Article Text
Statistics from Altmetric.com
Autoimmune hepatitis (AIH) is a disease of unknown aetiology but is presumed to have a basis in aberrant autoreactivity. It is characterised by hypergammaglobulinaemia due mainly to selective elevation of serum IgG, a wide range of circulating tissue autoantibodies, an association with other autoimmune disorders (particularly thyroid disease) in the patients or their first degree relatives, a picture of periportal (interface) hepatitis on liver biopsy with a predominantly lymphoplasmacytic necroinflammatory infiltrate without cholangiolitic or other changes normally associated with liver diseases of other aetiologies, and usually a notable response to immunosuppressive therapy.1 ,2 In common with other autoimmune diseases, it is also associated with inheritance of the HLA B8-DR3 haplotype and, particularly, with the DR3 and DR4 allotypes.3 Diagnosis is based on the combination of these features together with careful exclusion of all other possible causes of liver disease.
AIH is notably heterogeneous with respect to its presentation, severity and outcome.4 Presenting features vary from completely asymptomatic (the disease being revealed at routine health screening), through an insidious onset with signs and symptoms fluctuating with a periodicity of anything from a few months to one or two years, to a severe acute (occasionally fulminant) hepatitis that can be difficult to distinguish clinically from acute viral hepatitis. Although women predominate (4:1) it also affects men and it may present at any age—with two peaks of onset: peripubertally and between the fourth and sixth decades of life (fig 1). Younger patients usually have quite severe disease that can sometimes be difficult to control while older patients tend to have a less severe form.
During the past 10 years attempts …
Footnotes
Leading articles express the views of the author and not those of the editor and editorial board.