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2016 ; 5
(3
): 318-26
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English Wikipedia
Autoimmune pancreatitis
#MMPMID27294040
Omiyale AO
Gland Surg
2016[Jun]; 5
(3
): 318-26
PMID27294040
show ga
Autoimmune pancreatitis (AIP) is a rare, distinct and increasingly recognized
form of pancreatitis which has autoimmune features. The international consensus
diagnostic criteria (ICDC) for AIP recently described two subtypes; type
1[lymphoplasmacytic sclerosing pancreatitis (LPSP)] and type 2 [idiopathic
duct-centric pancreatitis (IDCP) or AIP with granulocytic epithelial lesion
(GEL)]. Type 1 is the more common form of the disease worldwide and current
understanding suggests that it is a pancreatic manifestation of immunoglobulin
G4-related disease (IgG4-RD). In contrast, type 2 AIP is a pancreas-specific
disease not associated with IgG4 and mostly without the overt extra-pancreatic
organ involvement seen in type 1. The pathogenesis of AIP is not completely
understood and its clinical presentation is non-specific. It shares overlapping
features with more sinister pathologies such as cancer of the pancreas, which
continues to pose a diagnostic challenge for clinicians. The diagnostic criteria
requires a variable combination of histopathological, imaging and serological
features in the presence of typical extrapancreatic lesions and a predictable
response to steroids.