What is autoimmune hepatitis?
Autoimmune hepatitis is a rare, chronic or acute autoimmune disease of the liver. The disease is not contagious. In an autoimmune disease, the body’s own immune system is misdirected and attacks the body’s own cells. In autoimmune hepatitis, the immune system attacks the liver cells (hepatocytes). The disease destroys the liver cells and often leads to severe liver inflammation(hepatitis). Severe autoimmune hepatitis must be treated quickly, otherwise the liver will fail within a short time. The disease is easily treatable with medication. Untreated AIH can be fatal. There are two other known autoimmune diseases of the liver: primary biliary cirrhosis (PBC) and primary sclerosing cholangitis (PSC).
How common is autoimmune hepatitis?
Autoimmune hepatitis can occur at any age. In Western Europe, around 10 to 17 out of every 100,000 inhabitants suffer from AIH. The number of diseases seems to be increasing, perhaps also because specialists are now more likely to look for them. Women are affected much more frequently than men.
Acute or chronic?
Acute autoimmune hepatitis has been present for less than six months. If the disease lasts longer, it is referred to as chronic autoimmune hepatitis.
Supposedly acute autoimmune hepatitis is not always really acute. Some sufferers have unrecognized chronic autoimmune hepatitis, which only becomes apparent when the symptoms worsen acutely. In these people, the liver is already scarred due to many years of undetected illness. Complete scarring leads to liver cirrhosis.
Risks of advanced autoimmune hepatitis
Liver cirrhosis is a serious finding. The liver is completely scarred. Once a certain point is exceeded, liver cirrhosis is life-threatening, as the organ is permanently damaged. Numerous complications can arise.
Three main problems with liver cirrhosis
- The liver is no longer able to fully perform its numerous tasks in the body (metabolism, digestion, detoxification, etc.). The loss of liver function can contribute to the development of a water belly (ascites). This can lead to a serious infection with bacteria. In addition, toxins can enter the brain and cause serious disorders such as hepatic encephalopathy.
- About one and a half liters of blood flow through a healthy liver every minute. If the liver is hardened and scarred, blood can back up in the portal vein (so-called portal hypertension). The pressure on the portal vein can contribute to the development of a hydrocephalus and lead to further complications. These include varicose veins in the stomach and oesophagus or an enlarged spleen. The varicose veins can burst and lead to dangerous bleeding.
- Even with liver cirrhosis, new liver cells are still formed. But the process is becoming increasingly uncontrolled. Cells can degenerate and liver cancer can develop (liver cell carcinoma or hepatocellular carcinoma).
Autoimmune hepatitis: causes and risk factors
In autoimmune hepatitis, the body’s own immune system attacks the liver cells for as yet unknown reasons. Various causes of autoimmune hepatitis are being discussed. It is assumed that those affected have a genetic predisposition to autoimmune hepatitis. If other triggers such as toxins, an infection, a drug intolerance or pregnancy are added, the “dormant” autoimmune hepatitis can break out for the first time.
Possible cause of autoimmune hepatitis
The following factors are possible triggers for autoimmune hepatitis:
- genetic predisposition
- Environmental toxins
- bacterial antigens (for example salmonella antigens)
- Viruses (hepatitis A, B, C and D viruses)
- Measles viruses
- Herpes viruses
- hepatotropic viruses that are still unknown today
However, the causes and risk factors for autoimmune hepatitis have not yet been conclusively researched. In many cases, those affected cannot report any particular event. Many people suddenly feel worse or their family doctor notices elevated liver values.
Symptoms: Autoimmune hepatitis is not immediately noticeable
The symptoms of AIH are similar to those of acute or chronic hepatitis. As a result of chronic inflammation of the liver, connective tissue remodeling of the liver, known as fibrosis, occurs. If left untreated, the remodeling ends in cirrhosis. Around a quarter of all autoimmune hepatitis sufferers have no symptoms prior to diagnosis. This is the case with many liver diseases, as our liver cannot feel pain. The fact that “something is wrong” with the liver is only determined by elevated liver values.
