Sausage-like enlargement of pancreas (with smooth contour) and loss of normal pancreatic lobulations
Hypoattenuating halo or capsule around pancreas
Absence of retroperitoneal fluid, fluid collections/pseudocysts, or inflammation
Less enhancement than expected in arterial phase; parenchyma/capsule may show delayed enhancement
Diffuse or segmental narrowing of pancreatic duct
MRCP: Multiple discontiguous MPD/bile duct strictures which resolve after secretin (duct penetrating sign)
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Focal form
Focal mass or localized enlargement of pancreas (usually head/uncinate) with delayed enhancement
Lack of biliary or pancreatic ductal dilatation
TOP DIFFERENTIAL DIAGNOSES
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Pancreatic ductal adenocarcinoma
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Acute edematous pancreatitis
PATHOLOGY
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Two distinct histologic subtypes
Type I: Lymphoplasmacytic sclerosing pancreatitis
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Positive IgG4 tissue staining; serum IgG4 elevated
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Extrapancreatic organ involvement common (∼ 60%); inflammatory bowel disease in only 2-6%
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Older patients (usually > age 60) with M > F
Type II: Idiopathic duct-centric pancreatitis
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No IgG4 tissue staining; serum IgG4 not elevated
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No extrapancreatic organ involvement; inflammatory bowel disease in 30%
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Younger patients (mean age 43) with M=F
TERMINOLOGY
Abbreviations
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Autoimmune pancreatitis (AIP)
Synonyms
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Lymphoplasmacytic sclerosing pancreatitis; primary sclerosing pancreatitis; tumefactive pancreatitis; non-alcoholic destructive pancreatitis
Definitions
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Immune-mediated fibroinflammatory disease primarily involving pancreas responding to steroid therapy
IMAGING
General Features
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Best diagnostic clue
Diffusely/focally enlarged pancreas with hypodense halo
No vascular involvement, calcifications, or pseudocysts
Lack of significant dilatation of main pancreatic duct
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Location
May be diffuse, multifocal, or focal/mass-forming
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Morphology
Sausage-shaped appearance of pancreas
Imaging Recommendations
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Best imaging tool
MRCP and gadolinium-enhanced MR
CT Findings
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Diffuse form
Diffuse sausage-like enlargement of pancreas (with smooth contour) and loss of pancreatic lobulations
Hypoattenuating halo or capsule around pancreas
Often less enhancement than expected in arterial phase; delayed enhancement of involved parenchyma/capsule
No retroperitoneal fluid collections or inflammation
Lymphadenopathy common (20%) with similar halo or capsule
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Focal form
Focal mass or localized enlargement of pancreas (usually head/uncinate) with delayed enhancement
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Diffuse or segmental narrowing of pancreatic duct
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Extrapancreatic imaging findings
IgG4 cholangitis in 90%: May be indistinguishable from primary sclerosing cholangitis
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Stricture of common bile duct (CBD) ± intrahepatic ducts with hyperenhancement of duct wall
Renal involvement in 35% of patients with AIP
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Round or wedge-shaped low attenuation parenchymal lesions
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Diffuse renal enlargement
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Perirenal soft tissue rind (mimicking lymphoma)
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Urothelial thickening in renal pelvis
Retroperitoneal fibrosis, IgG4-related lung disease, and enlarged salivary glands or salivary gland mass
IgG4-related lung disease
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Solid nodules, ground glass opacities, interstitial opacities
MR Findings
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Diffuse enlargement of pancreas (T1WI hypointense and T2WI hyperintense)
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Capsule of peripheral hypoenhancement and low T2WI signal with delayed enhancement
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Delayed enhancement of involved parenchyma
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MR cholangiopancreatography (MRCP): Multiple discontiguous main pancreatic duct/bile duct strictures
Strictures resolve after secretin (duct-penetrating sign)
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DWI: Mildly restricted diffusion of affected tissue
Cannot use DWI to differentiate focal AIP from pancreatic cancer
Ultrasonographic Findings
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Transcutaneous ultrasound is of limited value; may have normal appearance
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Endoscopic ultrasound
Enlarged hypoechoic gland with sausage-like appearance, narrowed MPD, and thickening of CBD wall
Echogenic interlobular septa
Nuclear Medicine Findings