Auto‐immune hepatitis: Lifting the seventh veil

Abstract

Knowledge of auto‐immune chronic active hepatitis (AI‐CAH) has developed since the 1940s. Using the analogy of the seven veils, the lifting of the first six could be represented by the finding of: hypergammaglobulinaemia; recognition of the clinical syndrome; the hepatic histopathology; the auto‐immune serological markers; the response to corticosteroid/immuno‐suppressive drugs; and the immunogenetic association with HLA B8; DR3. The lifting of the seventh veil, the ultimate understanding of AI‐CAH, will require the following: consensus on criteria for specifying auto‐immune hepatitis as an entity; identification of a liver‐specific auto‐antigen (or hepatocellular neo‐antigen) as target of the immune attack; the nature of the immune effector process which causes hepatocellular destruction; the basis of the deranged immunoregulatory processes expressed as decreased immune suppressor activity; and processes by which ‘anti‐immune’ drugs exert their ameliorative effects. Auto‐immune hepatitis is not unique in the paucity of data on these questions.

How reversible is hepatic functional impairment in autoimmune hepatitis?

“…Patients were recruited into this study between January 1986 and August 1994. All cases were required to satisfy previous criteria for the diagnosis of autoimmune hepatitis, 10 and subsequent review has confirmed that all cases also conform to a ‘definite’ or ‘probable’ diagnosis of autoimmune hepatitis according to the criteria of the International Autoimmune Hepatitis Group 11 . The study conformed to current international guidelines for human experimentation, was approved by the Human Ethics Committee of the Western Sydney Area Health Service, and all subjects gave written informed consent.…”

Cirrhosis of undefined pathogenesis: Absence of evidence for unknown viruses or autoimmune processes

“…Diagnosis of autoimmune liver disease is often difficult and should be based on clinical findings and the presence of autoantibodies in the serum (20). To determine whether our patients with cryptogenic cirrhosis had a previously unrecognized form of autoimmune liver disease, we (a) reevaluated clinical findings typically associated with autoimmune disease by forming an autoimmune score from five clinical criteria typically found in autoimmune liver disease and (b) tested sera of patients with cryptogenic cirrhosis for the presence of various autoantibodies.…”