Autoimmune Pancreatitis



Autoimmune Pancreatitis


Mari Mino-Kenudson, MD









Hematoxylin & eosin section of lymphoplasmacytic sclerosing pancreatitis shows a prominent fibroinflammatory process with a periductal accentuation image.






The fibroinflammatory process is limited to the periductal region, without significant lobular destruction or interlobular fibrosis, in idiopathic duct-centric chronic pancreatitis.


TERMINOLOGY


Abbreviations



  • Autoimmune pancreatitis (AIP)


Synonyms



  • Lymphoplasmacytic sclerosing pancreatitis (LPSP)


  • Idiopathic duct-centric chronic pancreatitis (IDCP)


  • Primary sclerosing pancreatitis


  • Nonalcoholic duct destructive chronic pancreatitis


Definitions



  • Fibroinflammatory disease of presumed autoimmune etiology that affects pancreas



    • Similar fibroinflammatory process often affects other organs such as bile ducts, salivary glands, retroperitoneum, and lymph nodes


    • Associated with many other autoimmune diseases



      • Specific antigenic trigger unknown


CLINICAL ISSUES


Presentation



  • Jaundice


  • Weight loss


  • Vague abdominal pain


Laboratory Tests



  • Elevated serum IgG4


  • Elevated pancreatic enzymes


  • ANA often positive


Treatment



  • Surgical approaches



    • Surgically resected when differentiation from pancreatic cancer is difficult or impossible


  • Drugs



    • Steroids


Prognosis



  • Steroid therapy is usually very effective



    • Natural regression seen in some cases


    • Recurrence reported in 6-26%

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Jul 7, 2016 | Posted by in PATHOLOGY & LABORATORY MEDICINE | Comments Off on Autoimmune Pancreatitis
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