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Definition
Demographics
Causes
Clinical features
Variation in skin types
Complications
Diagnosis
Differential diagnoses
Treatment
Prevention
Outcome
Cholestasis is defined as a decrease in bile flow secondary to impairment or obstruction of bile secretion. Cholestatic pruritus is pruritus that occurs in people with cholestatic liver diseases, of which there are numerous types.
Pruritus may occur in any cholestatic disease. This includes diseases in which there is bile duct damage in the liver, reduced secretion of bile by the liver, or obstruction to the flow of bile inside or outside the liver.
Cholestatic pruritus may also be referred to as the pruritus of cholestasis.
Cholestatic pruritus is experienced by 80-100% of patients with cholestatic liver disease. Interestingly, the severity of the pruritus has no correlation to the severity of the cholestasis.
The prevalence of cholestatic pruritus varies depending on the underlying disease:
The exact cause is unknown. Several theories have been proposed but it is deemed most likely that cholestatic pruritus is mediated by several pruritogenic substances, including bile acids, serotonin, progesterone derivatives, endogenous opioids, and lysophosphatidic acids. These substances are often elevated in cholestatic liver disease.
A genetic component is also likely to be a factor, as not all patients with cholestasis experience pruritus.
It is thought that the pruritogens mediating cholestatic pruritus are made in the liver and excreted in bile, triggering the sensation of itch through activation of a complex neural network, although the pathophysiological mechanism is still not well defined. This is supported by the fact that itching improves or resolves with liver transplantation and may reduce in intensity with advancing liver failure as the liver’s exocrine function becomes progressively impaired.
Cholestatic pruritus has no specific cutaneous features. Skin lesions caused by scratching may appear different in darker skin types compared to lighter skin types, for example, nodular prurigo tends to be firmer, larger, and darker in those with darker skin.
Cholestatic pruritus can be diagnosed in a patient with cholestasis who complain of itching. Provided the underlying cholestatic disease is known, an extensive diagnostic evaluation is unnecessary.
Other causes of pruritus must be excluded. This requires detailed history and examination, blood tests, and may include imaging studies.
Cholestatic pruritus has a broad differential diagnosis, as even if someone has a diagnosed cholestatic liver disease they may have a concurrent issue causing pruritus.
Some differential diagnoses include:
The first step of treatment for cholestatic pruritus is the identification and treatment of the underlying cholestatic disease.
Treatments specific to certain types of cholestatic liver diseases are as follows:
In addition to the above, for those who do not have intrahepatic cholestasis of pregnancy, there are four classes of medications commonly used to manage cholestatic pruritus. One option is usually given for 2-4 weeks, before being replaced with the next line of therapy.
Refractory cholestatic pruritus:
There is no known way to prevent cholestatic pruritus.
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