From the Guidelines
The initial treatment of Autoimmune Hepatitis (AIH) should be prednisolone plus azathioprine, with the recommended regime being prednisolone initially 30 mg/day (reducing to 10 mg/day over 4 weeks) plus azathioprine 1 mg/kg/day 1. The treatment goals for AIH include normalizing transaminases and achieving histological remission.
- Initial diagnosis requires liver function tests, autoantibody testing (ANA, ASMA, anti-LKM1), and liver biopsy to confirm the diagnosis and assess disease severity.
- Prednisone is usually tapered over several weeks once improvement is seen, while azathioprine is maintained for long-term management.
- For patients who don't respond to standard therapy, second-line options include mycophenolate mofetil, tacrolimus, or cyclosporine.
- Treatment should continue for at least 2-3 years after biochemical remission before considering withdrawal, with close monitoring for relapse.
- Lifelong therapy is often necessary for patients with cirrhosis or those who relapse after treatment withdrawal.
- Regular monitoring of liver enzymes every 3-6 months is essential during maintenance therapy. The most recent guidelines from the American Association for the Study of Liver Diseases (AASLD) in 2020 provide guidance on the diagnosis and management of AIH in adults and children, including recommendations for glucocorticoid and azathioprine therapy as first-line management 1.
- The AASLD guidelines recommend using the Grading of Recommendations Assessment, Development, and Evaluation (GRADE) system to categorize each recommendation as strong or conditional.
- The guidelines also address second-line medications after failure of first-line therapy and maintenance management after liver transplantation (LT). Overall, AIH treatment is crucial as untreated disease can progress to cirrhosis, liver failure, and increased mortality, while appropriate immunosuppression can effectively control inflammation and prevent progression in most patients 1.
From the Research
Autoimmune Hepatitis (AIH) Management Guidelines
The management of Autoimmune Hepatitis (AIH) involves the use of immunosuppressive therapy to induce remission. The American Association for the Study of Liver Diseases (AASLD) recommends initial treatment with a glucocorticoid alone or a combination of a glucocorticoid and an immunosuppressant 2.
First-Line Treatment
- The standard of care for AIH is a prednisone/azathioprine combination, which has been well established for decades and significantly improves long-term survival 3.
- Budesonide, an alternative topical steroid with fewer systemic side effects, was approved for AIH therapy in non-cirrhotic patients in 2011 3.
- The therapeutic goal of complete biochemical remission is achieved in about 80% of patients, and liver transplantation is rarely necessary 3.
Second-Line Treatment
- Second-line immunosuppressive therapies are used for patients who are unresponsive to first-line treatment 4.
- The most studied second-line immunosuppressive is mycophenolate mofetil (MM), with a reasonable histological remission rate of 88.6% 4.
- Other second-line immunosuppressants include tacrolimus, cyclosporine, and budesonide, which have been shown to be effective in normalizing aminotransferases 4.
Prednisone Dosage
- The European Association for the Study of the Liver clinical practice guidelines advise a predniso(lo)ne dose range of 0.50-1 mg/kg/day 5.
- A study found that an initial predniso(lo)ne dose below 0.50 mg/kg/day substantially decreases unnecessary exposure to predniso(lo)ne in patients with AIH, with no significant difference in rates of normalization of transaminases between the high-dose and low-dose groups 5.