IgG4-related disease

IgG4関連疾患は21世紀になって提唱された新しい疾患である｡組織学的にはIgG4陽性形質細胞やリンパ球浸潤が涙腺, 唾液腺, 後腹膜, 膵臓, 胆管などで起こり, 臨床的にはMikulicz病, 後腹膜線維症, 自己免疫膵炎, 糖尿病, 原発性硬化性胆管炎類似の胆管病変などを呈する全身性疾患であり, ステロイド治療に対する良好な反応性を認める｡その診断基準は確立されておらず, われわれは, ①血清IgG4の高値, ②本疾患に特徴的な臓器の障害(唾液腺,涙腺, 膵臓, 後腹膜), ③組織学的にIgG4陽性形質細胞とリンパ球の浸潤の確認, の３項目のうち２項目以上認めれば, IgG4関連疾患とするという診断基準を提言する｡IgG4-related disease is a new concept which was established in 21st century. Histologically, infiltrations of IgG4-positive plasma cells and lymphocytes occur in the lacrimal gland, salivary gland , retroperitoneum , pancreas , and biliary tract. IgG4-related disease is a systematic disease presenting symptoms such as Mikulicz's disease, retroperitoneal fibrosis, autoimmune pancreatitis, diabetes and sclerotic cholangitis, and oral steroid therapy is effective. In this paper, we propose diagnostic criteria for IgG4-related disease that have not been established yet. If two (or more) of three following features are fulfilled, IgG4-related disease is diagnosed ； elevated levels of serum IgG4, involvement of organs which is characteristic in IgG4-related disease (salivary gland, lacrimal gland, pancreas and retroperitonium) ,histological findings with IgG4-positive plasma cells and lymphocytes