Population-based studies of the incidence and prevalence of autoimmune liver diseases in the world is scarce. We conducted a nationwide study of the prevalence of these diseases in our country, Iran. The diseases we included were autoimmune hepatitis, primary sclerosing cholangitis (PSC), primary biliary cholangitis (PBC), variant (overlap) syndrome, autoimmune cholangiopathy and IgG4 related cholangiopathy.

We contacted all gastroenterologists and internists who were registered as members of Iranian association of gastroenterology and asked them to fill our questionnaire. We gathered some minimal data set to design a primary questionnaire including demographic data, national code, birth date, sex, race and final diagnosis including the date and physician who diagnosed the disease. The data were then entered into a data base and descriptive pooled information analyzed.

: : We have registered 4,491 patients with autoimmune liver diseases from all around the country so far. Of these, 1334(29.7%) were male. Mean age of the patients was 43.5 years (41.0 years among males and 44 years among females, P=NS). There were 2,525 (56.2%) autoimmune hepatitis cases (1938, 61.6% female), 836 (18.6%) PSC cases (493, 37% male), 557 (12.4%) PBC cases (14.7% female), 540 (12%) variant (overlap) syndrome (12.4% female), and 18 (0.4%) and 3 (0.1%) with as autoimmune cholangiopathy and IgG-4 related cholangiography respectively.

Autoimmune liver diseases are important causes of chronic liver disease in Iran. This is the first nation-wide report of autoimmune liver disease registry in Iran showing a picture of distribution and composition of various autoimmune liver diseases in the country. Completion of this registry will help to depict a better picture of the burden of disease in Iran as a and a base for regional and international comparisons as well as further research in the field.