CAH, particularly its nomenclature and classification, has long been regarded as "muddled" (1) and "one of the most confused and confusing areas of medicine" (2). Reasons include a paucity of knowledge on etiology and pathogenesis, difficulties in defining the disease and changing clinical appearance since the archetypal syndrome was described some 40 years ago. After 1970, CAH was seen to comprise various subgroups of the disease, although the early descriptions during the 1950s and 1960s were mostly of the autoimmune subgroup. Recently interest in CAH due to chronic infection with hepatitis viruses B and C (HBV, HCV) has overshadowed the autoimmune type by reason of the increasing proportion of cases of infectious CAH and possibilities for its treatment and advances in knowledge in molecular virology. However, attention to autoimmune CAH should not diminish; 20% of 500 cases in a US. referral practice were considered to be of autoimmune type, and new cases continue to appear regularly (3); there is established benefit from long-term treatment with prednisolone (1); and an understanding of autoimmune CAH could provide key insights into autoimmunity in general.
This issue of HEPATOLOGY contains the Brighton Report, based on the deliberations of an international autoimmune hepatitis group of 27 experts from the United States, Europe, Japan and Australia who met in Brighton, UK, in 1992 under the aegis of the International Association for the Study of the Liver. Their aim was to search for a consensus on criteria for diagnosis and response to treatment. Groups who succeed in deriving acceptable criteria for diagnosis of disease render a signal service (4) of benefit to nosologists, epidemiologists, therapists and others, including clinical authors whose reports then only need state that their cases fulfilled particular nominated criteria. Successful examples include the initial and revised criteria of the American Rheumatism Association for the diagnosis and classification of rheumatoid arthritis (5, 6) and systemic lupus erythematosus (7); on the other hand, attempts at criteria for Sjogren's syndrome have not gained general agreement (8).
Diagnostic criteria for various liver diseases were first developed in the Fogarty Report of 1976 (91, which considered CAH but did not make a clear distinction