Pruritus cholestatic L29.9

Author: Prof. Dr. med. Peter Altmeyer

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Last updated on: 19.09.2022

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Synonym(s)

Cholestatic itching; cholestatic pruritus; Cholestatic Pruritus; Pruritus in cholestasis

History
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Pregnancy cholestasis:

  • Generalized pruritus with inconspicuous skin during pregnancy, especially on palms and soles, disappears after delivery.
  • Laboratory: GPT and bilirubin are often elevated; an increase in serum bile acid concentration > 10 µmol/l (take fasting values!) is conclusive.
  • With bile acids > 40 µmol/l, there is an increased rate of premature births and stillbirths as well as increased perinatal mortality; the fetal prognosis correlates with the severity of the clinical picture
  • Therapy: ursodeoxycholic acid 12.5-20 mg/kg bw/d, daily dose about 1g (4 tbls), leads to reduction of pruritus and fetal risks; close monitoring, early initiation if necessary.
  • There is a risk of recurrence in subsequent pregnancy as well as when taking oral contraceptives

Definition
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Pruritus caused by various hepatobiliary disorders. Pruritus is an early symptom of cholestasis and thus a common complication of acquired or congenital liver disease.

Etiopathogenesis
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In a larger study, 60% of pruritus patients suffered from either viral hepatitis (40%), mainly hepatitis C, or biliary tract disease (primary or secondary sclerosing cholangitis). Recent underestimates indicate an increased concentration of lysophosphatidic acid (LPA) in the serum of patients with cholestatic pruritus. LPA acts via LPA receptors.

This is caused by an increased activity of the membrane ectoenzyme autotaxin (ATX). Autotaxin catalyzes the formation of LPA from lysphosphatidylcholine. Autotaxin activity correlates with the severity of itching. Autotaxin inhibitors will be of particular interest for this form of itching.

In addition to autotaxin and its cleavage product lysophosphatidic acid, endogenous opioids play a role, since a reduced hepatobiliary excretion probably leads to an accumulation of these substances. This could play a role in the action of u-opioid receptor antagonists (e.g. naltrexone) (Ständer S 2018).

Manifestation
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It is not uncommon (about 25%) for years of pre-existing pruritus to occur even before the hepatic disease was diagnosed.

Clinical features
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It is not uncommon for cholestatic pruritus to begin as localized itching on the soles of the feet or palms of the hands or other sites (arms and legs). Over time, frequent development of generalized pruritus. Genitoanal pruritus is uncommon.

The pruritus is experienced as "pure" itching in only a smaller proportion of patients; mixed sensations such as burning or stinging are more common. Triggers include tight-fitting clothing, sweating, or stress. Rare is aquagenic triggering (showering, bathing). In most cases, itching occurs throughout the day; about 25% of patients notice an intensification or even the exclusive occurrence in the evening or at night.

A special form of cholestatic pruritus is pruritus in pregnancy cholestasis.

Internal therapy
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Therapy primarily depends on the treatment of the underlying diseases (DD: intrahepatic (K71.0) and extrahepatic cholestasis (K83.1)).

Cholestyramine (anion expander): Results are interpreted very differently.

Alternative: ursodeoxycholic acid (UDCA: 10-15mg(kgkgkg/day); even with this medication only little success is to be expected.

Alternative: it is accepted that gabapentin should be used as an initial antipruritic therapy (Cave: efficacy is only expected after 8 weeks).

Alternative: Opioid receptor antagonists (naltrexone, naloxone)

Alternative: controversial is the use of systemic antihistamines (the influence of histamine is controversial in the CP)

Alternatively, in case of resistance to therapy: phototherapy, plasmapheresis, albumin dialysis, nasobiliary drainage.

Literature
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  1. Hegade VS et al (2016) The safety and efficacy of nasobiliary drainage in the treatment of refractory cholestatic pruritus: a multicentre European study. Aliment Pharmacol Ther 43: 294-302.
  2. Hegade VS et al (2019) Autotaxin, bile acid profile and effect of ileal bile acid transporter inhibition in primary biliary cholangitis patients with pruritus. Liver Int 39:967-975.
  3. Huesmann M et al (2013) Cholestatic pruritus: a retrospective analysis of clinical characteristics and treatment response. JDDG 11: 158-169
  4. Kremer AE et al (2015) Pathogenesis and management of pruritus in PBC and PSC. Dig Dis 33 Suppl 2:164-175.
  5. Stand S (2018) Pruritus, Prurigo. In: Braun-Falco`s Dermatology, Venereology Allergology G. Plewig et al (eds) Springer Verlag p 587.
  6. Stull C et al (2016) Advances in therapeutic strategies for the treatment of pruritus. Expert Opin Pharmacother 17: 671-687.

Incoming links (1)

Cholestatic pruritus;

Disclaimer

Please ask your physician for a reliable diagnosis. This website is only meant as a reference.

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Last updated on: 19.09.2022