Possible (non-specific) symptoms of autoimmune hepatitis
- Loss of appetite
- heavy weight loss
- Fatigue
- mild flu-like symptoms
- Discomfort
- light-colored chair
- dark urine
- Fever, rheumatic joint pain
- Yellowing of the skin and eyes
- dry mucous membranes
- Hair loss
- Absence of menstruation (amenorrhea)
When first diagnosed, up to a third of adults affected already have cirrhosis. In the case of sick children, almost half. This is probably due to the sometimes rapid progression of the disease or the fact that autoimmune hepatitis is often diagnosed late.
Possible concomitant diseases
Autoimmune hepatitis can be accompanied by other diseases that need to be treated separately:
- Thyroid diseases (Hashimoto’s thyroiditis)
- Primary biliary cholangitis (PBC)
- Diabetes mellitus
- primary sclerosing cholangitis (PSC)
- Sjögren’s syndrome
- Psoriasis
- inflammatory bowel diseases
- systemic lupus erythematosus
- Kidney inflammation (glomerulonephritis)
- Celiac disease
- multiple sclerosis
Gluten intolerance has an impact on therapy, as gluten intolerance can hinder the absorption and effectiveness of AIH medication.
Why doesn’t the liver hurt?
There are no nerves in the liver that allow us to feel pain. Therefore, the liver itself cannot hurt. However, there are nerve fibers in the liver capsule that can trigger pain. We notice this, for example, when there is swelling of the liver in certain liver diseases.
Autoimmune hepatitis: Diagnosis
The diagnosis of autoimmune hepatitis is very complex. It should only be made by experienced specialists. When making a diagnosis, it is important to rule out other liver diseases such as fatty liver, viral hepatitis or metabolic diseases.
The following examinations are carried out for the diagnosis:
- Liver values, immunoglobulins (blood test)
- Autoantibodies such as ANA, ASMA, anti-LKM, SLA/LP-AK (blood test)
- Typical cell changes in the liver tissue (liver puncture)
- Exclusion of other liver diseases (blood tests and other tests if necessary)
Classification according to antibody detection
We differentiate between three types of autoimmune hepatitis based on the antibody spectrum that can be detected in each case:
- AIH type 1: most common form in around 80 percent of cases; with detection of SMA-AK, ANA and p-ANCA,
- AIH type 2: in around 10 percent of cases; with evidence of LKM1-AK,
- AIH type 3: in around 10 percent of cases; with evidence of SLA/LP-AK.
Autoimmune hepatitis: prevention, early detection, prognosis
In an autoimmune disease, the immune system fights its own body. This dysregulation of the immune system can neither be prevented nor corrected. If left untreated, autoimmune hepatitis can lead to liver cirrhosis, which can be fatal. It is therefore important to recognize the disease as early as possible. Once the disease has been diagnosed, the misdirected immune reaction is suppressed with the help of medication to prevent further damage to the liver.
Course and prognosis
The disease can usually be treated well with immunosuppressants. Affected persons have an almost normal life expectancy. In rare cases, there may be no response to the drug therapy. Then there is a risk of liver failure. The last treatment option in this case is a liver transplant.
Self-help groups
The exchange with people who are affected by the same disease can be a great support in coping with the disease. Advice on finding a suitable self-help group is available from
Selbsthilfe Zürich. Self-Help Zurich and the University Hospital Zurich are cooperation partners in the national project “Health literacy thanks to self-help-friendly hospitals”.
Autoimmune hepatitis: treatment with immunosuppressants
The aim of treating autoimmune hepatitis is to stop or mitigate the immune reaction against the patient’s own liver cells. To this end, the immune system is suppressed with drugs known as immunosuppressants. Two immunosuppressive drugs are often given for effective therapy. Therapy with immunosuppressants often has side effects and places a long-term strain on the body. For most patients, however, immunosuppressive therapy is essential for survival. Without immunosuppression, the disease is usually fatal.
In rare mild cases, treatment can be delayed.
The following drugs are currently approved for autoimmune hepatitis:
- Prednisone/prednisolone,
- Budesonide,
- Azathioprine.
Details of the treatments