Autoimmune Hepatitis - Causes, Symptoms And Treatment Of Autoimmune Hepatitis

2024 Author: Josephine Shorter | [email protected]. Last modified: 2023-12-16 21:43

Autoimmune hepatitis

What is Autoimmune Hepatitis?

Autoimmune hepatitis (AIH) is a progressive inflammatory necrotic liver disease, in which the presence of liver-oriented antibodies in the blood serum and an increased content of immunoglobulins are detected. That is, with autoimmune hepatitis, the liver is destroyed by the body's own immune system. The etiology of the disease is not fully understood.

The direct consequences of this rapidly progressing disease are renal failure and cirrhosis of the liver, which can ultimately be fatal.

According to statistics, autoimmune hepatitis is diagnosed in 10-20% of all chronic hepatitis cases and is considered a rare disease. Women suffer from it 8 times more often than men, while the peak incidence falls on two age periods: 20-30 years and after 55 years.

Content:

• What is Autoimmune Hepatitis?
• Causes of Autoimmune Hepatitis
• Types of autoimmune hepatitis
• Symptoms of autoimmune hepatitis
• Diagnostics
• Autoimmune hepatitis treatment
• Forecast and prevention

Causes of Autoimmune Hepatitis

The causes of autoimmune hepatitis are not well understood. The fundamental point is the presence of a deficiency in immunoregulation - a loss of tolerance to its own antigens. It is assumed that hereditary predisposition plays a role. It is possible that such a reaction of the body is a response to the introduction of an infectious agent from the external environment, the activity of which plays the role of a "trigger" in the development of the autoimmune process.

The viruses of measles, herpes (Epstein-Barr), hepatitis A, B, C and some medications (Interferon, etc.) can act as such factors.

Also, more than 35% of patients with this disease have other autoimmune syndromes.

Diseases associated with AIH:

• Autoimmune thyroiditis;
• Graves' disease;
• Vitiligo;
• Hemolytic and pernicious anemia;
• Dermatitis herpetiformis;
• Gingivitis;
• Glomerulonephritis;
• Insulin-dependent diabetes mellitus;
• Irit;
• Lichen planus;
• Local myositis;
• Neutropenic fever;
• Nonspecific ulcerative colitis;
• Pericarditis, myocarditis;
• Peripheral nerve neuropathy;
• Pleurisy;
• Primary sclerosing cholangitis;
• Rheumatoid arthritis;
• Cushing's syndrome;
• Sjogren's syndrome;
• Synovitis;
• Systemic lupus erythematosus;
• Erythema nodosum;
• Fibrosing alveolitis;
• Chronic urticaria.

Of these, the most common in combination with AIH are rheumatoid arthritis, ulcerative colitis, synovitis, Graves' disease.

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Types of autoimmune hepatitis

Depending on the antibodies detected in the blood, 3 types of autoimmune hepatitis are distinguished, each of which has its own characteristics of the course, a specific response to therapy with immunosuppressive drugs and a prognosis.

Type 1 (anti-SMA, anti-ANA positive)

It can appear at any age, but is more often diagnosed between 10-20 years and over 50 years of age. Without treatment, 43% of patients develop cirrhosis within three years. In most patients, immunosuppressive therapy yields good results; stable remission after discontinuation of drugs is observed in 20% of patients. This type of AIH is most common in the United States and Western Europe.

Type 2 (anti-LKM-l positive)

It is observed much less frequently, it accounts for 10-15% of the total number of AIH cases. Mostly children are ill (from 2 to 14 years old). This form of the disease is characterized by a stronger biochemical activity, liver cirrhosis within three years is formed 2 times more often than in type 1 hepatitis.

Type 2 is more resistant to drug immunotherapy, and stopping the drug usually leads to a relapse. More often than with type 1, there is a combination with other immune diseases (vitiligo, thyroiditis, insulin-dependent diabetes mellitus, ulcerative colitis). In the United States, type 2 is diagnosed in 4% of adult patients with AIH, while type 1 is diagnosed in 80%. It should also be noted that 50-85% of patients with type 2 of the disease and only 11% with type 1 suffer from viral hepatitis C.

Type 3 (anti-SLA positive)

With this type of AIH, antibodies to the liver antigen (SLA) are formed. Quite often, with this type, rheumatoid factor is detected. It should be noted that 11% of patients with type 1 hepatitis also have anti-SLA; therefore, it remains unclear whether this type of AIH is a type 1 type or should be isolated as a separate type.

In addition to traditional types, sometimes there are forms that, in parallel with the classical clinic, may have signs of chronic viral hepatitis, primary biliary cirrhosis or primary sclerosing cholangitis. These forms are called cross-autoimmune syndromes.

Symptoms of autoimmune hepatitis

In about 1/3 of cases, the disease begins suddenly, and its clinical manifestations are indistinguishable from those of acute hepatitis. Therefore, the diagnosis of viral or toxic hepatitis is sometimes mistakenly made. Severe weakness appears, there is no appetite, urine becomes dark in color, intense jaundice is observed.

With the gradual development of the disease, jaundice may be insignificant, periodically there is severity and pain on the right under the ribs, vegetative disorders play a predominant role.

At the peak of symptoms, nausea, pruritus, lymphadenopathy (swollen lymph nodes) are added to the above symptoms. Pain and jaundice are intermittent, aggravated during exacerbations. Also, during exacerbations, signs of ascites (accumulation of fluid in the abdominal cavity) may appear. There is an increase in the liver and spleen. Against the background of autoimmune hepatitis, 30% of women develop amenorrhea, hirsutism (increased hair growth) is possible, and gynecomastia in boys and men.

