Abstract
Autoimmune hepatitis is a rare and chronic liver disease that is characterised by increased serum transaminases and immunoglobulin G, inflammatory liver histology and presence of circulating autoantibodies. An autoimmune hepatitis diagnosis justifies life-long treatment in most patients in order to prevent development of cirrhosis and end-stage liver disease. The cornerstone of treatment is steroid induction therapy followed by maintenance therapy with azathioprine, which is effective in most cases. For patients who do not respond to standard treatment, second-line treatment with other immunosuppressants can be effective. Treatment should be aimed at biochemical remission of the disease, which is defined as normalization of transaminases and immunoglobulin G. Patients should be monitored intensively during the first months of treatment in order to monitor side-effects, assess symptoms and individualise treatment. Specialist consultation should be sought in difficult-to-treat patients. Future studies and networking initiatives should result in optimization of current treatment strategies in autoimmune hepatitis.
Highlights
Autoimmune hepatitis (AIH) is a chronic inflammatory liver disease that predominantly affects women, but can occur in all ages and races
The exact cause of AIH is unknown, it is hypothesised that loss of tolerance against liver antigens is the main pathophysiological mechanism, which is triggered by environmental factors in individuals with a certain genetic susceptibility.[1]
AIH is characterised by hypergammaglobulinaemia, circulating auto-antibodies and distinctive histology
Summary
Autoimmune hepatitis (AIH) is a chronic inflammatory liver disease that predominantly affects women, but can occur in all ages and races. AIH is characterised by hypergammaglobulinaemia, circulating auto-antibodies and distinctive histology. Based on these characteristics, the International Autoimmune Hepatitis Group (IAIHG) has established diagnostic criteria (Table 1) that aid physicians in establishing a correct AIH diagnosis. A diagnosis of AIH remains a clinical one, since a gold standard for diagnosis is lacking.[2,3]. Even though establishing the diagnosis of AIH is sometimes complex and cumbersome, clinical management of AIH can be a challenging journey, given the lifelong therapy and potential side-effects. We will discuss the clinical management of adult AIH patients and its latest developments, based on recent literature. Our aim is to assist the general gastroenterologist and hepatologist in the management of AIH, once an AIH diagnosis has been confirmed
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