Acute recurrent pancreatitis: An autoimmune disease?

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

Criteria	Definition
I Imaging criterion	Diffuse narrowing of the main pancreatic duct with an irregular wall (more than 1/3 length of the entire pancreas) and enlargement of the pancreas
II Laboratory criterion	Abnormally elevated levels of serum gammaglobulin and/or IgG, or the presence of autoantibodies
III Histopathologic criterion	Marked lymphoplasmacytic infiltration and dense fibrosis

For diagnosis, criterionImust be present, together with criterion II and/ or III.

Table 2 Italian diagnostic criteria for autoimmune pancreatitis

Criteria	Definition
CriterionI	Histology and cytology
Criterion II	Association with other postulated autoimmune disease
Criterion III	Response to steroid therapy

One or more criteria must be present in order to diagnose autoimmune pancreatitis.

Table 3 Korean diagnostic criteria for autoimmune pancreatitis released by the Asian Medical Center

Criteria	Definition
CriterionI	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 steroids

Definite diagnosis: CriterionIand any of criteria II-IV.

Table 4 A proposal of revised Korean diagnostic criteria for autoimmune pancreatitis

Criteria	Definition
CriterionI	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 with other postulated autoimmune disease

Definite diagnosis: I + II + III + IV or I + II + III or I + II or I + III; Probable diagnosis: I + IV (Rediagnosed as “definite” if “response to steroids” is present); Possible diagnosis:I(Rediagnosed as “definite” if “response to steroids” is present).

Table 5 Clinical and morphological data of autoimmune pancreatitis patients with recurrent pancreatitis

Study	Type of study	Patients studied	Associated disease	Median FU (mo)	FU range (mo)	Criteria adopted	Imaging alterations	IgG elevation	IgG4 elevation	Antibodies present
Borum[140]	CR	2	SLE		132-253	Histology	Yes	NR	NR	Yes
Wakabayashi[13]	R	7	5 U		28-75	JPS	Yes	5/7	NR	1/8
Hardacre[142]	R	37	1 SS, 2 UC	33	NR	Histology	NR	NR	NR	NR
Weber[145]	P	31	1 BP + LD + F, 1 IN,	38	0-72	Histology	Yes	NR	NR	NR
			1 Pa, 1 RA,
			1 SS+GD+AI,
			1 UC+GS+G
Fernandez-del Castillo[141]	CR	1	None	5		Histology	Yes	NR	NR	NR
Zamboni[146]	R	53	2 SS, 1 SS + T, 1 SS + RF, 2 CD, 1 CD + RA, 3 UC, 4 U	361	6-2761	Histology	NR	NR	NR	NR
Tanvetyanon[143]	CR	1	MS	14		JPS	Yes	NR	No	No
Wayne[144]	R	6	None	60	48-240	Histology	3/6	NR	NR	1/7
Abisi[139]	CR	1	IN, SS, LA	NR	NR	Histology	Yes	Yes	NR	Yes

FU: Follow-up; CR: Case report; R: Retrospective; P: Prospective; JPS: Japanese Pancreatic Society criteria for autoimmune pancreatitis; NR: Not reported; SLE: Systemic lupus erythematosus; SS: Sjogren syndrome; UC: Ulcerative colitis; BP: Bell's palsy; LD: Lyme disease; F: Fibromylagia; IN: Interstitial nephritis; Pa: Parotiditis; RA: Rheumatoid arthritis; GA: Grave's disease; AI: Adrenal insufficiency; GS: Granulomatous sialoadenitis; G: Goiter; T: Thyroiditis; RF: retroperitoneal fibrosis; CD: Crohn's disease; LA: Lupus anticoagulant; MS: Myelodysplastic sindrome: U: Unspecified.

¹The follow-up period has been reported for only 22 patients.

Table 6 Clinical characteristic, medical/surgical treatment and complications of autoimmune pancreatitis patients with recurrent pancreatitis

Study	No.patients with acute recurrent pancreatitis	Age at diagnosis (yr)	Sex	Localization	No. of recurrences	Surgery	No. of operation	Type of surgery	Steroid therapy	Improvement with steroids	Pancreatic complication
Borum[140]	1	34	Female	Tail	2	Yes	1	DP	Yes	No	Calcifications
											Pseudocyst
Wakabayashi[13]	3	NR	NR	NR	1	No			NR		NR
Hardacre[142]	1	NR	NR	NR	NR	Yes	1	NR	No		NR
Weber[145]	1	NR	NR	NR	1	No			No		NR
Fernandez-del Castillo[141]	1	36	Male	Head	> 2	Yes	1	PD	NR		NR
Zamboni[146]	1	49	Male	Head	1	Yes	1	PD	No		No
Tanvetyanon[143]	1	44	Male	Diffuse	> 10	No			Yes	Yes	Pseudocysts
Wayne[144]	5	30 (25-39)1	5 females	3 tail,	2-> 10	Yes	1 (1-3)*	4 DP,	1/5	No	1 pseudocyst,
				2 diffuse				2 PD,			1 calcifications
								1 PP,
								1 TP
Abisi[139]	1	78	Male	Diffuse	4	Yes	2	1 DP,	Yes	Yes	Calcifications
								1 CJ+G			Pseudocyst

DP: Distal pancreatectomy; PD: Pancreaticoduodenectomy; PP: Puestow procedure; TP: Total pancreatectomy; CJ: Choledochojejunostomy; G: Gastroenterostomy; NR: Not reported.

¹median and (range).