IgG4‐related sclerosing cholangitis with autoimmune pancreatitis and periaortitis: Case report and review of the literature

Immunoglobulin G4 (IgG4)-related systematic disease (IgG4-RSD) is a systemic fibroinflammatory condition characterized by extensive IgG4-positive plasma cells and T-lymphocyte infiltration of various organs including pancreas, bile duct, gallbladder, salivary glands, kidney, lung, prostate, retroperitoneum and even the thyroid gland. It may affect one or two and sometimes more than three organs. Early diagnosis and treatment could not prevent sclerosis and permanent damage of the affected organ but also avoid other organ involvement. Here we present a rare case of IgG4-RSD affecting pancreas, biliary duct system and abdominal aorta. A review of the literature was also made mainly concerning IgG4-related sclerosing cholangitis (IgG4-SC) and periaortitis.