IgG4-related sclerosing disease

查看全文 作　　者：Terumi [1]Kamisawa;Atsutake [2]Okamoto

机构地区：[1]Department of Internal Medicine, Tokyo Metropolitan Komagome Hospital;[2]Department of Surgery, Tokyo Metropolitan Komagome Hospital

出　　处：《World Journal of Gastroenterology》索引2008年第14卷第25期,共8页

摘　　要：Based on histological and immunohistochemical exami-nation of various organs of patients with autoimmune pancreatitis (AIP), a novel clinicopathological entity of IgG4-related sclerosing disease has been proposed. This is a systemic disease that is characterized by ex-tensive IgG4-positive plasma cells and T-lymphocyte inf iltration of various organs. Clinical manifestations are apparent in the pancreas, bile duct, gallbladder, sali-vary gland, retroperitoneum, kidney, lung, and pros-tate, in which tissue fi brosis with obliterative phlebitis is pathologically induced. AIP is not simply pancreatitis but, in fact, is a pancreatic disease indicative of IgG4-related sclerosing diseases. This disease includes AIP, sclerosing cholangitis, cholecystitis, sialadenitis, retro-peritoneal fi brosis, tubulointerstitial nephritis, intersti-tial pneumonia, prostatitis, inflammatory pseudotumor and lymphadenopathy, all IgG4-related. Most IgG4-related sclerosing diseases have been found to be as-sociated with AIP, but also those without pancreatic involvement have been reported. In some cases, only one or two organs are clinically involved, while in oth-ers, three or four organs are affected. The disease occurs predominantly in older men and responds well to steroid therapy. Serum IgG4 levels and immunos-taining with anti-IgG4 antibody are useful in making the diagnosis. Since malignant tumors are frequently suspected on initial presentation, IgG4-related scleros-ing disease should be considered in the differential diagnosis to avoid unnecessary surgery. 关键词：自身免疫性疾病肝硬化 IGG4 腹膜纤维化

相关文献

参考文献（70）
引证文献（55）
耦合文献（8）

参考文献(70)

引证文献(55)

耦合文献(8)

互联网出版许可证新出网证(渝)字10号全国400电话 - 免长途话费

IgG4-related sclerosing disease

参考文献(70)

引证文献(55)

耦合文献(8)

用户提示