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World J Gastroenterol. Nov 28, 2014; 20(44): 16559-16569
Published online Nov 28, 2014. doi: 10.3748/wjg.v20.i44.16559
Published online Nov 28, 2014. doi: 10.3748/wjg.v20.i44.16559
Table 1 Clinical diagnostic criteria for autoimmune pancreatitis in 2011 by Japan Pancreas Society (JPS-2011)[16]
A: Diagnostic items |
I: Enlargement of the pancreas: |
(a) Diffuse enlargement |
(b) Segmental/focal enlargement |
II: ERP (endoscopic retrograde pancreatography) shows irregular narrowing of the main pancreatic duct |
III: Serological findings |
Elevated level of serum IgG4 (≥ 135 mg/dL) |
IV: Pathological findings: Among (1)-(4) listed below |
(a) Three or more are observed |
(b) Two are observed |
(1) Prominent infiltration of lymphocytes and plasmacytes and fibrosis |
(2) More than 10 IgG4-positive plasmacytes per high-power microscope field |
(3) Storiform fibrosis |
(4) Obliterative phlebitis |
V: Extra-pancreatic lesions: sclerosing cholangitis, sclerosing dacryoadenitis/sialoadenitis/retroperitoneal fibrosis |
(a) Clinical lesions |
Extrapancreatic sclerosing cholangitis, sclerosing dacryoadenitis/ sialoadenitis (Mikulicz disease) or/retroperitoneal fibrosis |
(b) Pathological lesions |
Pathological examination shows characteristic features of sclerosing cholangitis, sclerosing dacryoadenitis sialoadenitis or/retroperitoneal fibrosis |
<Option> Effectiveness of steroid therapy |
A specialized facility may include in its diagnosis the effectiveness of steroid therapy, once pancreatic or bile duct cancers have been ruled out. When it is difficult to differentiate from malignant conditions, it is desirable to perform cytological examination using an endoscopic ultrasound-guided fine needle aspiration (EUS-FNA). Facile therapeutic diagnosis by steroids should be avoided unless the possibility of malignant tumor has been ruled out by pathological diagnosis. |
B: Diagnosis |
I: Definite diagnosis |
(1) Diffuse type |
Ia + III/IVb/V (a/b) |
(2) Segmental/focal type |
Ib + II + two or more of < III/IVb/V (a/b) > |
or |
Ib + II + < III/IVb/V (a/b) > + Option |
(3) Definite diagnosis by histopathological study |
IVa |
II: Probable diagnosis |
Segmental/focal type: Ib + II + < III/IVb/V(a/b) > |
III: Possible diagnosis1 |
Diffuse type: Ia + II + Option |
Segmental/focal type: Ib + II + Option |
Table 2 Diagnosis of definitive and probable type 1 autoimmune pancreatitis using international consensus diagnostic criteria[25]
Diagnosis | Primary basis for diagnosis | Imaging evidence | Collateral evidence |
Definitive type 1 AIP | Histology | Typical/indeterminate | Histologically confirmed LPSP (level 1 H) |
Imaging | Typical | Any non-D level 1/level 2 | |
Indeterminate | Two or more from level 1 (+ level 2 D1) | ||
Response to steroid | Indeterminate | Level 1 S/OOI + Rt or level 1 D + | |
Level 2 S/OOI/H + Rt | |||
Probable type 1 AIP | Indeterminate | Level 2 S/OOI/H + Rt | |
Criterion | Level 1 | Level 2 | |
P: Parenchymal imaging | Typical: | Indeterminate (including atypical3): | |
Diffuse enlargement with delayed enhancement (sometimes associated with rim-like enhancement) | Segmental/focal enlargement with delayed enhancement | ||
D: Ductal imaging (ERP) | Long (> 1/3 length of the main pancreatic duct) or multiple strictures without marked upstream dilatation | Segmental/focal narrowing without marked upstream dilatation (duct size, < 5 mm) | |
S: Serology | IgG4, > 2 × upper limit of normal value | IgG4, 1-2 × upper limit of normal value | |
OOI: Other organ involvement | a or b | a or b | |
a: Histology of extrapancreatic organs | a: Histology of extrapancreatic organs including endoscopic biopsies of bile duct4: | ||
Any three of the following: | Both of the following: | ||
(1) Marked lymphoplasmacytic infiltration with fibrosis and without granulocytic infiltration | (1) Marked lymphoplasmacytic infiltration without granulocytic infiltration | ||
(2) Storiform fibrosis | (2) Abundant (> 10 cells/HPF) IgG4-positive cells | ||
(3) Obliterative phlebitis | |||
(4) Abundant (> 10 cells/HPF) IgG4-positive cells | |||
b: Typical radiological evidence | b: Physical or radiological evidence | ||
At least one of the following: | At least one of the following | ||