Typical skin reactions are capillaritis, erythema, telangiectasias (spider veins) on the face, neck, arms, and acne, since almost all patients have abnormalities in the endocrine system. Hemorrhagic rash leaves behind pigmentation.

The systemic manifestations of autoimmune hepatitis include polyarthritis of large joints. This disease is characterized by a combination of liver damage and immune disorders. There are diseases such as ulcerative colitis, myocarditis, thyroiditis, diabetes, glomerulonephritis.

At the same time, in 25% of patients, the disease is asymptomatic in the early stages and is detected only at the stage of liver cirrhosis. If there are signs of any acute infectious process (herpesvirus type 4, viral hepatitis, cytomegalovirus), the diagnosis of autoimmune hepatitis is questioned.

Diagnostics

The diagnostic criteria for the disease are serological, biochemical and histological markers. Research methods such as ultrasound, MRI of the liver do not play a significant role in terms of diagnosis.

The diagnosis of "autoimmune hepatitis" can be made under the following conditions:

• There is no history of blood transfusion, taking hepatotoxic drugs, recent alcohol consumption;
• The level of immunoglobulins in the blood exceeds the norm by 1.5 times or more;
• No markers of active viral infections (hepatitis A, B, C, Epstein-Barr virus, cytomegalovirus) were found in blood serum;
• Antibody titers (SMA, ANA and LKM-1) exceed 1:80 for adults and 1:20 for children.

The final diagnosis is confirmed by liver biopsy results. Histological examination should reveal stepped or bridging tissue necrosis, lymphoid infiltration (accumulation of lymphocytes).

Autoimmune hepatitis must be differentiated from chronic viral hepatitis, Wilson's disease, drug and alcoholic hepatitis, non-alcoholic fatty liver disease, cholangitis, primary biliary cirrhosis. Also, the presence of such pathologies as damage to the bile ducts, granulomas (nodules formed against the background of an inflammatory process) is unacceptable - most likely, this indicates some other pathology.

AIH differs from other forms of chronic hepatitis in that in this case, in order to make a diagnosis, one does not need to wait until the disease becomes chronic (that is, about 6 months). AIH can be diagnosed at any time during its clinical course.

Autoimmune hepatitis treatment

The therapy is based on the use of glucocorticosteroids - immunosuppressive drugs (suppressing immunity). This helps to reduce the activity of autoimmune reactions that destroy liver cells.

Currently, there are two treatment regimens: combined (prednisolone + azathioprine) and monotherapy (high doses of prednisolone). Their effectiveness is about the same, both schemes allow you to achieve remission and increase the survival rate. However, combination therapy is characterized by a lower incidence of side effects, which is 10%, while with treatment with prednisolone alone, this figure reaches 45%. Therefore, if azathioprine is well tolerated, the first option is preferable. Especially combination therapy is indicated for elderly women and patients with diabetes, osteoporosis, obesity, increased nervous irritability.

Monotherapy is prescribed for pregnant women, patients with various neoplasms, suffering from severe forms of cytopenia (deficiency of certain types of blood cells). With a course of treatment not exceeding 18 months, pronounced side effects are not observed. During treatment, the dose of prednisone is gradually reduced. The duration of treatment for autoimmune hepatitis is from 6 months to 2 years, in some cases, therapy is carried out throughout life.

Indications for steroid therapy

Treatment with steroids is necessarily prescribed in case of disability, as well as detection of bridging or stepwise necrosis during histological analysis. In all other cases, the decision is made on an individual basis. The effectiveness of treatment with corticosteroid drugs has been confirmed only in patients with an actively progressive process. In case of mild clinical symptoms, the ratio of benefit and risk is unknown.

In case of ineffectiveness of immunosuppressive therapy, carried out for four years, with frequent relapses and severe side effects, the only solution is liver transplantation.

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Forecast and prevention

If there is no treatment, then autoimmune hepatitis progresses, spontaneous remissions are impossible. The inevitable consequence is liver failure and cirrhosis. The five-year survival rate in this case is within 50%.

With timely and correctly selected therapy, it is possible to achieve stable remission in most patients, the 20-year survival rate in this case is 80%.

The combination of acute liver inflammation with cirrhosis has a poor prognosis: 60% of patients die within five years, 20% - within two years.

In patients with graded necrosis, the incidence of cirrhosis within five years is 17%. If there are no complications such as ascites and hepatic encephalopathy, which reduce the effectiveness of steroid therapy, the inflammatory process in 15-20% of patients self-destructs, regardless of the activity of the course of the disease.

The results of liver transplantation are comparable to the remission achieved with medication: 90% of patients have a favorable 5-year prognosis.

With this disease, only secondary prevention is possible, which consists in regular visits to the gastroenterologist and constant monitoring of the level of antibodies, immunoglobulins and the activity of liver enzymes. Patients with this disease are advised to follow a gentle regimen and diet, limit physical and emotional stress, refuse preventive vaccination, and limit the intake of various medications.

Author of the article: Kletkin Maxim Evgenievich | Hepatologist

Education: Diploma in the specialty "General Medicine" received at the Military Medical Academy. S. M. Kirov (2007). At the Voronezh Medical Academy. NN Burdenko graduated from residency in the specialty "Hepatologist" (2012).