g Jaundice occurs in 10%
g Xanthelasma may occur in the later stages
g Only in advanced disease will there be cirrhosis with associated features (including ascites, spider naevi and other features of portal hypertension)
Associations
There may be other diseases present too, especially those of auto-immune origin. These include:
g Thyroid disease
g Systemic sclerosis including its variant of CREST (calcinosis, Raynaud's phenomenon (o)esopogeal motility disorder, sclerodactyly and telangiectasiae)
g Coeliac disease
g Extrahepatic malignancy
g Hepatocellular carcinoma
g Seropositive arthritis
g Seronegative arthritis
g Cholelithiasis (gallstones)
The link with extrahepatic malignancy has been disputed and the association with hepatocellular carcinoma may be more than for any other form of cirrhosis.
Investigations
g FBC is often normal but ESR is elevated
g Abnormal LFTs are to be expected but are not invariable. Alkaline phosphatase is usually elevated as it is a cholestatic condition and, less consistently, transaminases are raised. Bilirubin is often normal at first but rises as the disease progresses whilst PTT and albumin are normal until a late stage. IgM is raised.
g When bilirubin rises this suggests disease progression and it heralds liver failure.
g Lipid levels and cholesterol levels are raised in 85% but risk of CHD is not raised as HDL cholesterol is elevated.
g Auto-antibodies are characteristic and in this disease the most specific are anti-mitochondrial antibodies that are present in 90 to 95% with a specificity of 98%. About 35% also have anti- nuclear antibodies. There may well be other autoantibodies, especially related to the thyroid.
g Monitor thyroid function
g Imaging of the liver excludes causes of obstruction like stones. Ultrasound is most commonly used but CT and MRI may be employed. As the disease progresses there may be features suggestive of portal hypertension and cirrhosis.
g Finally, liver biopsy is required to stage the disease.
Staging
Various staging systems have been developed, but the most prominent are those proposed by Ludwig et al and Scheuer.
g Stage 1 (portal stage of Ludwig): Portal inflammation, bile duct abnormalities, or both are present.
g Stage 2 (periportal stage): Periportal fibrosis is present, with
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or without periportal inflammation or prominent enlargement of the portal tracts with seemingly intact, newly formed limiting plates.
g Stage 3 (septal stage): Septal fibrosis with active inflammatory, passive paucicellular septa, or both are present.
g Stage 4 (cirrhosis): Nodules with various degrees of inflammation are present.
TREATMENT
There is no cure for PBC. Treatments aim to ease symptoms and to slow the progression of the disease. A liver transplant can be life-saving in the later stages of the disease.
Treatments to ease symptoms
Itch can be a distressing symptom and can be difficult to treat.
Cholestyramine is the most commonly used medicine for itch. It works by 'binding' to bile in the gut. (When bile gets into the gut, it travels down to the large intestine where some of it is absorbed back into the bloodstream, and goes back to the liver to be reused. As cholestyramine binds to bile in the large intestine, it stops this 'reabsorption' and so more bile than usual is passed out with the faeces. This helps to reduce a build up of bile in the liver and bloodstream which often eases itch.) There may be a delay of 1-4 days after starting treatment before itch improves. Other bile-binding medicines are sometimes used.
Other medicines are sometimes used to ease itch if the above are not helpful. For example, rifampacin, naloxone or phenobarbitone. It is not clear how these work, but they do in some cases. UDCA (see below) may also relieve itch. Plasmapheresis (like a plasma exchange) has been used in some severe cases of persistent, severe itch.
Dry skin can make itch worse, so using liberal amounts of moisturiser is useful if you have dry skin.
No medicines seem to ease tiredness which is often a main symptom.
Treatments to slow the progression of the disease
There is no medicine that stops or reverses the disease. However, some medicines may slow down the progression of the disease in some cases. |
