November 12, 2014
Notes: Kalb, Bobby
Martin, Diego R
Sarmiento, Juan M
Erickson, Sarah H
Tapper, Elliot B
Adsay, N Volkan
Radiology. 2013 Nov;269(2):475-81. doi: 10.1148/radiol.13112056. Epub 2013 Jul 11.
Author Address: Department of Radiology, University of Arizona College of Medicine, 1501 N Campbell Ave, Tucson, AZ 85724; Departments of Surgery and Pathology, Emory University School of Medicine, Atlanta, Ga; Department of Radiology, University of Virginia School of Medicine, Charlottesville, Va; Rome Radiology Group, Rome, Ga; Department of Medicine, Beth Israel Deaconess Medical Center, Boston, Mass.
Reference Type: Journal Article
Record Number: 5172Author: Kamisawa, T., Chari, S. T., Lerch, M. M., Kim, M. H., Gress, T. M. and Shimosegawa, T.
Title: Recent advances in autoimmune pancreatitis: type 1 and type 2
Short Title: Recent advances in autoimmune pancreatitis: type 1 and type 2
Alternate Journal: Gut
ISSN: 1468-3288 (Electronic)
Accession Number: 23749606
Keywords: *Autoimmune Diseases/classification/diagnosis/drug therapy/immunology
Immunologic Factors/*therapeutic use
Medication Therapy Management
*Pancreatitis, Chronic/classification/diagnosis/drug therapy/immunology
Recurrence/prevention & control
Abstract: Autoimmune pancreatitis (AIP) is a form of chronic pancreatitis characterised clinically by frequent presentation with obstructive jaundice, histologically by a lymphoplasmacytic infiltrate with fibrosis, and therapeutically by a dramatic response to steroids. When so defined, AIP can be sub-classified into two subtypes, 1 and 2. Recent international consensus diagnostic criteria for AIP have been developed for diagnosis of both forms of AIP. Type 1 AIP is the pancreatic manifestation of a multiorgan disease, recently named IgG4-related disease. Little is known about the pathogenesis of either form of AIP. Despite frequent association of type 1 AIP with elevated serum IgG4 levels and infiltration with IgG4-positive plasma cells, it is unlikely that IgG4 plays a pathogenic role in AIP. Type 1 AIP responds to steroids, but there needs to be consensus on treatment regimens for induction and therapeutic end points. Relapses are common, but can be reduced by long-term use of low-dose steroids. Recent reports suggest that immunomodulators (azathioprine, 6-mercaptopurine and mycophenolate mofetil), as well biological agents (the antibody to CD20, rituximab) may have a role in maintaining remission in relapsing type 1 AIP. Future studies should clarify the best management options for treatment of relapses and maintenance of remission. Type 2 AIP is a pancreas-specific disorder not associated with IgG4. It presents in younger individuals equally with obstructive jaundice and pancreatitis. The inflammatory process responds to steroid therapy; relapses are uncommon. The clinical spectrum and long-term outcomes of medically treated type 2 AIP are still being evaluated.