Auto-immune (lupoid) hepatitis: an entity in the spectrum of chronic active liver disease.
The concept of auto-immune hepatitis as a disease entity evolved from the descriptions of 'chronic active hepatitis' (CAH) in the 1950s. Several types of CAH are distinguished by disease-specific features. The distinctive (but not exclusive) markers for auto-immune CAH include: a negative test for HBsAg; female; Northern European ethnic background; multisystem disease expression; histological CAH with large areas of periportal piecemeal necrosis and plasmacytosis; pronounced hypergammaglobulinaemia; serum auto-antibodies the HLA B8-DR3 phenotype; responsiveness to corticosteroid therapy; and rarity of supervening hepatocellular carcinoma. Much weight is attached to the serological marker auto-antibodies to nuclear or smooth muscle (actin) antigens (ANA, SMA). However, these auto-antibodies do not have an absolute association with auto-immune CAH: the serological reactions are not yet standardized; titres decrease with remission of disease; and other auto-antibodies mark variant forms of auto-immune hepatitis. A more confident acceptance of auto-immune hepatitis as an entity requires detection of a liver-specific antigen, a valid experimental disease model in animals, and a better understanding of immune-mediated damage to liver cells.
Mackay
I R
IR
Centre for Molecular Biology and Medicine, Monash University, Clayton, Victoria, Australia.
D001323:Autoantibodies / Q000276:immunology*; D001327:Autoimmune Diseases; D015415:Biomarkers; D005260:Female; D006521:Hepatitis, Chronic / Q000276:immunology*; D006801:Humans; D008180:Lupus Erythematosus, Systemic; D008297:Male; D009626:Terminology as Topic
D001323:Autoantibodies; D015415:Biomarkers