Diagnostic criteria for autoimmune chronic pancreatitis revisited

Table 1 Diagnostic criteria for autoimmune pancreatitis by the Japan Pancreas Society

Diagnostic criteria
I	Imaging criterion: Diffuse narrowing of the main pancreatic duct with irregular wall (more than 1/3 length of the entire pancreas) and enlargement of the pancreas
II	Laboratory criterion: Abnormally elevated levels of serum gamma-globulin and/or IgG, or the presence of autoantibodies
III	Histopathologic criterion: Marked lymphoplasmacytic infiltration and dense fibrosis
For diagnosis, criterion I must be present, together with criterion II and/or III

Table 2 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 Iand any of criterion II-IV

Table 3 Clinical characteristics of 28 patients with autoimmune chronic pancreatitis

Age/sex	Other autoimmunediseases	IgG (mg/dL)	IgG4 (mg/dL)	Auto antibody	ERCP	CT findings	Pancreaspathology	Response to steroid
54/M	+	3 570	1 764	-	S	DE	N-C	Y
60/M	+	4 500	810	+	S	DE	N-C	Y
32/M	-	97	13	-	S	DE	ND	Y
68/M	-	2 010	350	-	S	DE	Diagnosticb	Y
52/M	+	1 440	32	+	S	DE	ND	Y
59/M	-	1 880	324	-	S	DE	Diagnostic	Y
53/M	+	1 990	150	+	S	DE	Diagnostic	Y
56/F	-	2 730	1 464	+	Sa	DE	N-C	Y
74/M	-	2 210	N-C	-	S	DE	N-C	Y
52/M	-	2 100	N-C	-	D	DE	N-C	Y
53/F	-	1 240	29	-	Sa	DE	Diagnostic	Y
45/M	-	1 470	N-C	+	D	DE	Diagnostic	Y
58/M	-	4 100	780	+	S	DE	Diagnostic	Y
63/F	-	1 200	10	-	D	DE	Diagnostic	Y
61/M	-	1 500	190	-	Sa	DE	Diagnostic	Y
63/M	-	1 780	658	+	Sa	DE	Diagnostic	Y
49/F	-	2 000	445	+	D	DE	N-C	Y
59/M	-	1 230	840	+	D	DE	Diagnostic	Y
51/M	-	1 570	310	-	D	DE	Diagnostic	Y
68/M	+	3 550	1 360	+	S	DE	N-C	Y
33/M	-	1 350	66	-	Sa	DE	N-C	Y
36/M	-	1 060	50	+	D	DE	ND	Y
60/M	-	1 930	N-C	+	Sa	DE	Diagnostic	Y
64/F	-	1 470	N-C	+	S	DE	NDc	Y
70/M	+	1 820	310	+	D	DE	Diagnostic	Y
44/M	-	1 110	36	-	S	DE	Diagnostic	Y
34/F	+	1 070	11	-	S	DE	ND	Y
78/M	-	4 570	1 580	+	Sa	DE	N-C	Y

S: Segmental irregular narrowing of main pancreatic duct; D: Diffuse irregular narrowing of main pancreatic duct; DE: Diffuse enlargement of pancreas; N-C: Not checked; ND: Non-diagnostic;

^a: Extent of irregular narrowing was less than 1/3 of the entire length of main pancreatic duct;

^b: Lymphoplasma cell infiltration and fibrosis;

^c: Eosinophilic infiltration.

Table 4 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

Table 5 A proposal of revised diagnostic criteria for autoimmune pancreatitis

Diagnostic 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 lymphoplasmocytic infiltration
Criterion IV. Association of other postulated autoimmune disease
Definite
I+II+III+IV or I+II +III or I+II or I+III
Probable	Rediagnosed as “definite” if “response to the steroid” is present
I+IV
Possible
I only

Table 6 General features of autoimmune diseases

General features of autoimmune diseases
1 Elevated levels of serum gammaglobulin and/or IgG, or detected autoantibody
2 Lymphoplasma cell infiltration and fibrosis at lesional site
3 Association with other autoimmune diseases
4 Response to the steroid