Autoimmune Hepatitis - Symptoms

Autoimmune hepatitis occurs predominantly in young people; half of the patients are between 10 and 20 years old. The second peak of the disease is observed during menopause. Three quarters are women.

In most cases, the pattern of liver disease is inconsistent with the established duration of symptoms. Chronic hepatitis may remain asymptomatic for months (and possibly years) before jaundice becomes apparent and the diagnosis can be made. The disease may be recognized earlier if routine testing reveals stigmata of liver disease or if liver function tests are abnormal.

Some patients are jaundiced, although serum bilirubin levels are usually elevated. Overt jaundice is often episodic. Rarely, marked cholestatic jaundice occurs.

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

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

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, etc.

During the period of advanced symptoms of autoimmune hepatitis, the following clinical manifestations are characteristic:

1. The main complaints of patients are severe general weakness, fatigue, significant reduction in work capacity; loss of appetite, pain and a feeling of heaviness in the right hypochondrium, nausea; the appearance of jaundice, skin itching; joint pain; increased body temperature; the appearance of various rashes on the skin; menstrual irregularities.
2. During examination of patients, jaundice of varying severity, skin changes (hemorrhagic exanthema in the form of clear dots or spots, after their disappearance pigment spots remain; lupus-like erythema, often a "butterfly" on the face; erythema nodosum; focal scleroderma); lymphadenopathy are detected. In severe cases of the disease, spider veins and hyperemia of the palms (palmer liver) appear.
3. Damage to the digestive system.

The most characteristic symptom of autoimmune hepatitis is hepatomegaly. The liver is painful, its consistency is moderately dense. The spleen may be enlarged (not in all patients). During periods of pronounced hepatitis activity, transient ascites is observed. Almost all patients have chronic gastritis with reduced secretory function.

It should be noted that some patients, usually during periods of exacerbation of the disease, experience episodes of reversible “minor” liver failure.

4. Extrahepatic systemic manifestations.

Autoimmune hepatitis is a systemic disease that affects many organs and systems in addition to the liver. Patients are diagnosed with myocarditis, pericarditis, pleurisy, ulcerative colitis, glomerulonephritis, iridocyclitis, Sjogren's syndrome, autoimmune thyroiditis, diabetes mellitus, Cushing's syndrome, fibrosing alveolitis, hemolytic anemia.

In people with severe disease, hyperthermia may occur. Such patients may also have acute recurrent polyarthritis involving large joints, which is not accompanied by their deformation and is of a migratory nature. In most cases, pain and stiffness are observed without noticeable swelling. Usually, such changes completely resolve.

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

Cutaneous manifestations include allergic capillaritis, acne, erythema, lupus-like changes, and purpura.

Splenomegaly without portal hypertension may be noted, often with generalized lymphadenopathy, apparently 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. Impoverished pulmonary markings on the chest radiograph 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 detected. Another possible variant is fibrosing alveolitis.

Primary pulmonary hypertension has been described in only one patient with multisystem involvement.

Endocrine changes include Cushingoid appearance, acne, hirsutism, and striae. Boys may develop gynecomastia. Hashimoto's thyroiditis and other thyroid abnormalities, including myxedema and thyrotoxicosis, may develop. Patients may develop diabetes mellitus both before and after diagnosis of chronic hepatitis.

Mild anemia, leukopenia, and thrombocytopenia are associated with an enlarged spleen (hypersplenism). Hemolytic anemia with a positive Coombs test is another rare complication. Rarely, eosinophilic syndrome is associated with chronic hepatitis.

Non-specific 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 usually develop not simultaneously with the symptoms of hepatitis, but much later.

In contrast, in systemic lupus erythematosus, with which autoimmune hepatitis often has to be differentiated, extrahepatic symptoms dominate the clinical picture and develop significantly earlier than hepatitis symptoms.

Characteristic signs of autoimmune chronic hepatitis

• Usually women get sick
• Age 15-25 years or menopause
• Serum: 10-fold increase in transaminase activity, 2-fold increase in gamma globulin level
• Liver biopsy: active non-specific process
• Antinuclear antibodies > 1:40, diffuse
• Antibodies to actin > 1:40
• Good response to corticosteroids
• The serum alpha-fetoprotein level in a third of patients may be more than 2 times higher than the upper limit of normal. Its concentration decreases with corticosteroid therapy.

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