Korean J Gastroenterol.  2011 Jul;58(1):53-57. 10.4166/kjg.2011.58.1.53.

Steroid Responsive Pancreatic Mass-Forming Type 2 Autoimmune Pancreatitis

• Chun YJ
• Chang JH
• Lee IS
• Lee JE
• Chung MK
• Jung CK
• Choi MG
• Chung IS

• KMID: 1792816
• DOI: http://doi.org/10.4166/kjg.2011.58.1.53

Abstract

Autoimmune pancreatitis (AIP) has two distinct subsets. Type 1 AIP or lymphoplasmacytic sclerosing pancreatitis is systemic disease with the elevation in serum levels of the IgG4. Type 2 AIP, also called duct-centric pancreatitis, features granulocyte epithelial lesions with duct obstruction in the pancreas without systemic involvement. Here, we report a case of type 2 AIP diagnosed by pathology, which is the first report in Korea. The case is a 56-year-old woman who presented with anorexia and vomiting. Computed tomography revealed mass-like lesion in the pancreatic head and the compression of the distal common bile duct and the head portion of the main pancreatic duct. Serum levels of the IgG4 were normal. Histologic examination revealed a dense neutrophil infiltration in the pancreatic parenchyme associated with extensive fibrosis, thereby confirming the diagnosis of type 2 AIP. The abnormalities in the clinical, laboratory, and radiological findings improved after oral steroid treatment.

Keyword

Pancreatitis; Autoimmune diseases

MeSH Terms

Autoimmune Diseases/blood/immunology/*pathology
Female
Fibrosis
Humans
Immunoglobulin G/blood
Magnetic Resonance Imaging
Middle Aged
Neutrophils/immunology
Pancreas/pathology
Pancreatitis/*drug therapy/immunology/pathology
Steroids/*therapeutic use
Tomography, X-Ray Computed

Figure

• 

  Fig. 1. Abdominal CT and MRI/MRCP findings. (A) Abdominal CT showed bulging contour, mass-like lesion in the head of the pancreas (arrow). (B, C) Pancreas and bile duct MRI/MRCP showed diffuse swelling of the pancreas head and compression of the distal common bile duct and proximal pancreatic duct, resulting in dilatation of the extrahepatic bile duct and pancreatic duct (arrows).

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  Fig. 2. Pathological findings. (A) Biopsy showed focal fibrosis and parenchymal destruction of pancreas (H&E stain, ×100). (B) Higher magnification showed diffuse infiltration of neutrophils in the parenchyme of the pancreas (arrow) in the epithelial cells of the ductule (circle)(H&E stain,×400). L, lumen.

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  Fig. 3. Abdominal CT findings. (A) Follow up CT scan after 15 days from the start of prednisolone. Globular swelling of the pancreas head decreased comparing with Fig. 1A. (B) Follow up CT scan after 2 and half months from the start of predni-solone showed marked decrease of pancreas swelling.

Reference

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