Clinical Characteristics of Autoimmune Pancreatitis

Mi Jin Kim; Cho Rong Oh; Kyu Taek Lee

doi:10.4166/kjg.2010.56.5.276

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Kim, Oh, and Lee: Clinical Characteristics of Autoimmune Pancreatitis

Review

Korean J Gastroenterol 2010;56(5):276-284.

Published online: 21 February 2010

DOI: https://doi.org/10.4166/kjg.2010.56.5.276

Clinical Characteristics of Autoimmune Pancreatitis

Mi Jin Kim, M.D., Cho Rong Oh, M.D., Kyu Taek Lee, M.D.

Department of Internal Medicine, Samsung Medical Center, Sungkyunkwan University College of Medicine, Seoul, Korea

Correspondence to: Kyu Taek Lee, M.D. Departments of Internal Medicine, Samsung Medical Center, Sungkyunkwan University College of Medicine, 50, Irwon- dong, Gangnam-gu, Seoul 135-710, Korea Tel: +82-2-3410-3409, Fax: +82-2-3410-6983 E-mail: happymap@skku.edu

Abstract

Korean autoimmune pancreatitis (AIP) criteria 2007 was aimed to diagnose the wide spectrum of AIP with high sensitivity. The most crucial issue when caring for patients with suspected AIP is to differentiate AIP from pancreatic cancer. Pancreatic cancer can be distinguished from AIP by pancreatic imaging, measurement of serum IgG4 levels, endoscopic ultrasound guided fine needle aspiration and trucut biopsy, and steroid trial. Autoimmune pancreatitis is a rare systemic fibroinflammatory disease which can affect not only the pancreas, but also a varie-ty of organs such as the bile ducts, salivary glands, retroperitoneum, and lymph nodes. Organs affected by AIP have a lymphoplasmacytic infiltrate rich in IgG4-positive cells. This inflammatory process responds dramatically to oral steroid therapy. Granulocytic epithelial lesion (GEL) positive AIP patients differ from GEL negative AIP patients in clinical features such as equal gender ratio, younger mean age, no increase in serum IgG4, no association with extrapancreatic involvement, no relapse, and frequent association with inflammatory bowel disease. Further investigation is needed to clarify the pathogenic mechanisms including more definite serological markers for theses two entities.

Keywords: Autoimmune pancreatitis, Pancreatic cancer, Cholangitis, Sclerosing, Granulocytic epithelial lesion

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Table 1.

Korean Criteria for Autoimmune Pancreatitis (Korean Pancreatobiliary Association, 2007)

Definite diagnosis: Criterion I together with any of criterion II to IV

Criterion I. Imaging (Both required)

1. Imaging (CT or MRI) of pancreatic parenchyma; Diffusely/segmentally/focally enlarged gland, occasionally with mass and/or hypoattenuation rim

2. Imaging (ERCP or MRCP) of pancreaticobiliary ducts; Diffuse/segmental/focal pancreatic ductal narrowing, often with the stensosis of bile duct

Criterion II. Serology (One required)

1. Elevated level of serum IgG or IgG4

2. Detected autoantibodies

Criterion III. Histopathology of pancreatic/extrapancreatic Lesions (One required)

1. Lymphoplasmacytic infiltration & fibrosis, often with obliterative phlebitis

2. Presence of abundant (＞10 cells/HPF) IgG4-positive plasma cells

Criterion IV. Response to steroids

1. Resolution/marked improvement of pancreatic/extrapancreatic lesion with steroid therapy

Probable diagnosis: Criterion V or VI

Criterion V.

1. Unexplained pancreatic disease but only with characteristic pancreatic histology

Criterion VI. (Both required)

1. Other organ involvement and/or serologic abnormalities

2. Various atypical pancreatic imaging suggesting chronic pancreatitis with negative workup for known etiologies

Table 2.

Clinicopathological Differences between LPSP and IDCP

	LPSP	IDCP
Age	Elderly	Relatively young
Gender	Male preponderance	No gender preponderance
Associated diseases
Sclerosing extrapancreatic lesions	Frequent	Rare
Acute pancreatitis	Rare	20-30%
Ulcerative colitis	Rare	20-30%
Elevation of serum IgG4 levels	Frequent	Rare
Relapse after therapy	20-40%	Rare
Pathologic findings
Ductal lesion
Epithelium	Intact	Often regenerative
Neutrophilic infiltration	Rare	Common within lumen and/or epithelium
Lymphoplasmacytic infiltration around the epithelium	Common; sometimes with storiform fibrosis	Common; no storiform fibrosis
Lobular lesion
Size of lobules	Normal to enlarged	Atrophic
Inflammatory cells	Lymphocytes and plasma cells	Neutrophils, lymphocytes and plasma cells
Abscess	Absent	Sometimes present
Interlobular lesion	Fibrotic tissue with lymphoplasmacytic infiltration	Loosely fibrotic tissue with fibroblasts and scarce inflammatory cells
Involvement of peripancreatic adipose tissue	Common; often like a sheath covering the pancreatic parenchyma	Usually minimal
Obliterative phlebitis	Common	Rare
IgG4-positive plasma cells	Numerous	Usually scarce

LPSP, lymphoplasmacytic sclerosing pancreatitis; IDCP, idiopathic duct centric pancreatitis.

^∗ This table was adopted from reference 55.

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