IgG4-related Disorders of the Gastrointestinal Tract

IgG4-related disease, a newly established multisystemic disease can affect virtually every organ. Histologically, it is characterized by the presence of a dense lymphoplasmacytic infiltrate, storiform-type fibrosis, and obliterative phlebitis. The disease shows elevated serum and tissue IgG4. The pancreas and hepatobiliary tract are involved far more commonly than the tubular gut. This review summarizes the clinical and pathologic features of the gastrointestinal manifestations of IgG4-related disease and discusses the wide spectrum of diseases that this entity may mimic.