Type:
Educational Exhibit
Keywords:
Abdomen, Pancreas, CT, MR, Ultrasound, Diagnostic procedure, Inflammation
Authors:
R. P. Patel1, T. M. Chandler1, S. Barrett2, J. Coyle3, C. Zwirewich1, A. Harris4; 1Vancouver, BC/CA, 2Dublin /IE, 3Cork/IE, 4BC/CA
DOI:
10.1594/ecr2013/C-2649
Background
Autoimmune pancreatitis (AIP) is the pancreatic manifestation of IgG4 related sclerosing disease which was recently recognized as distinct disease entity. Known as a benign disease process,
it can clinically mimic pancreatic cancer.
Early diagnosis and management is important as it can slowly progress to end stage pancreatic disease.
Numerous extrapancreatic organs may be involved either synchronously or metachronously.
Most cases of AIP are associated with elevated IgG4 levels; extensive IgG4 positive plasma cells; and infiltration of lymphocytes into various organs which lead to fibrosis.
It is important to recognize multiorgan involvement of IgG4 related sclerosing disease and be familiar with its clinical and imaging features because it demonstrates a favourable response to corticosteroid treatment.