Type 2 autoimmune pancreatitis associated with severe ulcerative colitis: Three case reports

Table 1 Diagnostic criteria for type 2 autoimmune pancreatitis

	Level 1	Level 2
Clinical	A response to a steroid trial with rapid (< 2 wk) radiologically demonstrable resolution or marked improvement in manifestations	Clinically diagnosed inflammatory bowel diseases
Parenchymal imaging	Diffuse enlargement with delayed enhancement	Segmental/focal enlargement with delayed enhancement
Ductal imaging	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)
Histology	Granulocytic infiltration of duct wall, granulocytic epithelial lesions with or without granulocytic acinar inflammation and; absent or scant (0-10 cells/HPF) IgG4-positive cells	Granulocytic and lymphoplasmacytic acinar infiltrate and; again absent or scant (0-10 cells/HPF) IgG4-positive cells

Table 2 Diagnosis of definitive and probable type 2 autoimmune pancreatitis

	Imaging evidence	Collateral evidence
Probable	Level 1 or 2	Level 2 histology OR; IBD and response to steroids
Definitive	Level 1 or 2	Level 1 histology OR; IBD, level 2 histology and response to steroids

OR: Odds ratio; IBD: Inflammatory bowel diseases.

Table 3 Case summaries

	Case 1	Case 2	Case 3
Gender	Male	Male	Female
Age at IBD diagnosis	54	56	46
Extent of UC (Montreal)	A3 E3	A3 E3	A3 E3
Extraintestinal manifestations	Recurrent mouth ulceration	Nil	Nil
Surgery	Colectomy	Colectomy	Nil
Occurrence of cancers	Nil	Nil	Nil
Age at AIP diagnosis	55	62	46
AIP type	Probable type 2	Probable type 2	Definitive type 2
Other organ involvement	Nil	Nil	Nil
Presentation	Obstructive jaundice	Obstructive jaundice	Acute pancreatitis
IBD or AIP first	IBD	IBD	IBD
Treatment	Corticosteroids	Corticosteroids	Corticosteroids
Relapse	Yes (spontaneous resolution)	Nil	Yes (treated with corticosteroids)
Evolution (diabetes, exocrine insufficiency)	Diabetes	Diabetes, Exocrine insufficiency	Nil

IBD: Inflammatory bowel diseases; UC: Ulcerative colitis; AIP: Autoimmune pancreatitis.