Autoimmune hepatitis: 50 years of (slow) progress

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The first textbook on autoimmune diseases was published 50 years ago in 1963 by Ian R. Mackay and F. Macfarlane Burnet, about a decade after the concept of immunologic tolerance had been established. It was entitled “Autoimmune Diseases: Pathogenesis, Chemistry and Therapy (1).” Interestingly, it was published as part of a series called “Living Chemistry”. In this seminal work there were 13 chapters, of the approximately 30 known autoimmune diseases, including what was called “Active chronic and lupoid hepatitis”, now known as autoimmune hepatitis (AIH). There are now more than 100 well-defined autoimmune diseases and within that group, the liver, essential to normal immune tolerance, can itself become a victim of an autoimmune attack, in such diseases as primary biliary cirrhosis (PBC), primary sclerosing cholangitis (PSC), AIH, autoimmune sclerosing cholangitis and conditions with overlapping features within this spectrum, referred to as overlap or variant syndromes (2, 3).