Treatment of Autoimmune Hepatitis Type 2
Initial Treatment Regimen
- The American College of Gastroenterology recommends combination therapy with prednisone (30 mg/day initially, tapering to 10 mg/day over 4 weeks) plus azathioprine (1-2 mg/kg/day) for at least 2 years and for at least 12 months after normalization of liver enzymes, with a strength of evidence based on clinical guidelines 1
- Combination therapy from the outset produces fewer corticosteroid-related side effects compared to prednisone monotherapy (10% versus 44%) 1
- The initial dosing schedule is: Prednisone 30 mg/day + Azathioprine 50 mg/day (US) or 1-2 mg/kg/day (Europe) for week 1, tapering to Prednisone 10 mg/day + Azathioprine (same dose) for maintenance 1
Monitoring and Response Assessment
- Assess treatment response at 4-8 weeks after initiation, with serum aminotransferase levels improving within 2 weeks of starting therapy 1, 2
- Monitor serum aminotransferase levels monthly, as small decrements in prednisone dose can be associated with marked increases in aminotransferase levels 3
Treatment Duration and Endpoints
- Continue treatment until remission is achieved, defined as normal laboratory indices and resolution of liver inflammation on biopsy, with an average duration of initial treatment of 18-24 months 1
- Complete normalization of transaminases and IgG levels should be the aim of treatment, with biochemical remission achieved within 6 months associated with lower frequency of progression to cirrhosis 2
Management of Non-Response
- For non-responding patients, consider higher doses of steroids combined with azathioprine 2 mg/kg/day, or alternative options such as tacrolimus or cyclosporine for steroid-refractory cases 1
- Treatment failure should be managed with high doses of prednisone alone (60 mg daily) or prednisone (30 mg daily) in conjunction with azathioprine (150 mg daily), continued for at least 1 month 3
Long-Term Maintenance After Relapse
- Relapse occurs in 50-90% of patients within 12 months of stopping treatment, and after relapse, consider long-term maintenance with azathioprine 2 mg/kg/day 1
- For patients who have relapsed more than once, treat with combination prednisone and azathioprine therapy, low-dose prednisone, or azathioprine only, with 87% of adult patients remaining in remission during a median observation interval of 67 months 3
Prevention of Treatment Complications
- All patients should receive calcium and vitamin D supplementation, and bone mineral density should be monitored with DEXA scanning at 1-2 year intervals, with vaccination against hepatitis A and B performed early in susceptible patients 1
- Severe complications (osteoporosis, vertebral compression, diabetes, cataracts, hypertension, psychosis) typically develop after 18 months of therapy at prednisone doses >10 mg daily 2