Autoimmune pancreatitis

Autoimmune pancreatitis 

Updated: 02/13/2021

© Jun Wang, MD, PhD

General features
  • AKA primary sclerosing cholangitis-like pancreatitis, non-alcoholic duct destructive chronic pancreatitis
  • Two subtypes
Lymphoplasmacytic sclerosing autoimmune pancreatitis
Idiopathic duct centric autoimmune pancreatitis
  • Forms mass that may constrict bile duct
  • Clinically and radiological features may mimic malignant tumors
  • May cause exocrine and endocrine deficiencies
  • Risk factor for pancreatic cancer
  • Overall excellent prognosis
Lymphoplasmacytic sclerosing autoimmune pancreatitis
Idiopathic duct centric autoimmune pancreatitis
  • No gender preference, younger patients, associated with inflammatory bowel diseases, not with obliterative phlebitis
  • No known causes for usual chronic pancreatitis, nor features of classic chronic pancreatitis (fat necrosis, pseudocyst, calcification, etc.)
  • Compared to chronic pancreatitis NOS, more foci of periductal inflammation and neutrophilic microabscesses
Key pathological features
  • Lymphoplasmacytic sclerosing autoimmune pancreatitis
Hypercellular lymphoplasmacytic infiltrate of interlobular stroma
Storiform type fibrosis
>10 IgG4+ plasma cells/high-power field
  • Idiopathic duct centric autoimmune pancreatitis
Confined to pancreas (i.e. not systemic)
Microabscesses and ductal ulceration, granulocyte epithelial lesions
Occasional IgG4 plasma cells (<10/HPF)
 Treatment
  • Steroid



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Contents

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