Disease Description (Causes and Symptoms)
- Definition and causes: This is chronic hepatitis caused by the immune system abnormally attacking its own liver cells. It is known to develop through interactions between genetic predisposition and environmental triggers (e.g., infections and medications), without a single clearly defined cause. Pathological findings are characterized by inflammation around the portal tracts (interface hepatitis) and infiltration of plasma cells and lymphocytes.
- Pathophysiology: It is accompanied by autoantibodies (e.g., ANA, SMA, anti‑LKM1, etc.) and hypergammaglobulinemia (IgG), and persistent immune-mediated liver cell damage may progress to fibrosis and cirrhosis.
- Main symptoms: Fatigue, malaise, loss of appetite, nausea and vomiting, jaundice, itching, arthralgia/arthritis, generalized edema, and a tendency to bleed (when liver function is impaired). It is also often detected without symptoms.
- Key diagnostic points: Exclusion of viral, alcoholic, and metabolic liver diseases; blood tests (elevated ALT/AST, elevated IgG, autoantibody tests); liver ultrasound and imaging tests; and liver biopsy (interface hepatitis and plasma cell infiltration). International scoring systems (IAIHG) or diagnostic criteria are used comprehensively.
Treatment Methods
- Drug treatment (standard treatment)
- First-line: Corticosteroids (e.g., prednisone) alone or in combination with azathioprine. After initial induction therapy, the dose is gradually reduced for maintenance.
- Alternatives and adjuncts: If there is intolerance or inadequate response to azathioprine, other immunosuppressants such as mycophenolate mofetil (MMF), tacrolimus, or cyclosporine may be considered. In patients without cirrhosis, budesonide plus azathioprine may be used (according to applicable local guidelines).
- Procedures/Surgery
- In acute liver failure or end-stage cirrhosis, liver transplantation is the only survival strategy. Recurrence after transplantation is possible but manageable.
- Lifestyle management and prevention
- Abstinence from alcohol (strongly recommended), avoidance of hepatotoxic medications, and recommended vaccinations: hepatitis A and B vaccinations, and influenza and pneumococcal vaccines (caution with live vaccines during immunosuppression). Avoid live vaccines during immunosuppressive treatment or consult the attending physician.
- Prevention of steroid side effects: Manage the risk of osteoporosis (vitamin D, calcium, and bone density testing), and monitor blood glucose, weight, and blood pressure.
- Infection prevention (during immunosuppression): Hand hygiene, caution regarding contact-transmitted infections, and preventive testing when necessary (e.g., tuberculosis and hepatitis B markers).
- Treatment duration and prognosis: Treatment often continues for several years, and the risk of relapse is high when medications are discontinued. The prognosis is favorable with appropriate control, but untreated disease may progress to cirrhosis and liver failure.
Checklist Before an Outpatient Visit (Items to Bring, Patient Preparation, and Sample Questions)
- Items to bring/submit
- A list of all current medications, supplements, and herbal medicines (including doses and duration of use)
- Previous blood test results (liver enzyme levels, IgG, and autoantibody tests), liver biopsy results, and ultrasound/CT/MRI reports
- A record of changes in symptoms (fatigue, jaundice, itching, weight changes, etc.) and notes on bleeding, bruising, or ascites
- Recent vaccination history and whether pregnancy or breastfeeding is planned
- Tests expected during the outpatient visit: LFTs (ALT/AST, ALP, bilirubin), IgG, blood coagulation tests (PT/INR), autoantibodies (ANA, SMA, anti‑LKM, etc.), liver ultrasound, etc.
- Examples of questions the patient may be asked (what the doctor may ask)
- When and how did your symptoms begin? Are there factors that make them worse or better?
- What medications have you taken recently (especially prescription drugs, over-the-counter drugs, and herbal products)?
- What is your alcohol use history and family history (liver disease or autoimmune disease)?
Examples of General Questions to Ask the Doctor
- What are the major side effects of these medications (steroids/azathioprine), and how can they be prevented?
- What are the treatment goals, and how will the treatment response be assessed (which tests and criteria)?
- How long should I take the medication, and what are the criteria for reducing or discontinuing it?
- I am planning a pregnancy. Do I need to change my medication or follow any safety precautions?
- Which vaccinations are needed to prevent infections, and when should I receive them?
Examples of Disease-Specific Questions
- How should my blood test results (ANA, SMA, anti‑LKM, IgG) be interpreted, and are treatment initiation or discontinuation decisions made based on these levels?
- When symptoms improve with steroids, what are the criteria for safely reducing or discontinuing them (such as clinical symptoms and the duration of normalization of LFTs and IgG)?