What is autoimmune hepatitis?
Autoimmune hepatitis (AIH) is a so-called autoimmune disease. These are diseases in which the immune system forms antibodies against the body’s own structures (autoantibodies). In the case of autoimmune hepatitis, these are autoantibodies against the liver tissue: they attack the liver cells and ultimately destroy them as if they were foreign cells or dangerous intruders. Autoimmune hepatitis is usually chronic. However, an acute course is also possible.
Approximately 80 percent of all people with autoimmune hepatitis are women. The disease occurs at any age, but is most common in young to middle-aged adults between the ages of 20 and 50. In Europe, around one to two in 100,000 people develop autoimmune hepatitis every year. AIH is therefore a relatively rare disease.
Combination with other diseases
Autoimmune hepatitis often occurs together with other immune-mediated diseases. These include, for example
- Autoimmune thyroid inflammation (autoimmune thyroiditis = Hashimoto’s thyroiditis)
- Autoimmune inflammation of the bile ducts within the liver (primary biliary cholangitis)
- Autoimmune inflammation of the bile ducts inside and outside the liver (primary sclerosing cholangitis)
- Rheumatoid arthritis (RA)
- Sjögren’s syndrome
- Diabetes mellitus type 1
- Celiac disease
- Inflammatory bowel diseases
- Multiple sclerosis (MS)
- Vitiligo (white spot disease)
- Psoriasis (psoriasis)
What are the symptoms of autoimmune hepatitis?
Acute autoimmune hepatitis causes symptoms of acute liver inflammation such as fever, nausea and vomiting, upper abdominal pain and jaundice. In rare cases, the disease progresses quickly and severely (fulminant) with acute liver failure. This can be recognized, for example, by jaundice, blood clotting and impaired consciousness.
However, most sufferers develop chronic autoimmune hepatitis with a gradual progression. There are usually no or only unspecific symptoms for a long time:
- Fatigue and poor performance
- lack of appetite
- weight loss
- Aversion to fatty foods and alcohol
- Abdominal pain and headaches
- Fever
- Rheumatic joint pain
- Pale stools and dark urine
- Yellowing of the skin, mucous membranes and the white sclera in the eye (jaundice)
In most cases, chronic autoimmune hepatitis leads to liver cirrhosis.
If autoimmune hepatitis occurs together with other autoimmune-related diseases, further symptoms are added.
What should I pay attention to in my diet?
If possible, people with liver disease should avoid alcohol completely, as it is detoxified in the liver and puts additional strain on the organ. It is also advisable to maintain a normal body weight.
Causes and risk factors
In autoimmune hepatitis, autoantibodies attack the liver tissue. This triggers inflammation, which ultimately destroys the liver cells. It is not known why the immune system turns against the body’s own tissue in those affected. Experts suspect that those affected have a genetic predisposition to autoimmune hepatitis. If external factors (triggers) are added, the disease breaks out. Possible triggers include infections, environmental toxins and pregnancy.
Autoimmune hepatitis: classification
Autoimmune hepatitis (AIH) was originally divided into three variants according to the type of autoantibodies present:
- Type 1 autoimmune hepatitis (AIH1): It is the most common form of autoimmune hepatitis. Those affected have antinuclear antibodies (ANA) and antibodies against smooth muscle fibers (anti-SMA). Certain antibodies against neutrophil granulocytes, known as p-ANCA (ANCA = anti-neutrophil cytoplasmic antibodies), are also often present.
- Type 3 autoimmune hepatitis (AIH3): Only antibodies against soluble liver antigens/liver-pancreas antigens (anti-SLA/LP) can be detected in the blood of those affected.
Type 3 autoimmune hepatitis is considered a variant of type 1: the autoantibodies typical of AIH3 (anti-SLA/LP) sometimes occur together with ANA and/or anti-SMA (typical autoantibodies in type 1 autoimmune hepatitis).
Examinations and diagnosis
Diagnosing autoimmune hepatitis is not easy – there is currently no diagnostic test that can prove AIH. Instead, it is a diagnosis of exclusion: only when the doctor has ruled out all other possible causes for the symptoms (for example, virus-related hepatitis) can they make the diagnosis of “autoimmune hepatitis”. This requires various examinations, which should be carried out by an experienced specialist.
