Autoimmune hepatitis (AIH) is a chronic, autoimmune disease of the liver that occurs when the body's immune system attacks liver cells, causing the liver to be inflamed. Common initial symptoms may include fatigue, nausea, muscle ache, or signs of acute liver inflammation including fever, jaundice, and right upper abdominal pain. 

The exact cause of autoimmune hepatitis is unclear, but genetic and environmental factors appear to interact over time to trigger the disease. Untreated autoimmune hepatitis can lead to scarring of the liver, called cirrhosis. It can also eventually lead to liver failure. When diagnosed and treated early, however, autoimmune hepatitis often can be controlled with medicines that suppress the immune system such as corticosteroids.

There are approx. 10,000 AIH patients in the UK.

In clinical practice AIH is diagnosed by a combination of abnormal liver function tests, immunoglobulins and auto-immune antibodies and liver biopsy. 

It is necessary to exclude other liver disease like viral hepatitis, alcohol or drug induced liver disease, and there are blood tests which can help establish this – elevated liver function parameters (IgG) and specialist autoimmune antibodies (ANA, SMA, LKM) are usually used.

About 1/3 of the patients present with liver cirrhosis.

Liver biopsy is performed to confirm diagnosis and ascertain presence or absence of liver cirrhosis.

Steroids (usually prednisolone or budesonide) have been proven to induce remission in AIH patients by treating the active inflammation. Immunosuppressants (azathioprine) are usually added to the steroid therapy after 4 weeks. For maintenance of remission it is recommended to continue with immunosuppressant therapy alone and add in steroids if required.

The therapy should be started immediately after diagnosis.

Treatment with systemic steroids (prednisolone) is known to cause side effects:

• Short term – weight gain, moon face, mood swings, acne and hirsutism
• Long term – diabetes, osteoporosis, glaucoma, hypertension
• Physiological effects like sleep disturbances, mood changes and depression are also common and can be short or long term side effects 

For patients who are non-cirrhotic budesonide can be prescribed as it offers similar efficacy to prednisolone with a significantly reduced risk of side effects. It is important to discuss available treatment options with your doctor t as the therapy is commonly lifelong. Relapse is very frequent and it is crucial to adhere to the prescribed therapy to reduce the risk of frequent relapse.

Primary Biliary Cholangitis (PBC) is a chronic disease of the liver that starts in the bile ducts within the liver. As these ducts are destroyed by inflammatory processes, bile accumulates in the liver (cholestasis). Ultimately the active liver tissue is destroyed and replaced by fibrous tissue. This is the basis for the development of cirrhosis of the liver.

PBC most frequently affects women over the age of 35 to 40 years. At first, the disease may progress without specific symptoms, although patients may notice increased fatigue, occasional itching (pruritus) or dry mouths. Not infrequently, the disease is discovered by coincidence when elevated liver enzyme levels are noticed as part of a routine examination. As the disease progresses, patients with PBC may develop jaundice and yellowish-gray fat deposits in the skin of their eyelids (xanthelasmas) and some patients may report pain in the joints.

PBC can be recognized based on a few simple laboratory tests. Increased levels of certain liver enzymes such as alkaline phosphatase may indicate bile accumulation, however the transaminases are only slightly elevated. If, in addition, patients show increased levels of the immunoglobulin IgM and antimitochondrial antibodies (AMA) are detected in the blood, the diagnosis can be confirmed.

Ursodeoxycholic acid (UDCA) is the drug that has been proven to have beneficial effects not only on symptoms of PBC but also on laboratory parameters (blood test) and life expectancy.

The UDCA therapy should start immediately after the diagnosis has been confirmed and should be continued indefinitely. This at least slows down the progress of the disease and in favorable cases the liver transplantation may be postponed or even avoided. Using the correct dose of UDCA and adherence to therapy are crucial in treatment of PBC. Dosing is calculated on individual basis and is dependent on patient body weight which should be monitored regularly in order to maintain the correct dose.