Autoimmune hepatitis

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Classification according to ICD-10
K75.4 Autoimmune hepatitis
ICD-10 online (WHO version 2019)
Microfoto of autoimmune hepatitis. HE staining .

The autoimmune hepatitis (AIH) is a rare, acute or chronic inflammatory autoimmune disease of the liver . The own immune system attacks liver cells, which leads to inflammation of the liver ( hepatitis ). In autoimmune hepatitis, the liver has lost its immune tolerance .

Occurrence

In Europe and North America, around 0.2–1.2 new cases per 100,000 people are diagnosed in one year. Autoimmune hepatitis is therefore responsible for around 10–20% of chronic liver diseases. The disease is around four times more common in women than in men. Around half of the new cases affect people before the age of 30.

causes

It can possibly be triggered by other diseases. Environmental toxins , bacterial antigens (e.g. Salmonella antigens) and viruses are discussed as triggers of autoimmune hepatitis , such as B. the viruses of hepatitis A, B, C and D or also measles and herpes viruses , which may be the cause of the disease, but cannot be detected in the autoimmune hepatitis phase.

Disease emergence

Due to the lack of immune tolerance, the T cells attack antigens on the membrane of the liver cells. An antibody-mediated reaction against the liver cells also occurs. The exact mechanism of the disease is still unclear.

Clinical manifestations

Autoimmune hepatitis is mainly associated with severe symptoms. These include tiredness, nausea, loss of appetite, fever, joint pain and the typical jaundice as soon as the liver is no longer able to break down the bilirubin that occurs . In addition, autoimmune inflammations of the thyroid gland , blood vessels, large intestine, pleura or skin often occur . Autoimmune anemias also occur more frequently. Older patients are more likely to have a gradual progression of the disease with less severe symptoms.

Diagnosis

In laboratory terms, the disease is characterized by an increase in transaminases and an increase in antibodies , especially immunoglobulin (IgG). Every third person has an increase in bilirubin. Characteristic, however, are the occurrence of antibodies such as antibodies against cell nuclei ( ANA ), smooth muscle fibers (SMA), microsomes of liver and kidney cells (LKM) or a soluble liver protein ( Soluble Liver Antigen SLA, correctly abbreviated as anti-Soluble Liver Antigen ) ASLA, synonymous with liver pancreas antigen LP). These antibodies are only found in approx. 80% of the sick and occasionally also in other diseases. They are not responsible for the pathogenesis of chronic autoimmune hepatitis, but represent leading serological markers of the disease for the diagnosis . On the basis of different antibody spectra, a distinction is made today between three types of hepatitis, although there are overlaps between them. The treatment of the three types does not differ. An assessment of the liver histology is essential for estimating the progression of the disease.

pathology

A histological examination of the liver can determine the degree of inflammation and its consequences. In the mild form of inflammation, the invasion of lymphocytes is limited to the portal field . In the more severe form, the inflammatory cells spread to the liver lobule itself. Both single cell necrosis and necrosis at the boundary plate of the lobule occur. In the advanced stage, the liver undergoes fibrotic remodeling and finally liver cirrhosis .

to form

Type I.

Type I (formerly: lupoid hepatitis) is the most common type of autoimmune hepatitis. It makes up around four fifths of diseases. Type I occurs more frequently between the ages of 20 and 40. 40-70% of the patients show an increased level for antibodies . SMA antibodies occur in 70–100% of cases. Antinuclear antibodies often only appear in the course of the disease. Type I shows very good therapy results. Nevertheless, 40–45% of those affected develop cirrhosis of the liver.

Type II

Type 2 is characterized by LKM antibodies, which are not found in other forms of AIH. It affects around 10% of the sick and manifests itself mainly in childhood or adolescence. Also typical are strong clinical symptoms that resemble acute hepatitis. Around four fifths of patients develop cirrhosis in their lifetime.

Type III

Type III was previously assumed in the presence of LP / SLA antibodies. The clinical course is similar to type I. In the meantime, it is no longer seen as an independent entity and has been assigned to type I in more recent publications.

