Lymphoplasmacytic sclerosing pancreatitis forming a localized mass: a variant form of autoimmune pancreatitis |
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Authors: | Go Kobayashi Naotaka Fujita Yutaka Noda Kei Ito Jun Horaguchi Osamu Takasawa Takashi Obana Kazunari Nakahara Miwa Uzuki Takashi Sawai |
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Institution: | (1) Department of Gastroenterology, Sendai City Medical Center, 5-22-1 Tsurugaya, Miyagino-ku, Sendai 983-0824, Japan;(2) First Department of Pathology, Iwate Medical University School of Medicine, Morioka, Japan |
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Abstract: | Background To elucidate the correlation of autoimmune pancreatitis (AIP) and mass-forming pancreatitis, in which a localized mass is
formed in the pancreas.
Methods Nine cases of mass-forming pancreatitis were divided into two groups, one consisting of cases that met the histological diagnostic
criteria for AIP by the Japan Pancreas Society (JPS) and the other consisting of cases which did not. Histological findings,
immunohistological findings, and pancreatograms were compared between these groups.
Results Six cases met the histological criteria of JPS (group A) and the other three did not (group B). In the mass-forming portion
in group A, the wall of the pancreatic duct showed marked thickening. However, the epithelium was well preserved, and dilatation
of the branch ducts or protein plugs were rarely observed. All cases showed marked obliterative phlebitis. The IgG4 labeling
index (LI) was 25% or more in all but one case. In the portion other than the mass, the lobular structure was well preserved
and the IgG4 LI was less than 10%. The pancreatogram showed localized stenosis or obstruction at the site of the mass associated
with a normal-appearing main pancreatic duct in the remaining portion. In group B, histological findings typical of chronic
pancreatitis with dilated branch ducts and protein plugs were observed. Obliterative phlebitis was not confirmed. The IgG4
LI in the mass-forming portion was low (2.3%–11.1%).
Conclusions There exists a subgroup of AIP showing localized mass formation and stenosis or obstruction of the main pancreatic duct with
prominent obliterative phlebitis associated with a normal ductal segment. |
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Keywords: | lymphoplasmacytic sclerosing pancreatitis mass-forming pancreatitis autoimmune pancreatitis obliterative phlebitis IgG4 |
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