From the Guidelines
The prognosis of autoimmune hepatitis, even with liver lesions, is generally favorable with appropriate treatment, which typically involves corticosteroids like prednisone followed by maintenance therapy with azathioprine, as outlined in the EASL clinical practice guidelines 1. The goal of treatment is to achieve complete normalization of transaminases and IgG levels, as persisting elevations are predictive of relapse, activity on liver biopsy, progression to cirrhosis, and poor outcome 1. Key aspects of management include:
- Initial therapy with prednisolone, followed by the addition of azathioprine after two weeks, as first-line treatment of AIH 1
- Regular monitoring with liver function tests every 3-6 months and periodic imaging to evaluate liver lesions and disease progression
- Consideration of liver transplantation for end-stage liver disease, with good post-transplant outcomes despite a risk of disease recurrence Without treatment, autoimmune hepatitis can progress to cirrhosis, liver failure, and potentially hepatocellular carcinoma, emphasizing the importance of prompt and effective management 1. The 10-year survival rate with appropriate treatment exceeds 80%, although patients with advanced fibrosis or cirrhosis at diagnosis have a less favorable prognosis, highlighting the need for early diagnosis and intervention 1.
From the Research
Prognosis of Autoimmune Hepatitis with Liver Lesions
The prognosis of autoimmune hepatitis, particularly when liver lesions are present, is a complex topic.
- The standard treatment for autoimmune hepatitis consists of steroids with the addition of azathioprine, which results in remission in approximately 80% of patients 2.
- Despite significant advancements in the understanding of the immune system, few modifications have been made to treatment algorithms, which have remained largely unchanged since they were first proposed more than 40 years ago 2.
- For patients who do not respond to standard treatment, second-line therapies such as mycophenolate mofetil (MMF) may be considered 3, 4, 5, 6.
- MMF has been shown to be effective in patients who are intolerant to azathioprine, with remission rates of 81.5% in one study 6.
- However, the efficacy of MMF is considerably lower in patients who are switched to MMF due to an insufficient response to first-line treatment, with remission rates of 30% in one study 6.
Treatment Options
- Other treatment options for autoimmune hepatitis include:
- Cyclosporin A, a calcineurin inhibitor that decreases T cell activation 2.
- Tacrolimus, a drug belonging to the same family as cyclosporin A, which has been used in patients with refractory diseases with fewer side effects 2.
- Sirolimus and everolimus, which have interesting effects on regulatory T cell populations and may become viable options in the future 2.
- B cell-depleting drugs, such as rituximab and belimumab, which have been successfully used in refractory cases and are useful in both the short and long term 2.
- Anti-tumor necrosis factors, Janus kinases inhibitors, and chimeric antigen receptor T cell therapy, which are promising treatments that may allow for a more tailored approach to the treatment of autoimmune hepatitis in the near future 2.
Disease Management
- The therapeutic aim of autoimmune hepatitis is the complete normalization of aminotransferases and immunoglobulin G 4.
- The majority of patients require life-long immunosuppressive therapy because of high relapse rates after discontinuation of immunosuppressants 4.
- Liver transplantation is rarely necessary, and the therapeutic goal of complete biochemical remission is achieved in about 80% of patients 4.