Autoimmune liver and pancreatic disease

UEG 2013

A strong relationship between liver and pancreatic diseases has been reported, both inflammatory (i.e. biliary lithiasis and acute pancreatitis, pancreatic involvement in primary sclerosing cholangitis and primary biliary cirrhosis) and neoplastic (i.e. common bile duct stenosis in pancreatic cancer, pancreatitis in cholangio-carcinoma.

Autoimmunity is one of the mechanism involved in common diseases of the pancreas and biliary tree, biliary involvement in autoimmune pancreatitis (AIP) probably the most known nowadays.

In clinical practice, the autoimmune process may involve both organs, but also only one at least clinically and morphologically. Symptoms at clinical presentation may therefore be pancreatic (pancreatitis, diabetes, steatorrhea with weight loss) and/or biliary (jaundice, cholestasis, abnormal liver function tests).

IgG4-related syndromes

• The term IgG4 related syndromes encompasses a variety of clinical entities once regarded as being entirely separated diseases. The list of organs associated with this condition is growing steadily.

IgG4 related syndromes are characterized by diffuse lymphoplasmacytic infiltrates, presence or abundant IgG4 positive plasma cells and extensive fibrosis. Tumorous swelling, eosinophilia are frequently observed features.

• IgG4 related syndromes are rare diseases, which occur beyond the fourth decade of life with an overall good prognosis if treated with steroids.

• Awareness for those disease and consideration of the diagnosis can save patients from unnecessary surgery.

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