AUTOIMMUNE (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. Copyright © 1990, Wiley Blackwell. All rights reserved