(1) Segmental/multiple proximal (hilar/intrahepatic) or proximal and distal bile duct stricture | (1) Symmetrically enlarged salivary/lachrymal glands | ||
(2) Retroperitoneal fibrosis | (2) Radiological evidence of renal involvement described in association with AIP | ||
H: Histology of the pancreas | LPSP (core biopsy/resection) | LPSP (core biopsy) | |
At least 3 of the following: | Any 2 of the following: | ||
(1) Periductal lymphoplasmacytic infiltrate without granulocytic infiltration | (1) Periductal lymphoplasmacytic infiltrate without granulocytic infiltration | ||
(2) Obliterative phlebitis | (2) Obliterative phlebitis | ||
(3) Storiform fibrosis | (3) Storiform fibrosis | ||
(4) Abundant (> 10 cells/HPF) IgG4-positive cells | (4) Abundant (> 10 cells/HPF) IgG4-positive cells | ||
Response to steroid (Rt)2 | Diagnostic steroid trial | ||
Rapid ( ≤ 2 wk) radiologically demonstrable resolution or marked improvement in pancreatic/extrapancreatic manifestations |
Table 3 Diagnosis of definitive and probable type 2 autoimmune pancreatitis using international consensus diagnostic criteria[25]
Diagnosis | Imaging evidence | Collateral evidence |
Definitive type 2 AIP | Typical/indeterminate | Histologically confirmed IDCP (level 1 H) or clinical inflammatory bowel disease + level 2 H + Rt |
Probable type 2 AIP | Typical/indeterminate | Level 2 H/clinical inflammatory bowel disease + Rt |
Criterion | Level 1 | Level 2 |
P: Parenchymal imaging | Typical: | Indeterminate (including atypical2): |
Diffuse enlargement with delayed enhancement (sometimes associated with rim-like enhancement) | Segmental/focal enlargement with delayed enhancement | |
D: Ductal imaging (ERP) | Long (> 1/3 length of the main pancreatic duct) or multiple strictures without marked upstream dilatation | Segmental/focal narrowing without marked upstream dilatation (duct size, < 5 mm) |
OOI: Other organ involvement | Clinically diagnosed inflammatory bowel disease | |
H: Histology of the pancreas (core biopsy/resection) | IDCP | |
Both of the following: | Both of the following: | |
(1) Granulocytic infiltration of duct wall (GEL) with or without granulocytic acinar inflammation | (1) Granulocytic and lymphoplasmacytic acinar infiltrate | |
(2) Absent or scant (0-10 cells/HPF) IgG4-positive cells | (2) Absent or scant (0-10 cells/HPF) IgG4-positive cells | |
Response to steroid (Rt)1 | Diagnostic steroid trial | |
Rapid ( ≤ 2 wk) radiologically demonstrable resolution or marked improvement in manifestations |
Table 4 Diagnosis of autoimmune pancreatitis-not otherwise specified using international consensus diagnostic criteria[25]
Diagnosis | Collateral evidence (case with only D1/2) |
AIP-not otherwise specified | D1/2 + Rt |
Table 5 Characteristics of clinicopathological findings in type 1 and type 2 autoimmune pancreatitis
Type 1 AIP | Type 2 AIP | |
Geographical distributuion | Asia > United States, Europe | Europe > United States > Asia |
Age at presentation | 60-70 s | 40-50 s |
Gender | Male >> Female | Male = Female |
Symptoms | Jaundice, Abdominal pain | Jaundice, Abdominal pain |
Serology | IgG4, IgG, Autoantibodies | Usually negative |
Pancreatic images | Enlarged (focal, diffuse) | Enlarged (focal, diffuse) |
Pancreatic histology | LPSP | IDCP with GEL |
Extrapancreatic lesions | Sclerosing cholangitis, sialoadenitis, retroperitoneal fibrosis, interstitional nephritis, etc. | Inflammatory bowel disease |
Steroid response | Excellent | Excellent |
Relapse | High rate | Rare |
Table 6 Extrapancreatic lesions associated with type 1 autoimmune pancreatitis
Close association | Possible association |
Lachrymal gland inflammation | Hypophysitis |
Sialoadenitis | Autoimmune neurosensory hearing loss |
Hilar lymphadenopathy | Uveitis |
Interstitial pneumonitis | Chronic thyroiditis |
Sclerosing cholangitis | Pseudotumor (breast, lung, liver) |
Retroperitoneal fibrosis | Gastric ulcer |
Tubulointestinal nephritis | Swelling of Papilla of Vater |
IgG4 hepatopathy | |
Periaortitis | |
Prostatitis | |
Schonlein-Henoch purpura | |
Autoimmune thrombocytopenia |
- Citation: Matsubayashi H, Kakushima N, Takizawa K, Tanaka M, Imai K, Hotta K, Ono H. Diagnosis of autoimmune pancreatitis. World J Gastroenterol 2014; 20(44): 16559-16569
- URL: https://www.wjgnet.com/1007-9327/full/v20/i44/16559.htm
- DOI: https://dx.doi.org/10.3748/wjg.v20.i44.16559