This document summarizes the relationship between autoimmune diseases of the liver and pancreas. It discusses how the autoimmune process can involve both organs clinically and morphologically, causing pancreatic and/or biliary symptoms. It also introduces IgG4-related syndromes, which are characterized by lymphoplasmacytic infiltrates, IgG4-positive plasma cells, and fibrosis. IgG4-related syndromes encompass conditions once considered separate diseases and can affect multiple organs. They typically occur later in life and have a good prognosis with steroid treatment.

1. Autoimmune liver and
pancreatic disease
UEG 2013

2. 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.

3. 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.

4. 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).

5. IgG4-related syndromes

6. • 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.

8. 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.

9. • 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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