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Intrahepatische Schwangerschaftscholestase

Selten, aber relevant

Intrahepatic cholestasis of pregnancy

Rare but important

Zusammenfassung

Die intrahepatische Schwangerschaftscholestase ist eine schwangerschaftsspezifische Lebererkrankung, die durchschnittlich in etwa 0,5–2,0 % aller Schwangerschaften auftritt. Sie zeigt meist einen benignen Verlauf für die Mutter und ist durch maternalen Pruritus v. a. im dritten Trimester, erhöhte Transaminasen und Nüchtern-Gallensalzkonzentrationen im Serum sowie ein gesteigertes fetales Risikoprofil charakterisiert. Die Ätiologie der intrahepatischen Schwangerschaftscholestase ist bisher nur teilweise geklärt, scheint aber multifaktoriell bedingt zu sein. Cholestase verursachende Effekte bestimmter weiblicher Schwangerschaftshormone und deren Metabolite spielen dabei eine entscheidende Rolle bei genetisch prädisponierten Frauen. Ebenfalls sind die Mechanismen unbekannt, die für die fetalen Komplikationen wie spontane Frühgeburten, Mekoniumfärbung des Fruchtwassers, Neugeborenenasphyxie bis hin zu fetalen Todesfällen verantwortlich sind. Für die Pathogenese des Pruritus in der Schwangerschaftscholestase scheinen bestimmte sulfatierte Progesteronmetabolite verantwortlich zu sein. Im Gegensatz zu Pruritus bei schwangerschaftsassoziierten Dermatosen liegen bei Patienten mit intrahepatischer Schwangerschaftscholestase keine primären Hautveränderungen vor. Allerdings kann intensives Kratzen zu sekundären Hautveränderungen wie Erosionen, Exkoriationen, Krusten und selten auch zu Prurigo nodularis führen. Die Behandlung basiert entsprechend den aktuellen Leitlinien in der oralen Gabe von Ursodeoxycholsäure und der teils praktizierten elektiven Geburtseinleitung in der 37. bis 38. Schwangerschaftswoche, um möglichen fetalen Risiken, insbesondere der Totgeburt, zuvorzukommen. Dieser Übersichtsbeitrag fasst Epidemiologie, klinische Symptome, Diagnose, Behandlung und insbesondere die Pathogenese des Pruritus zusammen.

Abstract

Intrahepatic cholestasis of pregnancy (ICP) is a liver-specific disorder occurring in approximately 0.5–2.0% of all pregnancies with a considerable variation in certain ethnic groups. ICP usually runs a benign course for the mother and is characterized by maternal pruritus mainly in the third trimester, elevated transaminases and fasting total serum bile salts and increased fetal adverse events. The etiology of ICP is only partially understood but seems to be multifactorial. Cholestasis-inducing effects of certain female sex hormones and their metabolites play an important role in genetically susceptible women. The mechanisms resulting in fetal complications such as spontaneous preterm labour, antepartum passage of meconium, asphyxia events, still birth and fetal death are not well understood. Certain sulfated progesterone metabolites are likely to play a role in the pathogenesis of pruritus in ICP. In contrast to pregnancy-related dermatoses, pruritus does not present with primary skin alterations. However, intense scratching may cause secondary skin changes such as abrasions, excoriations and sometimes prurigo nodularis. Treatment is based on ursodeoxycholate treatment to reduce pruritus and hepatic impairment as well as elective delivery between gestation week 37–38 to pre-empt potential stillbirths. This article reviews clinical symptoms, diagnosis, treatment and in particular pathogenesis of pruritus in ICP.

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Correspondence to A. E. Kremer.

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A.E. Kremer, K. Wolf und S. Ständer geben an, dass kein Interessenkonflikt besteht.

Dieser Beitrag beinhaltet keine von den Autoren durchgeführten Studien an Menschen oder Tieren.

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Kremer, A.E., Wolf, K. & Ständer, S. Intrahepatische Schwangerschaftscholestase. Hautarzt 68, 95–102 (2017). https://doi.org/10.1007/s00105-016-3923-y

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  • DOI: https://doi.org/10.1007/s00105-016-3923-y

Schlüsselwörter

  • Gallensalze
  • Progesteron
  • Juckreiz
  • Pruritus
  • Ursodeoxycholsäure

Keywords

  • Bile salts
  • Progesterone
  • Itch
  • Pruritus
  • Ursodeoxycholic acid