The disease is similar to other autoimmune diseases in many ways and can be seen together with other autoimmune diseases. It is common in young women. It causes chronic liver inflammation with extrahepatic findings.
Clinic
• Patients may present with asymptomatic liver enzyme elevation, chronic hepatitis findings or rarely acute hepatitis.
• As extrahepatic findings;
Acne, anorexia, hirsutism, amenorrhea, arthralgia, maculopapular rash, erythema nodosum, colitis, pericarditis, pleuritis and sicca syndrome may accompany.
Laboratory and Diagnosis
• Mild-moderate ALT and AST elevation and polyclonal hypergammaglobulinemia (IgG elevation) are frequently seen.
• It is divided into two main groups as Type 1 and Type 2:
Type I autoimmune hepatitis is the most common.
Antinuclear antibody (ANA) and anti-smooth muscle antibody (ASMA) positivity are associated with type 1 autoimmune hepatitis.
Anti-LKM1 (liver kidney microsomal antibody) and Anti-LC1 (liver cytosol) are seen in type 2. (LKM-2 is drug related, LKM-3 is HDV related).
Anti-LKM 1 can sometimes be seen in the course of HCV.
• In the biopsy, there is interface hepatitis with an increase in lymphocytes and plasma cells.
• In cases where viral hepatitis and other causes are excluded, the diagnosis is made by combining clinical findings, autoantibody positivity, IgG elevation and biopsy findings.
Treatment
• First choice is corticosteroid therapy.
• Azathioprine is added in the treatment of resistant and recurrent cases.
• Liver transplantation is performed in cases where medical treatment is insufficient and cirrhosis progresses.