Primary Biliary Cholangitis (Primary Biliary Cirrhosis) Clinical Presentation

Updated: Nov 08, 2017
  • Author: Nikolaos T Pyrsopoulos, MD, PhD, MBA, FACP, AGAF, FAASLD, FRCP(Edin); Chief Editor: BS Anand, MD  more...
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Of patients with primary biliary cholangitis, 25% are incidentally diagnosed during a routine blood evaluation.

Fatigue (65%)

Fatigue is the first reported symptom. It can cause disability in some patients and has been associated with depression and obsessive-compulsive behavior. The etiology is unknown; however, a sleep abnormality, particularly excessive daytime somnolence, has been identified in a significant proportion of patients and has been associated with the degree of fatigue.

No correlation exists between this symptom and the stage of the liver disease, the height of the levels of liver enzymes, the Mayo model score, or the duration of therapy.

The etiology of fatigue is unclear; although some evidence suggests that abnormalities of the hypothalamic-pituitary-adrenal axis, decreased release of serotonin, and increased production of proinflammatory cytokines (ie, interleukin-1 [IL-1], interleukin-6 [IL-6], tumor necrosis factor-α [TNF-α] ) may be responsible. [7]

Pruritus (55%)

According to estimates, 10% of patients experience severe pruritus.

The cause of this symptom is not known, but pruritus appears unrelated to the deposition of bile acids in the skin.

Increased opioidergic tone (ie, increased production of endogenous opioid peptides, and the upregulation of endogenous opioid receptors) appears to be the major mechanism. The height of the bilirubin level is proportionally related to the production of these peptides.

Right upper quadrant discomfort

This occurs in 8-17% of patients.


Physical Examination

Physical examination findings depend on the stage of the disease. In the early stages, examination findings are normal. As the disease advances, excoriations of the skin, xanthomas, xanthelasmas, or findings of cirrhosis may be present.

Other findings may include the following:

  • Hepatomegaly (25%)

  • Hyperpigmentation (25%)

  • Splenomegaly (15%)

  • Jaundice (10%)

  • Xanthelasma (10%) - In late stages of the disease

  • Sicca syndrome (50-75%) - Xerophthalmia (ie, dry eyes), xerostomia (ie, dry mouth)

  • Kayser-Fleischer rings (extremely rare)

  • Stigmata of advanced liver disease (ie, cirrhosis), such as spider nevi, palmar erythema, ascites, temporal and proximal muscle wasting, and peripheral edema