Distribution pattern of antibodies

Autoantibodies ANA SMA SLA LKM pANCA AMA
Autoimmune hepatitis type I (lupoid hepatitis) 100% 60-90% O O O O
Autoimmune hepatitis type II (LKM-positive hepatitis) O O 100%
Autoimmune hepatitis type III (SLA-positive hepatitis) O 100% O
primary biliary cholangitis (PBC) <10% 10% O O <10% 95%
primary sclerosing cholangitis 50% <10% O O 80% O
Type II in chronic hepatitis C. 5% 5% O 100% O O

Explanations of the abbreviations

Abbr. English German
SMA smooth muscle antibody Smooth muscle antibodies
SLA soluble liver antigen soluble liver antigen
LKM liver kidney microsome Antibodies against endoplasmic reticulum (microsomes) of the liver and kidneys
pANCA perinuclear staining pattern of anti-neutrophil cytoplasmatic antibodies antineutrophil cytoplasmic antibodies with perinuclear pattern
AMA anti mitochondrial antibody antimitochondrial antibodies
ANA anti nuclear antibody antinuclear antibodies

therapy

The core of the therapy is the corticosteroid prednisone . It should be used in combination with the purine analog azathioprine in order to keep the prednisone dose as low as possible, since prednisone, like all corticosteroids , can trigger Cushing's syndrome as a dose-dependent side effect. Treatment should be continued for two to four years until a withdrawal attempt is made. In 80% of the cases it has to be continued for life. In addition, medical osteoporosis prophylaxis should be performed to reduce the bone-degrading effect of prednisone. In 2012, treatment with the synthetic glucocorticoid budesonide was approved in Germany . The main advantages here were the fewer side effects, and budesonide is also combined with azathioprine.

In the case of treatment failure, only data from small case series are available. In individual cases, good results have been achieved with cyclophosphamide . For Type I patients, tacrolimus , mycophenolate mofetil, and methotrexate have also had satisfactory results. The use of Cyclosporin A is controversial. When all drug measures have been exhausted, a liver transplant can be attempted. Autoimmune hepatitis can also affect the transplanted organ. In 7–10% of cases there is a relapse in the first year, and within five years the number rises to 60–70%. So far, no relapses have been described in patients with HLA type DR3.

Prospect of healing

Without treatment, autoimmune hepatitis is fatal. With adequate treatment, 90% of patients who respond to therapy have only an imperceptibly reduced life expectancy. However, this only affects patients who have not yet developed cirrhosis of the liver. Around 10% of patients do not respond to combination therapy with prednisone and azathioprine. Treatment attempts with non-approved drugs or a liver transplant can be carried out for this group.

Web links

Individual evidence

  1. a b c d Wolfgang Piper: Internal medicine. Heidelberg 2007, p. 406f.
  2. a b c d e E. Kuntz, H.-D. Kuntz: Hepatology - Textbook and Atlas. 3. Edition. Heidelberg 2008, pp. 656-667.
  3. a b c Gerd Herold u. a .: internal medicine. Cologne 2009, p. 507.
  4. a b c H. Denk, HP Dienes, W. Jochum, P. Schirmacher, M. Trauner: Liver and intrahepatic biliary tract. In: W. Böcker, H. Denk, Ph. U. Heitz, H. Moch: Pathology. 4th edition. Munich 2008, p. 791 f.
  5. MP Manns, M. Woynarowski, W. Kreisel, Y. Lurie, C. Rust, E. Zuckerman, MJ Bahr, R. Guenther, RW Hultcrantz, U. Spengler, AW Lohse, F. Szalay, M. Färkkilä, M Pröls, CP Strasbourg, European AIH-BUC-Study Group: Budesonide induces remission more effectively than prednisone in a controlled trial of patients with autoimmune hepatitis. In: Gastroenterology . 2010; 139, pp. 1198-1206, doi: 10.1053 / j.gastro.2010.06.046 .