Blood tests
The blood sample is also examined for autoantibodies against liver cells. Various autoantibodies can usually be detected in autoimmune hepatitis. They play a decisive role in the clarification of autoimmune hepatitis, but are not sufficient on their own for a definitive diagnosis.
If autoimmune hepatitis is acute or very sudden and severe (fulminant), autoantibodies and an increase in immunoglobulin G (IgG) may be absent.
The blood sample is also examined for antibodies against hepatitis viruses. If these are present, viral hepatitis rather than autoimmune hepatitis is probably responsible for the symptoms.
The TSH value should also be determined when clarifying autoimmune hepatitis. This hormone value provides an indication of thyroid function. Autoimmune hepatitis is often accompanied by autoimmune thyroid inflammation (autoimmune thyroiditis).
Ultrasound
An ultrasound examination of the liver can be used to detect general pathological changes in the tissue. These include the conversion of liver tissue into connective/scar tissue (fibrosis of the liver). This ultimately leads to liver cirrhosis. This is caused by chronic autoimmune hepatitis, among other things, but often also has other causes.
Attempted treatment with immune suppressants
Sometimes the doctor gives the patient medication that suppresses the immune system (immune suppressants), namely glucocorticoids (“cortisone”), on a trial basis. These are part of the standard treatment for autoimmune hepatitis. If the symptoms improve with the medication, this is an indication of autoimmune hepatitis, but not definitive proof.
Liver biopsy
To confirm the diagnosis of autoimmune hepatitis, the doctor takes a tissue sample from the liver (liver biopsy). This is then examined in the laboratory. If characteristic cell changes are found, it is very likely that autoimmune hepatitis is indeed present.
Treatment
Autoimmune hepatitis cannot yet be treated causally. This means that the dysregulation of the immune system cannot be corrected. However, the doctor will prescribe medication that suppresses the immune system. These immunosuppressants inhibit the inflammatory processes in the liver. This helps to combat the symptoms and generally prevents further liver damage (including cirrhosis and liver failure).
If the autoimmune hepatitis is very mild with low inflammatory activity, it is possible in individual cases to dispense with treatment with immunosuppressants.
If the chronic autoimmune hepatitis has not yet led to liver cirrhosis, the doctor sometimes prescribes the active ingredient budesonide in combination with azathioprine instead of prednisolone/prednisone. This is also a cortisone preparation, but it is said to cause fewer side effects than prednisolone.
In certain cases, other medications are also used. For example, if the therapy described above does not work, autoimmune hepatitis can be treated with other immunosuppressants such as ciclosporin, tacrolimus, sirolimus or everolimus on a trial basis. If the patient cannot tolerate azathioprine, the doctor will switch to alternatives, for example the immune suppressant mycophenolate mofetil. Regular check-ups with the doctor are necessary during treatment.
Prolonged cortisone treatment promotes bone loss (osteoporosis). Adult patients are therefore given calcium and vitamin D to prevent osteoporosis.
How long does immunosuppressive therapy last?
If the autoimmune hepatitis is very mild with low inflammatory activity, it is possible in individual cases to dispense with treatment with immunosuppressants.
If the chronic autoimmune hepatitis has not yet led to liver cirrhosis, the doctor sometimes prescribes the active ingredient budesonide in combination with azathioprine instead of prednisolone/prednisone. This is also a cortisone preparation, but it is said to cause fewer side effects than prednisolone.
In certain cases, other medications are also used. For example, if the therapy described above does not work, autoimmune hepatitis can be treated with other immunosuppressants such as ciclosporin, tacrolimus, sirolimus or everolimus on a trial basis. If the patient cannot tolerate azathioprine, the doctor will switch to alternatives, for example the immune suppressant mycophenolate mofetil. Regular check-ups with the doctor are necessary during treatment.
Prolonged cortisone treatment promotes bone loss (osteoporosis). Adult patients are therefore given calcium and vitamin D to prevent osteoporosis.
How long does immunosuppressive therapy last?
Chronic diseases such as autoimmune hepatitis may be recognized as a disability. The degree of disability is determined by the extent of the illness. If the degree of disability is over 50, this is considered a severe disability. Whether autoimmune hepatitis actually meets the criteria for severe disability in an individual case is assessed individually by the relevant pension office following a corresponding application.
