Autoimmune hepatitis – the progressing chronic gepatotsellyulyarny defeat proceeding with signs of periportalny or more extensive inflammation, a gipergammaglobulinemiya and existence serumal hepatoassociated autoantitet. Clinical displays of autoimmune hepatitis include astenovegetativny frustration, jaundice, pains in the right podreberye, skin rashes, a gepatomegaliya and a splenomegaliya, at women, a ginekomastiya – at men. Diagnosis of autoimmune hepatitis is based on serological identification of anti-nuclear antibodies (ANA), fabric antibodies to smooth muscles (SMA), antibodies to microsomes of a liver and kidneys, etc., gipergammaglobulinemiya, increases in a caption of IgG, and also given to a liver biopsy. The basis of treatment of autoimmune hepatitis is made by immunosupressivny therapy by glucocorticosteroids.
In structure of chronic hepatitises in gastroenterology 10-20% of cases at adults and 2% - at children fall to the share of autoimmune damage of a liver. Women get sick with autoimmune hepatitis by 8 times more often than men. The first age peak of incidence is the share of age up to 30 years, the second – for a postmenopause. The course of autoimmune hepatitis has the fast-progressing character at which cirrhosis, the portal hypertensia and a liver failure leading to death of patients early enough develops.
Reasons of autoimmune hepatitis
Questions of an etiology of autoimmune hepatitis are studied insufficiently. It is considered that the stseplennost with certain anti-genes of the main complex of a gistosovmestimost (HLA of the person) - alleles of DR3 or DR4, coming to light at 80-85% of patients is the cornerstone of development of autoimmune hepatitis. As the presumable trigger factors starting autoimmune reactions at genetically predisposed individuals Epstein-Barre's viruses, hepatitises (And, In, C), measles, herpes (HSV-1 and HHV-6), and also some medicines can act (for example, interferon). More than at a third of patients with autoimmune hepatitis also other autoimmune syndromes come to light – tireoidit, Greyvs's disease, sinovit, ulcer colitis, Shegren's disease, etc.
The basis of pathogenesis of autoimmune hepatitis is made by deficiency of immunoregulation: decrease in subpopulation T-supressornykh in lymphocytes that leads to uncontrollable synthesis by V-cages of IgG and to destruction of membranes of cells of a liver – hepatocytes, to emergence of characteristic serumal antibodies (ANA, SMA, anti-LKM-l).
Types of autoimmune hepatitis
Depending on the formed antibodies distinguish autoimmune hepatitis I (anti-ANA, anti-SMA positive), II (anti-LKM-l positive) and III (anti-SLA positive) types. Each of the allocated types of a disease is characterized by a peculiar serological profile, features of a current, a response to immunosupressivny therapy and the forecast.
Autoimmune hepatitis I of type proceeds with education and circulation in blood of anti-nuclear antibodies (ANA) – at 70-80% of patients; antigladkomyshechny antibodies (SMA) at 50-70% of patients; antibodies to cytoplasm of neutrophils (pANCA). Autoimmune hepatitis I of type develops aged from 10 up to 20 years and after 50 years more often. It is characterized by a good response to immunosupressivny therapy, a possibility of achievement of permanent remission in 20% of cases even after cancellation of corticosteroids. In the absence of treatment within 3 years cirrhosis is formed.
At autoimmune hepatitis II of type at blood 100% of patients have antibodies to microsomes of a liver and kidneys of the 1st type (anti-LKM-l). This form of a disease develops in 10-15% cases of autoimmune hepatitis, mainly at children's age and is characterized by high biochemical activity. Autoimmune hepatitis II of type more to immunosupression; at cancellation of medicines often there comes the recurrence; cirrhosis develops twice more often than at autoimmune hepatitis I of type.
At autoimmune hepatitis III of type antibodies to a soluble hepatic and pechenochio-pancreatic anti-gene (anti-SLA and anti-LP) are formed. Quite often at this type ASMA, a rheumatoid factor, anti-mitochondrial antibodies (AMA), antibodies to anti-genes of a hepatic membrane come to light (antilma).
Carry the cross syndromes including also symptoms of primary biliarny cirrhosis, primary skleroziruyushchy holangit, chronic viral hepatitis to options of atypical autoimmune hepatitis.
Symptoms of autoimmune hepatitis
In most cases autoimmune hepatitis demonstrates suddenly and in clinical manifestations does not differ from an acute hepatitis. In the beginning proceeds with the expressed weakness, lack of appetite, intensive jaundice, emergence of dark urine. Then within several months the clinic of autoimmune hepatitis is developed.
