From the Guidelines
Autoimmune hepatitis (AIH) is a chronic liver disease characterized by a heterogeneous clinical presentation, where the immune system attacks liver cells, causing inflammation and damage, and its management involves immunosuppressive medications to improve survival and quality of life. The disease can affect individuals of any age, from small infants to octogenarians, and may present with a wide range of symptoms, including fatigue, jaundice, abdominal discomfort, and elevated liver enzymes 1. According to the EASL clinical practice guidelines, AIH is a major diagnostic and therapeutic challenge due to its rarity and heterogeneity, which limits both diagnostic and therapeutic studies 1.
Key Characteristics of AIH
- Chronic inflammatory liver disease
- Immune system attacks liver cells, causing inflammation and damage
- Heterogeneous clinical presentation
- Affects individuals of any age
- May present with fatigue, jaundice, abdominal discomfort, and elevated liver enzymes
Diagnosis and Treatment
Diagnosis involves blood tests for autoantibodies (ANA, ASMA, anti-LKM), elevated IgG levels, and liver biopsy 1. Treatment typically involves immunosuppressive medications, with prednisone being the primary initial therapy, often combined with azathioprine as a steroid-sparing agent 1. The British Society of Gastroenterology guidelines suggest that treatment usually begins with higher doses that are gradually tapered once remission is achieved, typically over 1-2 years 1.
Management Strategies
- Immunousuppressive medications to improve survival and quality of life
- Regular monitoring of liver enzymes, complete blood counts, and occasional liver biopsies to assess treatment response
- Lifelong maintenance therapy is often necessary as relapse is common when medications are discontinued
- The decision to perform a repeat biopsy must be individualized, considering factors such as the patient's age, reluctance, and liver test normalization 1.
Prognosis and Quality of Life
Without treatment, AIH can progress to cirrhosis, liver failure, and potentially require liver transplantation 1. However, with proper management, patients can achieve excellent prognosis, both in respect to survival and quality of life, as stated in the EASL clinical practice guidelines 1.
From the Research
Definition and Characteristics of Autoimmune Hepatitis (AIH)
- Autoimmune hepatitis (AIH) is a progressive inflammatory hepatitis of unknown etiology that is responsive to immunosuppressive therapy 2
- AIH is characterized by autoimmunologic features, including the presence of circulating autoantibodies and high serum globulin concentrations 3
- It is an immune-mediated chronic liver disease that can progress to cirrhosis and fulminant hepatic failure 4
Diagnosis and Treatment of AIH
- The diagnosis of AIH should be guided by the descriptive criteria and scoring system set forth by the International Autoimmune Hepatitis Group 2
- Standard therapy for AIH includes corticosteroids alone or in combination with azathioprine 2, 4
- Combination therapy with prednisone and azathioprine is preferred, as it allows treatment with lower individual doses of each drug and is thus associated with fewer side effects 2
- Alternative treatments, such as mycophenolate mofetil (MMF), may be considered for patients who are intolerant or non-responsive to standard therapy 4, 5, 6
Treatment Outcomes and Management
- Treatment outcomes for AIH include complete biochemical, clinical, and histologic remission, incomplete response, or treatment failure 2
- Treatment withdrawal, once remission has been attained, may be associated with relapse of disease 2
- Recurrent relapse may be addressed with long-term, lower dose maintenance treatment with prednisone or azathioprine 2
- Management of recurrent AIH post-liver transplantation remains controversial, with insufficient data to support long-term steroid use 6