Unifying Concepts

Diagnostic Criteria for Autoimmune Pancreatitis

Diagnostic Criteria for Autoimmune Pancreatitis by the Japan Pancreas Society

Findings on Imaging Radiography (One Required)

Serologic and Histologic Findings (One Required)

Cross-sectional Imaging


Serologic Analysis

Pancreatic-Biliary Histologic Analysis

Nongastrointestinal Histologic Analysis

Diffusely enlarged pancreas

Segmental pancreatic ductal narrowing

Elevated serum IgG4 level

Periductal lympho-plasmacytic infiltration or fibrosis

Tubulointerstitial nephritis with immune deposits within tubular basement membranes

Enhanced peripheral rim of hypoattenuation “halo”

Focal pancreatic ductal narrowing

Elevated serum IgG or gamma globulin level

Obliterative phlebitis

Pulmonary interstitial lymphoplasmacytic infiltration with IgG4-positive plasma cells

Low-attenuation mass in head of pancreas

Diffuse pancreatic ductal narrowing

Presence of ALA, ACA II, ASMA, or ANA

IgG4-positive plasma cells in tissue

Chronic sialadenitis with IgG4-positive plasma cells

Criteria were modified from those of the Japan Pancreas Society. ERCP denotes endoscopic retrograde cholangiopancreatography, MRCP magnetic resonance cholangiopancreatography, ALA antilactoferrin antibody, ACA II anti-carbonic anhydrase II antibody, ASMA anti-smooth-muscle antibody, and ANA antinuclear body.

The presence of tissue IgG4-positive cells is not necessarily abnormal, but an increased number of infiltrating IgG4-positive plasma cells is abnormal.

Diagnostic Criteria for Autoimmune Pancreatitis in Asan Medical Center

Inclusion Criteria

Criterion I. Pancreatic imaging (essential)

  1. CT: Diffuse enlargement (swelling) of pancreas, and
  2. ERCP: Diffuse or segmental irregular narrowing of main pancreatic duct

Criterion II. Laboratory findings

  1. elevated levels of IgG and/or IgG4. or
  2. detected autoantibodies

Criterion III. Histopathologic findings: Fibrosis and lymphoplasmacytic infiltration

Criterion IV. Response to the steroid

Definite diagnosis: Criterion I and any of criterion II-IV

Diagnostic Criteria for Autoimmune Pancreatitis by Italian Group

Diagnostic criteria

  1. Histology and cytology
  2. The association with other postulated autoimmune disease
  3. Response to the steroid therapy



  1. Japan Pancreas Society. Diagnostic criteria for autoimmune pancreatitis. J Jpn Pancreas Soc 2002;17:585-7.
  2. Finkelberg DL, Sahani D, Deshpande V, Brugge WR. Autoimmune pancreatitis. N Engl J Med. 2006 Dec 21;355(25):2670-6. [Medline]
  3. Kim KP, Kim MH, Kim JC, Lee SS, Seo DW, Lee SK. Diagnostic criteria for autoimmune chronic pancreatitis revisited. World J Gastroenterol. 2006 Apr 28;12(16):2487-96. [Medline]


Created: Jan 07, 2006

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