ABSTRACT
Immunoglobulin G4 related disease (IgG4-RD) is a rare multisystem
inflammatory disease, in which one of the main gastrointestinal
manifestations is autoimmune pancreatitis (AIP) type 1. AIP type 1 is
correlated with a clinical presentation of obstructive jaundice,
increased serum levels of IgG4 and suggestive pancreatic findings on
radiologic imaging. Histo-pathology (HP) and immune-histochemistry (IHC)
examinations obtained by biopsy are the gold standard for establishing
the diagnosis.
Several theories tried to elucidate the correlation between IgG4-RD and
pancreatic or other extra-pancreatic malignancies. There are
controversial opinions whether AIP type 1 serves as a premalignant
state, or whether these two conditions are coexistent.
We present a patient who had clinical, laboratory, radiologic and
histologic findings that were consistent with a pancreatic space
occupying lesion (SOL). As a result, he underwent a Whipple operation,
but the IHC findings were compatible with AIP type 1. After 1 year of
remission, the patient presented with a new pancreatic SOL that was
diagnosed as adenocarcinoma.
Key words: autoimmune pancreatitis (AIP) type 1, pancreatic
malignancy, Immunoglobulin G4 related disease (IgG4-RD)