Disease Description (Causes and Symptoms)

Definition/Cause: Autoimmune hepatitis is chronic hepatitis caused by the immune system abnormally attacking the person’s own hepatocytes through immune-mediated mechanisms. It develops without external causes such as specific viral infections, medications, or alcohol, and is commonly accompanied by hypergammaglobulinemia (elevated IgG) and autoantibodies (e.g., ANA, SMA, LKM). Periportal (interface) inflammation and infiltration by plasma cells and lymphocytes are observed in liver tissue.
Pathophysiology: Autoantibody- and T-cell-mediated immune responses continuously damage the hepatic parenchyma, and recurrent inflammation can lead to fibrosis and eventually progress to cirrhosis and liver failure.
Main symptoms: Fatigue, loss of appetite, nausea and vomiting, jaundice, arthralgia and arthritis, generalized edema, and a tendency to bleed (when liver function is impaired), among others. Many cases are asymptomatic and are discovered incidentally through abnormal liver function test results.
Epidemiological characteristics: More common in women (approximately 9:1), occurring mainly in middle-aged people.

Treatment Methods

First-line treatment
- Corticosteroids (e.g., prednisone or prednisolone) alone or in combination with azathioprine are the standard initial treatment. The goals are improvement of symptoms, liver enzyme levels, and histologic inflammation.
- Azathioprine is widely used as a steroid-sparing agent to reduce the steroid dose. (Consider TPMT activity testing before treatment.)
Second-line treatment (when there is no response to first-line medications or they cannot be used because of adverse effects)
- Mycophenolate mofetil, tacrolimus, cyclosporine, multi-targeted therapies (investigational), etc.
End-stage or refractory disease
- Liver transplantation is a therapeutic option in liver failure, refractory jaundice, or end-stage cirrhosis. Recurrence after transplantation is possible but relatively uncommon.
Supportive treatment and lifestyle management
- Abstinence from alcohol; avoidance of hepatotoxic medications, herbal medicines, and folk remedies; infection prevention (vaccination: check A and B vaccines and receive them if necessary); osteoporosis prevention (in cases of long-term steroid use); and management of blood glucose, weight, and blood pressure, etc.
Monitoring during treatment
- Regular testing is necessary for liver function (AST/ALT/bilirubin), albumin and coagulation (PT/INR), blood IgG, follow-up of autoantibodies, and medication adverse effects (hematologic and hepatotoxicity tests).

Items to bring
- A list of all currently taken medications, supplements, and herbal medicines (including doses and times of administration)
- Recent test results (liver function tests, IgG, autoantibody tests, viral hepatitis tests, CBC, PT/INR, etc.) or test referral forms/results
- Previous liver tissue examination (biopsy) reports, and abdominal ultrasound, CT, or MRI results if available
- A record of when symptoms began and how they have changed (jaundice, fatigue, digestive symptoms, etc.) and a history of alcohol consumption
- For women of childbearing age, pregnancy status/plans and contraceptive method
Key information that the patient (or caregiver) should explain to the doctor during the outpatient visit (example questions)
- "I will briefly explain when my symptoms started and how they have progressed."
- "What medications, herbal medicines, and nutritional supplements am I currently taking?"
- "Do I have results from previous viral hepatitis tests (e.g., HBsAg, anti-HCV)?"

"What is the purpose of the prescribed medication, and what is the expected duration of treatment?"
"What are the main side effects of this medication, and what should I do if they occur?"
"Which tests will be used to evaluate my response to treatment, and how often will follow-up be needed?"
"I am planning to become pregnant. What are the effects of the medications (especially azathioprine, steroids, and MMF), and what measures are recommended?"
"How high is the risk of progressing to cirrhosis, and what lifestyle habits can help reduce it?"

"How do you assess the current activity of my disease based on my blood test levels (ALT/AST, IgG) and autoantibodies (ANA, SMA, etc.)? Is a tissue examination (biopsy) necessary?"
"How long do you expect me to use steroid treatment, and when do you plan to start a steroid-sparing agent such as azathioprine? Do I need preliminary tests such as a TPMT test?"

Additional Information

Important precaution: The risk of infection increases during immunosuppressive treatment, so consult a medical institution immediately if you suspect an infection, such as when you develop a fever or respiratory symptoms. Mycophenolate mofetil carries a risk of birth defects during pregnancy, so consultation with medical professionals is essential when planning a pregnancy.