Autoimmune Hepatitis (AIH)
Hepatitis means “inflammation of the liver”. There are many causes for this and some of these are discussed in depth on other pages. Autoimmune hepatitis (AIH) is an uncommon cause of hepatitis. It was previously known as “chronic active hepatitis”. It causes a chronic (or long standing) form of hepatitis which if left untreated, may progress to other stages of liver disease such as cirrhosis.
The exact reason for developing this condition remains uncertain. It is thought to be linked with a problem with the body’s own immune system. Normally, our immune system works in our favour, fighting off foreign germs and cells. But in those with various autoimmune diseases, the immune system begins to target its own body’s organs and treats these as though they are foreign.
The immune system works in 2 ways to protect the body;
- Formation of antibodies: these special proteins attack and destroy foreign tissues,
- Development of special white cells: there are several types of these cells.
In AIH, it is white cells called lymphocytes which are thought to be the main problem. These attack the body’s own hepatocytes (liver cells) causing inflammation and damage. The reason for why this happens is unclear. Possible triggers which have been speculated for this immune response include viral infections, chemicals, drugs and other factors. There is also thought to be an inherited factor which increases the susceptibility to autoimmune disorders.
Who gets Autoimmune Hepatitis?
AIH is an uncommon condition and the majority of sufferers are women. Most are between 15 and 40 years old. Occasional children may be affected and sometimes, those over 40 years old are also affected. Those affected by AIH are unfortunately also at greater risk of developing other autoimmune disorders such as;
- Diabetes mellitus type 1,
- pernicious anaemia: this is a deficiency of vitamin B12 which leads to anaemia,
- thyroid disease, causing either an over or under active thyroid,
- vitiligo: the development of pale patches of skin anywhere on the body,
- Sjogren’s syndrome
- and many other disorders.
What Are The Symptoms Of Autoimmune Hepatitis?
Symptoms in AIH follow a fairly similar path to other liver conditions. Many individuals have no symptoms in the beginning. But without treatment, mild symptoms develop followed by more severe symptoms. If left untreated, liver cirrhosis develops with the possibility of liver failure. The possible symptoms and signs can therefore be listed as;
None: liver disease may be found incidentally,
- A feeling of being unwell,
- Fatigue,
- Nausea,
- vomiting,
- Diarrhoea,
- Weight loss,
- Dry eyes and mouth,
- Red or pink, blotchy, mottled patches on the palms of hands (palmer erythema),
- Abnormal small blood vessels on the surface of the skin, mainly on the face, chest and arms (spider naevi),
- Abdominal pain or discomfort,
- Itching of skin,
- Enlargement of the liver (hepatomegaly),
- Jaundice: yellowing of the skin and whites of the eyes,
- Passing dark urine: associated with jaundice,
- Passing pale or clay coloured stools: associated with jaundice,
- Bruising,
- Bleeding from any site e.g. gums, nose, wounds, rectum, vagina,
- Abdominal distension from fluid collecting in the abdomen (ascites),
- Vomiting of blood (haematemesis), from oesophageal varices,
- Confusion and an altered level of consciousness (hepatic encephalopathy),
- Coma,
- Death.
In a minority of cases, there is a more rapid escalation of symptoms causing an acute hepatitis. This causes a rapid fever, abdominal pain, nausea & vomiting and lethargy. The course of this rapidly developing disease can be unpredictable. In some, it settles down to form a slowly progressing chronic hepatitis whereas in others, it continues to worsen with a severe and rapidly deteriorating hepatitis leading to rapid liver failure.
Even in the usual slowly progressive disease state, the chronic hepatitis does eventually lead to liver cirrhosis within approximately 10 years. But with adequate treatment, the outlook is good as treatment usually suppresses the condition and halts the inflammatory process that is hepatitis.
Diagnosing Autoimmune Hepatitis
Initially, testing is carried out in the same way as described for the many other liver conditions in this guide. This includes blood tests for liver function tests (LFT), clotting (INR) and several other blood tests. These may be followed by ultrasound scanning. More specialised blood tests may be required that may suggest an autoimmune cause. These include an “auto-antibody” screen. Specific examples of this include; anti-nuclear antibodies (ANA) and smooth muscle antibodies (SMA). If these are present and elevated, then the possibility of an autoimmune cause for the liver damage has to be considered.
But the main method used to make sure of the diagnosis is liver biopsy. This can be done in the following ways;
1. Percutaneous Liver Biopsy
This can be done unguided or guided by ultrasound. After the position of the liver has been established, a local anaesthetic is injected into the skin. This is usually done over the upper right side abdomen or between the lower ribs. After the skin is numb, the doctor makes a small incision into the skin and inserts a needle (sometimes called a Trucut) into the wound. This is inserted into the liver and removed. It will contain a small sample of liver tissue.
2. Laparoscopic Liver Biopsy
This is usually done under general anaesthetic. With this approach, a small thin tube containing a light and camera is inserted into the abdomen. Through a separate small incision, another instrument is used to target specific areas of the liver to collect samples which are then removed.
3. Transjugular Liver Biopsy
This approach is sometimes used when a patient has severe clotting problems or when there is fluid in the abdomen. It is done in an X-ray unit. A catheter is inserted into a vein in the neck. This is then guided to the liver. A biopsy needle is then passed through the catheter until it travels down to the liver. A sample of liver tissue is then obtained and removed. This is generally a less risky approach compared to the percutaneous approach.
How Is Autoimmune Hepatitis Treated?
The basis for treatment in AIH is to reduce and eliminate inflammation from the liver and suppress the immune symptom so that no further inflammation occurs. This is done using medicines such as;
- Steroid tablets (prednisolone): this reduces inflammation and is used as first line therapy. Treatment with high dosages of steroid tablets is started. Once the AIH is brought under control, the dosage is reduced. Maintenance treatment consists of the lowest possible dose of prednisolone required to control inflammation.
- Azathioprine: this is an immunosuppressant drug and may be used alongside steroids, but only once steroids have reduced inflammation. Azathioprine prevents the immune system being able to mount any further attacks on its own organs.
The use of these 2 drugs together is likely to be better than using either one alone. The success rate is good but it may take several months to a year to achieve adequate control. This treatment may continue for 2 or more years. If control is deemed adequate, a trial without treatment may be advised. In some, no further treatment is required but in others, the AIH flares up again and treatment needs to be re-started. For some, continuous lower dose treatment is required. This is called maintenance therapy.
Other treatment options are available for those who fail to respond to prednisolone and/or azathioprine or who develop side effects. These options include cyclosporine and tacrolimus.
But for a minority, if treatment fails to control AIH or if the disease is diagnosed late, the only option may be liver transplantation.
All those suffering from liver disease are strongly advised not to drink alcohol.
