Autoimmune hepatitis: symptoms

Autoimmune hepatitis occurs mainly in young people; the age of half of patients is from 10 to 20 years. The second peak of the disease is observed in the menopause period. Three quarters are women.

In most cases, the picture of liver damage does not match the established duration of the symptomatology. Chronic hepatitis can remain asymptomatic for several months (and possibly even years) until the moment when jaundice becomes obvious and a diagnosis can be made. The disease can be recognized earlier if a routine study reveals the stigma of a liver disease or the results of a biochemical study of liver function differ from the norm.

In some patients, jaundice is absent, despite the fact that the serum bilirubin level usually rises. Explicit jaundice is often episodic. Occasionally, severe cholestatic jaundice is observed.

The onset of the disease may be different. There are two variants of the onset of autoimmune hepatitis.

In some patients, the disease begins as acute viral hepatitis (weakness, lack of appetite, appearance of dark urine, then intense jaundice with severe hyperbilirubinemia and high levels of aminotransferases in the blood), further within 1-6 months. A clear clinical picture of autoimmune hepatitis is being developed.

The second variant of the onset of the disease is characterized by the predominance of extrahepatic manifestations and fever in the clinical picture, which leads to an erroneous diagnosis of systemic lupus erythematosus, rheumatism, rheumatoid arthritis, sepsis,

In the period of detailed symptoms of autoimmune hepatitis, the following clinical manifestations are characteristic:

1. The main complaints of patients - a pronounced general weakness, fatigue, a significant decrease in ability to work; a decrease in appetite, pain and a feeling of heaviness in the right hypochondrium, nausea; the appearance of jaundice, itchy skin; pain in the joints; increased body temperature; the appearance of various rashes on the skin; violation of the menstrual cycle.
2. When examining patients, jaundice of varying severity, skin changes (hemorrhagic exanthema in the form of clear spots or spots, after their disappearance, pigment spots remain, lupus-like erythema, often "butterfly" on the face, erythema nodosum, focal scleroderma); lymphadenopathy. In severe disease, there are vascular sprouts, palmar hyperemia (palmer liver).
3. The defeat of the digestive system.

The most characteristic sign of autoimmune hepatitis is hepatomegaly. The liver is painful, its consistency is moderately dense. Perhaps an increase in the spleen (not in all patients). During periods of severe hepatitis activity, transient ascites is observed . Virtually all patients have chronic gastritis with a decreased secretory function.

It should be noted that in some patients, as a rule, during periods of exacerbation of the disease, episodes of reversible "small" liver failure are noted.

4. Extrahepatic system manifestations.

Autoimmune hepatitis is a systemic disease in which, in addition to the liver, many organs and systems are affected. Myocarditis, pericarditis, pleurisy, ulcerative colitis, glomerulonephritis, iridocyclitis, Sjogren's syndrome, autoimmune thyroiditis, diabetes mellitus, Cushing syndrome, fibrosing alveolitis, hemolytic anemia are detected in patients.

Hyperthermia can occur in persons with a severe course of the disease. Such patients can also have acute recurrent polyarthritis with involvement of large joints, which is not accompanied by their deformation and is of a migratory nature. In most cases, pain and stiffness are noted without noticeable swelling. Usually such changes are completely resolved.

The most important of the extrahepatic systemic manifestations is glomerulonephritis, but it most often develops in the terminal stage of the disease. A kidney biopsy often reveals mild glomerulitis. Deposits of immunoglobulins and complement can be found in glomeruli. Complexes containing small nuclear ribonucleoproteins and IgG are characteristic mainly for patients with kidney diseases. Glomerular antibodies are detected in almost half of patients, but do not correlate with the volume of renal damage.

Skin manifestations include allergic capillaritis, acne, erythema, changes in the type of lupus erythematosus and purpura.

Splenomegaly can be noted without portal hypertension, often with generalized lymphadenopathy, apparently being part of a single process of lymphoid hyperplasia.

In the active phase of the disease, changes in the lungs are detected, including pleurisy, migratory pulmonary infiltrates and atelectasis. The depletion of the pulmonary pattern on the roentgenogram of the chest may be due to dilated precapillaries. High cardiac output in chronic liver disease "contributes" to the development of pulmonary hypervolemia. Multiple pulmonary arteriovenous anastomoses are also found. Another possible option is fibrosing alveolitis.

Primary pulmonary hypertension is described only in one patient with multisystemic lesion.

Endocrine changes include cushingoid appearance, acne, hirsutism and skin striae. Boys may develop gynecomastia. Perhaps the development of thyroiditis Hashimoto and other abnormalities on the part of the thyroid gland, including myxedema and thyrotoxicosis. Both before and after the diagnosis of chronic hepatitis in patients may develop diabetes.

Light anemia, leukopenia and thrombocytopenia are associated with an enlarged spleen (hypersplenism). Hemolytic anemia with a positive Coombs breakdown is another rare complication. Occasionally, chronic ectopic syndrome is associated with chronic hepatitis.

Nonspecific ulcerative colitis can occur together with chronic active hepatitis or complicate its course.

There are reports of the development of hepatocellular carcinoma, but this is very rare.

It should be emphasized that extrahepatic manifestations of autoimmune hepatitis, as a rule, do not dominate the clinical picture of the disease and develop usually not simultaneously with the symptoms of hepatitis, but much later.

In contrast, with systemic lupus erythematosus, which often has to differentiate autoimmune hepatitis, extrahepatic symptoms dominate the clinical picture and develop much earlier than the symptoms of hepatitis.

Characteristic signs of autoimmune chronic hepatitis

• Women are usually sick
• Age 15-25 years or the period of menopause
• Serum: an increase in the activity of transaminases 10 times the increase in the level of y-globulin in 2 times
• Liver biopsy: an active nonspecific process
• Antinuclear antibodies> 1:40, diffuse
• Antibodies to actin> 1:40
• A good response to corticosteroids
• The serum a-fetoprotein level in a third of patients may more than double the upper limit of the norm. When carrying out corticosteroid therapy, its concentration decreases.

[1], [2], [3], [4]