Update on Epidemiology of IgG4-related Disease Involving the Liver and Pancreas

IgG4-related disease (IgG4-RD) is a rare condition characterized by an immune-mediated fibro-inflammation affecting various organs (liver, pancreas, heart, kidney, brain among others) with peculiar histopathologic feature. Few epidemiological data have been published so far, although dramatic increase in the number of patients diagnosed IgG4-RD has recorded last years. The clinical manifestations involve frequently liver and pancreas. Specifically, crucial challenge differential diagnosis sclerosing cholangitis which accompanied pancreatic involvement. Inflammatory alterations parenchyma also described, new nosology IgG4-autoimmune hepatitis. Type 1 autoimmune pancreatitis manifestation IgG4-RD. first-ever study to estimate point prevalence recently conducted Japan. Moreover, several demographic studies on involving pancreas other countries, majority them are cohort incidence/prevalence lacking. This review aims update recent knowledge focusing risk malignancy.