TY - JOUR
T1 - Current challenges in the diagnosis of autoimmune pancreatitis
AU - Gupta, Rajib
AU - Deshpande, Vikram
N1 - Publisher Copyright:
© 2016
PY - 2016/6/1
Y1 - 2016/6/1
N2 - Autoimmune pancreatitis (AIP), an inflammatory disease of the pancreas first described in Japan, is characterized by a tumefactive lesion in the pancreas, thus clinically resembling pancreatic cancer. Currently, it is classified into two variants – type 1 AIP and type 2 AIP, each with distinct clinico-pathologic and epidemiologic features. Type 1 AIP is a part of IgG4 related disease, a generalized multi-systemic disease, and recapitulates its clinical and pathologic features. Type 2 AIP is an isolated pancreas-centric disease, and is not associated with elevated tissue or serum IgG4. Histologically, type 1 AIP is characterized by storiform-type fibrosis and obliterative phlebitis, while type 2 disease shows granulocytic epithelial lesions. Even when AIP is suspected, a diagnosis should seldom be made in isolation, but should be rendered only after collating clinical and radiologic data. Steroids remain the mainstay of treatment for both variants of AIP, although other immunosuppressive drugs like rituximab have proven successful. In this review, we summarize the current knowledge of this entity and discuss diagnostic challenges, with particular emphasis on the interpretation of needle biopsies. Recognition of AIP as a mimic of pancreatic cancer is imperative to reduce unnecessary morbidity and mortality associated with pancreatic surgery.
AB - Autoimmune pancreatitis (AIP), an inflammatory disease of the pancreas first described in Japan, is characterized by a tumefactive lesion in the pancreas, thus clinically resembling pancreatic cancer. Currently, it is classified into two variants – type 1 AIP and type 2 AIP, each with distinct clinico-pathologic and epidemiologic features. Type 1 AIP is a part of IgG4 related disease, a generalized multi-systemic disease, and recapitulates its clinical and pathologic features. Type 2 AIP is an isolated pancreas-centric disease, and is not associated with elevated tissue or serum IgG4. Histologically, type 1 AIP is characterized by storiform-type fibrosis and obliterative phlebitis, while type 2 disease shows granulocytic epithelial lesions. Even when AIP is suspected, a diagnosis should seldom be made in isolation, but should be rendered only after collating clinical and radiologic data. Steroids remain the mainstay of treatment for both variants of AIP, although other immunosuppressive drugs like rituximab have proven successful. In this review, we summarize the current knowledge of this entity and discuss diagnostic challenges, with particular emphasis on the interpretation of needle biopsies. Recognition of AIP as a mimic of pancreatic cancer is imperative to reduce unnecessary morbidity and mortality associated with pancreatic surgery.
KW - IgG4
KW - IgG4 related disease
KW - autoimmune pancreatitis
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U2 - 10.1016/j.mpdhp.2016.05.001
DO - 10.1016/j.mpdhp.2016.05.001
M3 - Review article
AN - SCOPUS:84996614753
SN - 1756-2317
VL - 22
SP - 211
EP - 218
JO - Diagnostic Histopathology
JF - Diagnostic Histopathology
IS - 6
ER -