caa a22 4500 445887788 CHVBK 20180317145605.0 cr unu---uuuuu 170323e20110101xx s 000 0 eng 10.1007/s00535-010-0317-2 doi (NATIONALLICENCE)springer-10.1007/s00535-010-0317-2 Serum IgG4-negative autoimmune pancreatitis [Elektronische Daten] [Terumi Kamisawa, Kensuke Takuma, Taku Tabata, Yoshihiko Inaba, Naoto Egawa, Koji Tsuruta, Tsunekazu Hishima, Tsuneo Sasaki, Takao Itoi] Background: Autoimmune pancreatitis (AIP) is considered to be a pancreatic lesion of IgG4-related systemic disease, but about 20% of AIP patients do not have elevated serum IgG4 levels. This study aimed to clarify the pathophysiology of AIP patients without elevated serum IgG4 levels. Methods: Fifty-eight AIP patients were divided into 2 groups: those with elevated serum IgG4 levels (>135mg/dl; SIgG4-positive AIP) and those without (SIgG4-negative AIP). The 2 groups' clinical, serological, radiological, and histological findings, as well as salivary and lacrimal gland function, were compared. Results: Serum IgG4 levels were elevated in 45 AIP patients and normal in 13 patients. In SIgG4-negative AIP patients, the female ratio tended to be high; obstructive jaundice was less likely; abdominal pain and acute pancreatitis were more likely; segmental swelling of the pancreatic body and/or tail was more common; sclerosing extrapancreatic lesions, salivary and lacrimal gland dysfunction, and abundant infiltration of IgG4-positive plasma cells in the gastric mucosa were less likely; and conservative follow-up was sometimes implemented. Histological examination of the pancreas of SIgG4-negative AIP showed lymphoplasmacytic sclerosing pancreatitis (LPSP) rather confined to the pancreas (n=4), inadequate material (n=2), and pancreatic fibrosis showing infiltration of lymphocytes without infiltration of IgG4-positive cells or neutrophils (n=2). Conclusions: Clinicopathological features of SIgG4-negative AIP differed from those of SIgG4-positive AIP. Some SIgG4-negative AIP cases are LPSP rather confined to the pancreas. SIgG4-negative AIP may include idiopathic duct-centric pancreatitis (IDCP) or sclerosing pancreatitis other than LPSP or IDCP, but further studies are needed to clarify this issue. Springer, 2010 Autoimmune pancreatitis nationallicence IgG4 nationallicence Lymphoplasmacytic sclerosing pancreatitis nationallicence Idiopathic duct-centric pancreatitis nationallicence Kamisawa Terumi Department of Internal Medicine, Tokyo Metropolitan Komagome Hospital, 3-18-22 Honkomagome, Bunkyo-ku, 113-8677, Tokyo, Japan aut Takuma Kensuke Department of Internal Medicine, Tokyo Metropolitan Komagome Hospital, 3-18-22 Honkomagome, Bunkyo-ku, 113-8677, Tokyo, Japan aut Tabata Taku Department of Internal Medicine, Tokyo Metropolitan Komagome Hospital, 3-18-22 Honkomagome, Bunkyo-ku, 113-8677, Tokyo, Japan aut Inaba Yoshihiko Department of Internal Medicine, Tokyo Metropolitan Komagome Hospital, 3-18-22 Honkomagome, Bunkyo-ku, 113-8677, Tokyo, Japan aut Egawa Naoto Department of Internal Medicine, Tokyo Metropolitan Komagome Hospital, 3-18-22 Honkomagome, Bunkyo-ku, 113-8677, Tokyo, Japan aut Tsuruta Koji Department of Surgery, Tokyo Metropolitan Komagome Hospital, Tokyo, Japan aut Hishima Tsunekazu Department of Pathology, Tokyo Metropolitan Komagome Hospital, Tokyo, Japan aut Sasaki Tsuneo Department of Chemotherapy, Tokyo Metropolitan Komagome Hospital, Tokyo, Japan aut Itoi Takao Department of Gastroenterology and Hepatology, Tokyo Medical University, Tokyo, Japan aut Journal of Gastroenterology Springer Japan 46/1(2011-01-01), 108-116 0944-1174 46:1<108 2011 46 535 https://doi.org/10.1007/s00535-010-0317-2 text/html Onlinezugriff via DOI 1 research-article jats NATIONALLICENCE 856 40 https://doi.org/10.1007/s00535-010-0317-2 text/html Onlinezugriff via DOI NATIONALLICENCE 700 1- Kamisawa Terumi Department of Internal Medicine, Tokyo Metropolitan Komagome Hospital, 3-18-22 Honkomagome, Bunkyo-ku, 113-8677, Tokyo, Japan aut NATIONALLICENCE 700 1- Takuma Kensuke Department of Internal Medicine, Tokyo Metropolitan Komagome Hospital, 3-18-22 Honkomagome, Bunkyo-ku, 113-8677, Tokyo, Japan aut NATIONALLICENCE 700 1- Tabata Taku Department of Internal Medicine, Tokyo Metropolitan Komagome Hospital, 3-18-22 Honkomagome, Bunkyo-ku, 113-8677, Tokyo, Japan aut NATIONALLICENCE 700 1- Inaba Yoshihiko Department of Internal Medicine, Tokyo Metropolitan Komagome Hospital, 3-18-22 Honkomagome, Bunkyo-ku, 113-8677, Tokyo, Japan aut NATIONALLICENCE 700 1- Egawa Naoto Department of Internal Medicine, Tokyo Metropolitan Komagome Hospital, 3-18-22 Honkomagome, Bunkyo-ku, 113-8677, Tokyo, Japan aut NATIONALLICENCE 700 1- Tsuruta Koji Department of Surgery, Tokyo Metropolitan Komagome Hospital, Tokyo, Japan aut NATIONALLICENCE 700 1- Hishima Tsunekazu Department of Pathology, Tokyo Metropolitan Komagome Hospital, Tokyo, Japan aut NATIONALLICENCE 700 1- Sasaki Tsuneo Department of Chemotherapy, Tokyo Metropolitan Komagome Hospital, Tokyo, Japan aut NATIONALLICENCE 700 1- Itoi Takao Department of Gastroenterology and Hepatology, Tokyo Medical University, Tokyo, Japan aut NATIONALLICENCE 773 0- Journal of Gastroenterology Springer Japan 46/1(2011-01-01), 108-116 0944-1174 46:1<108 2011 46 535 Metadata rights reserved Springer special CC-BY-NC licence nationallicence BK010053 XK010053 XK010000 NATIONALLICENCE NATIONALLICENCE NL-springer