peripheral pulmonic stenosis: a distinct form of intrahepatic cholestasis. The Journal of pediatrics 1978. link80428-4)

Progressive intrahepatic cholestasis