From the Guidelines
The first-line treatment for autoimmune hepatitis (AIH) should be prednisolone plus azathioprine, as recommended by the most recent guidelines 1. The treatment for AIH depends on the specific type and severity of the condition.
- First-line treatment typically involves corticosteroids, with prednisone being the most common, and azathioprine as an adjunct therapy.
- The recommended initial dose of prednisolone is 30 mg/day, reducing to 10 mg/day over 4 weeks, plus azathioprine 1 mg/kg/day 1.
- Higher initial doses of prednisolone (up to 1 mg/kg/day) may be used, but caution is advised in frail elderly patients.
- For patients who don't respond to steroids, alternative treatments such as tacrolimus or mycophenolate mofetil may be considered 1.
- In patients with liver failure, bridging necrosis on biopsy, or in jaundiced patients whose MELD score does not rapidly improve on treatment, contact should be made with a liver transplant centre 1.
- Treatment with azathioprine 1 mg/kg/day and prednisolone 5-10 mg/day should continue for at least 2 years and for at least 12 months after normalization of transaminases 1.
- Patients should receive calcium and vitamin D supplementation, and bone DEXA scanning should be performed at 1-2-yearly intervals while on steroids 1.
- Liver biopsy to confirm histological remission is of value in planning further management 1.
- The most recent guidelines from the American Association for the Study of Liver Diseases (AASLD) in 2019 recommend that treatment should aim to reduce antibody production and immune-mediated destruction of liver cells, allowing the liver to recover 1.
- Regular monitoring of liver function tests, complete blood counts, and markers of liver inflammation is essential to assess treatment response and guide therapy adjustments 1.
From the Research
Treatment Options for AIHA
- The treatment of autoimmune hemolytic anemia (AIHA) varies depending on the type of AIHA, with warm antibody type, cold agglutinin syndrome, paroxysmal cold hemoglobinuria, and secondary AIHA requiring different approaches 2.
- Initial therapy for warm antibody AIHA is typically corticosteroids, such as prednisone, at conventional doses of 1 to 1.5 mg/kg/d orally 2, 3, 4, 5.
- Splenectomy is a second-line treatment option for warm AIHA, with the potential for complete and long-term remission, but it carries the risk of overwhelming postsplenectomy infection 2, 3, 6, 4.
- Rituximab is a useful alternative for steroid-refractory patients or those requiring high maintenance doses, and it is becoming the preferred second-line treatment for warm AIHA 3, 6, 4, 5.
- For cold agglutinin disease, rituximab is now recommended as first-line treatment, with or without bendamustine 3, 4, 5.
- Other therapeutic options for AIHA include immunosuppressive drugs, danazol, intravenous immunoglobulin, and plasma exchange, but these are generally considered less effective or reserved for severe or refractory cases 2, 3, 4.
Treatment Approaches by AIHA Type
- Warm AIHA: corticosteroids as first-line therapy, followed by splenectomy or rituximab as second-line options 2, 3, 6, 4, 5.
- Cold agglutinin disease: rituximab as first-line treatment, with or without bendamustine 3, 4, 5.
- Paroxysmal cold hemoglobinuria: aggressive supportive therapy, generally supplemented by corticosteroids 2.
- Secondary AIHA: treatment of the underlying disorder, in addition to therapy for AIHA 2, 5.