The International Autoimmune Hepatitis Group
Autoimmune hepatitis (AIH) is a severe life threatening chronic progressive immune-mediated inflammatory disorder of the liver. The aetiology of AIH is largely unknown, but liver damage is believed to be the consequence of an aberrant immune response to an as yet unknown antigen in a genetically predisposed host. In all but the mildest form of AIH, fibrosis is frequently present at diagnosis, and with advanced disease bridging fibrosis and cirrhosis are often seen. More than 50% of patients have fibrosis at diagnosis and up to 20% have cirrhosis, which indicates that the disease has remained unrecognized for a significant period of time. Over the past four decades there has been only marginal development of novel therapies in AIH, current treatment schedules dating back to the mid-seventies. Such treatments include the long-term use of predniso(lo)ne, which not always arrests disease progression and has severe side effects. The slow progress both in terms of understanding disease pathogenesis and in the development of novel treatments has resulted in outdated guidelines. This stems from the fact that AIH is a relative uncommon disease, which has received limited interest from pharmaceutical companies and the scientific community. In contrast to primary sclerosing cholangitis (PSC) and primary biliary cirrhosis (PBC), so far there has not been an organised international initiative or registry that organized to address these issues.
The International Autoimmune Hepatitis Group (IAIHG) now aims to establish a web-based data registry for international, prospective, registration of AIH patients and follow-up data, according to the standardised directives as well as to develop resources and centres of excellence for the management of AIH accessible to clinicians, researchers, and patients.