The beginning of a disease gradual is more rare; in this case astenovegetativny frustration, an indisposition, weight and pains in the right podreberye, insignificant jaundice prevail. At a part of patients autoimmune hepatitis begins with fever and extra hepatic manifestations.
The period of the developed symptoms of autoimmune hepatitis includes the expressed weakness, feeling of weight and pain in the right podreberye, nausea, a skin itch, a limfadenopatiya. The changeable, amplifying during the periods of aggravations jaundice, increase in a liver are characteristic of autoimmune hepatitis (gepatomegaliya) and spleens (splenomegaliya). At a third of women at autoimmune hepatitis the girsutizm develops ; at boys the ginekomastiya can be observed.
Skin reactions are typical: kapillyarit, a palmarny and volchanochnopodobny eritema, purple, an acne, a teleangiektaziya on face skin, a neck and hands. During the periods of exacerbations of autoimmune hepatitis I can be observed the phenomena of passing ascites.
The recurrent migrating polyarthritis affecting large joints but not leading to their deformation belongs to system displays of autoimmune hepatitis. Rather often autoimmune hepatitis proceeds in combination with ulcer colitis, myocarditis, pleurisy, perikardity, glomerulonefrity, tireoidity, vitiligo, insulin-dependent diabetes, iridotsiklity, Shegren's syndrome, Cushing's syndrome, a fibroziruyushchy alveolit, hemolytic anemia.
Diagnosis of autoimmune hepatitis
As diagnostic criteria of autoimmune hepatitis serve serological, biochemical and histologic markers. According to the international criteria, it is possible to speak about autoimmune hepatitis in case:
- in the anamnesis there are no hemotransfusions, reception of gepatotoksichny medicines, abuse of alcohol;
- in blood markers of an active viral infection are not found (hepatitises A, B, C, etc.);
- level of γ-globulins and IgG exceeds normal indicators in 1,5 and more times;
- considerably hyperactivity of NUCLEAR HEATING PLANT, ALT;
- credits of antibodies (SMA, ANA and LKM-1) for adults higher than 1:80; for children higher than 1: 20.
The liver biopsy with a morphological research of a sample of fabrics allows to reveal a picture of chronic hepatitis with signs of the expressed activity. Bridge-like or step necroses of a parenchyma, lymphoid infiltration with abundance of plasmatic cages are histologic signs of autoimmune hepatitis.
Tool researches (ultrasonography of a liver, liver MRT, etc.) at autoimmune hepatitis have no independent diagnostic value.
Treatment of autoimmune hepatitis
Pathogenetic therapy of autoimmune hepatitis consists in performing immunosupressivny therapy by glucocorticosteroids. Such approach allows to reduce activity of pathological processes in a liver: to increase activity T-supressorov, to reduce intensity of the autoimmune reactions destroying hepatocytes.
Usually immunosupressivny therapy at autoimmune hepatitis is carried out by Prednisolonum or methylprednisolonum in an initial daily dose of 60 mg (the 1st week), 40 mg (the 2nd week), 30 mg (the 3-4th weeks) with decrease to 20 mg as the supporting dose. Reduction of a daily dosage is carried out slowly, considering activity of a clinical current and level of serumal markers. The patient has to accept the supporting dose before full normalization of clinical laboratory and histologic indicators. Treatment of autoimmune hepatitis can continue from 6 months to 2 years, and sometimes and throughout all life.
At inefficiency of monotherapy introduction to the scheme of treatment of autoimmune hepatitis of an azatioprin, chlorocinchona, a tsiklosporina is possible. In case of inefficiency of immunosupressivny treatment of autoimmune hepatitis within 4 years, a multiple recurrence, side effects of therapy the question and transplantations of a liver is raised.
The forecast at autoimmune hepatitis
In the absence of treatment of autoimmune hepatitis the disease steadily progresses; spontaneous remissions do not occur. As an outcome of autoimmune hepatitis serves cirrhosis and a liver failure; 5-year survival does not exceed 50%. By means of the timely and accurately carried out therapy it is possible to reach remission at most of patients; at the same time survival within 20 years makes more than 80%. Transplantation of a liver yields the results comparable with lekarstvenno the reached remission: the 5-year forecast is favorable at 90% of patients.
At autoimmune hepatitis only secondary prevention including regular observation of the gastroenterologist (hepatologist), control of activity of hepatic enzymes, contents of γ-globulins is possible, autoantitet for timely strengthening or renewal of therapy. The sparing mode with restriction of emotional and physical activities, observance of a diet, branch from preventive vaccination, restriction of reception of medicines is recommended to patients with autoimmune hepatitis.