[Hepatopancreaticobiliary diseases in IgG4-associated autoimmune diseases]

Abstract

Background: IgG4-related diseases are rare systemic multiorgan diseases and can thus affect any organ system. The incidence of diagnosis has significantly increased due to increasing awareness.

Objective: In the abdomen the hepatopancreaticobiliary system provides an essential organ system for the expression of IgG4-associated autoimmune diseases. The focus here is autoimmune pancreatitis type 1 but IgG4-associated sclerosing cholangitis and IgG4-associated hepatopathy, which can also occur in combination are less well-known.

Methods: Various mostly Asiatic, histologically-based diagnostic systems, such as HISORt or international consensus diagnostic criteria (ICDC) are available for the diagnostics of hepatopancreaticobiliary IgG4-related autoimmune diseases, in which imaging techniques playing an increasingly important role.

Results: In addition to generalized organ swelling further morphological and also functional imaging criteria have become increasingly well-known for autoimmune pancreatitis, such as late enhancement or the imaging response to steroid therapy. Magnetic resonance cholangiopancreatography (MRCP) and endoscopic retrograde cholangiopancreatography (ERP) can provide valuable information for the diagnostics of IgG4-related diseases in the hepatopancreaticobiliary system.

Conclusion: IgG4-related autoimmune diseases of the hepatopancreaticobiliary system are a rare group of diseases in which increasing knowledge of the radiological appearance also leads to an increasingly frequency of diagnosis. IgG4-related diseases must be distinguished from non-necrotizing pancreatitis and pancreatic cancer, which is often difficult but has significant therapeutic consequences for the patients.

Keywords: Autoimmune pancreatitis; Cholangitis; Hepatopathy; Lymphoplasmacytic sclerosing pancreatitis; Pancreatitis.