Department of Diagnostic Pathology, Kurume University Hospital, Japan. nomura
Pathol Res Pract. 2011 Aug 15;207(8):518-21. doi: 10.1016/j.prp.2011.05.009. Epub 2011 Jul 14.
IgG4-related disease has been recognized as a systemic syndrome characterized by mass-forming lesions with lymphoplasmacytic infiltration and sclerosis. This disease has been identified in various sites, including the pancreas, retroperitoneum, lung, head, and neck. Herein we report a case of IgG4-related sclerosing mesenteritis. An 82-year-old woman was admitted to our hospital due to persistent abdominal pain. Abdominal computed tomography demonstrated a solitary mass with a maximal diameter of 11.7cm in mesentrium of the small intestine. On her laboratory examination, only C-reactive protein level was elevated. Although the pre-operative diagnosis was indefinite, she underwent ileocecectomy. Grossly, an elastic soft mass with foci of hemorrhage was seen in the mesentrium. Microscopically, the lesion was composed of fibroblastic or myofibroblastic spindle cells with abundant stromal fibrosis and inflammatory infiltrate, such as lymphocytes and plasma cells accompanied by lymphoid follicles with a germinal center. Obstructive phlebitis was observed. Immunohistochemically, numerous IgG4-positive plasma cells were observed, and the IgG4/IgG ratio was 75.9%. The serum level of IgG4 examined at post-operation was high. These findings suggested that this lesion was consistent with IgG4-related sclerosing mesenteritis.
IgG4 相关疾病已被认为是一种以肿块形成病变伴淋巴浆细胞浸润和硬化为特征的系统性综合征。这种疾病已在各种部位被识别，包括胰腺、腹膜后、肺、头部和颈部。在此，我们报告一例 IgG4 相关硬化性肠系膜炎。一名 82 岁女性因持续性腹痛入院。腹部计算机断层扫描显示小肠系膜有一个直径最大为 11.7cm 的单发肿块。在她的实验室检查中，只有 C 反应蛋白水平升高。尽管术前诊断不确定，但她接受了回盲切除术。大体上，在肠系膜可见一个弹性软肿块，伴有出血灶。显微镜下，病变由成纤维细胞或肌纤维母细胞样梭形细胞组成，伴有丰富的间质纤维化和炎症浸润，如淋巴细胞和浆细胞，伴有生发中心的淋巴滤泡。观察到阻塞性静脉炎。免疫组化染色显示大量 IgG4 阳性浆细胞，IgG4/IgG 比值为 75.9%。术后检查的血清 IgG4 水平较高。这些发现表明该病变符合 IgG4 相关硬化性肠系膜炎。
