The following information was submitted:
Transactions: WSEAS TRANSACTIONS ON BIOLOGY AND BIOMEDICINE
Transactions ID Number: 89-513
Full Name: Teumi Kamisawa
Position: Doctor (Researcher)
Age: ON
Sex: Male
Address: Department of Internal Medicine, Tokyo Metropolitan Komagome Hospital, 3-18-22 Honkomagome, Bunkyo-ku, Tokyo 113-8677,
Country: JAPAN
Tel: 81-3-3823-2101
Tel prefix:
Fax: 81-3-3824-1552
E-mail address: kamisawa@cick.jp
Other E-mails:
Title of the Paper: IgG4-related sclerosing disease
Authors as they appear in the Paper: Terumi Kamisawa
Email addresses of all the authors: kamisawa@cick.jp
Number of paper pages: 10
Abstract: Based on histological and immunohistochemical examination of various organs of autoimmune pancreatitis (AIP) patients, we have found dense infiltration of IgG4-positive plasma cells and T lymphocytes, as well as fibrosis in the peripancreatic retroperitoneal tissue, bile duct wall, gallbladder wall, periportal area of the liver, salivary glands, as well as the pancreas. Furthermore, all of the extrapancreatic lesions associated with AIP, such as sclerosing cholangitis, sclerosing sialadenitis, and retroperitoneal fibrosis, show infiltration of abundant IgG4-positive plasma cells. Both the pancreatic and the extrapancreatic lesions of AIP respond well to steroid therapy. Therefore, we proposed the existence of a novel clinicopathological entity, an gIgG4-related sclerosing diseaseh, and suggested that AIP is a pancreatic lesion of this systemic disease. Some inflammatory pseudotumors may be involved in this disease. In some cases, only 1 or 2 organs are clinically!
involved, while in others, 3 or 4 organs are affected. The disease occurs predominantly in elderly males, is frequently associated with lymphadenopathy, and responds well to steroid therapy. Serum IgG4 levels and immunostaining with anti-IgG4 antibody are useful in making the diagnosis. The precise pathogenesis and pathophysiology of IgG4-related sclerosing disease remain unclear. Since malignant tumors are frequently suspected on initial presentation, IgG4-related sclerosing disease should be considered in the differential diagnosis to avoid unnecessary surgery.
Keywords: IgG4-related sclerosing disease, autoimmune pancreatitis, IgG4, sclerosing cholangitis, sclerosing sialadenitis, retroperitoneal fibrosis
EXTENSION of the file: .doc
Special (Invited) Session: IgG4-related sclerosing disease
Organizer of the Session: 640-128 (Conference in the University of Cambridge, UK, Febr. 2010,)
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