AND IAN G. MCFARLANE (UNITED KINGDOM), CONVENORS, ON BEHALF OF THE PANEL*
The syndrome of autoimmune CAH (AI-CAH) has been recognized in white subjects for more than 30 yr, and the characteristic features have been extensively reviewed (1-6). It is a disorder of unknown etiology in which progressive destruction of the hepatic parenchyma occurs, often progressing to cirrhosis, and in the more severe cases, it carries a high mortality if untreated (7-9). Earlier descriptions of the disease noted its chronic, but fluctuating, course and the marked hypergammaglobulinemia, circulating autoantibodies, female preponderance and several other features in common with disorders thought to have an autoimmune basis (10-19), including an association with the human leukocyte antigen-BSDR3 haplotype (20) and, more recently, with human leukocyte antigen-DR4 in older patients and in Japanese patients (21-23).
Although these observations, together with responsiveness to immunosuppressive therapy (a characteristic feature of the condition) (9, 24-35), have led to general acceptance that the disease has a basis in aberrant autoreactivity, many previous researchers have encompassed AI-CAH within chronic hepatitis generally rather than considering it as a disease in its own right (36-38).