A case of retroperitoneal and mediastinal fibrosis exhibiting elevated levels of IgG4 in the absence of sclerosing pancreatitis (autoimmune pancreatitis)
A case of retroperitoneal and mediastinal fibrosis exhibiting elevated levels of IgG4 in the absence of sclerosing pancreatitis (autoimmune pancreatitis)
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DOI：
10.1016/j.humpath.2005.11.001
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发表时间：
2006-02-01
期刊：
影响因子：
3.3
通讯作者：
Nakanuma, Y
中科院分区：
文献类型：
--
作者：
Zen, Y;Sawazaki, A;Nakanuma, Y
越来越多的证据表明，IgG 4与硬化性胰腺炎和硬化性涎腺炎等特发性硬化性病变密切相关。在这份报告中，我们描述了一例IgG 4相关的腹膜后和纵隔纤维化。一位52岁的男性，因背部隐痛而就诊，并被发现有一个持续环绕的主动脉旁肿块。从腹膜后取的活检标本显示弥漫性淋巴浆细胞浸润混合纤维化。免疫组化染色显示许多IgG 4阳性浆细胞。他的血清IgG 4浓度为392 mg/dL（参考范围，< 70）。我们用皮质类固醇治疗这个病人，它显著地减少了主动脉旁的质量沿着降低了他的血清IgG 4浓度。提示IgG 4可能参与了该患者腹膜后和纵隔纤维化的发病机制。IgG 4可能有助于腹膜后或纵隔纤维化的临床管理，以区分它们与恶性肿瘤，并预测类固醇敏感性。(c)2006年爱思唯尔公司All rights reserved.
There is now increasing evidence that IgG4 is closely involved ill idiopathic sclerosing lesions, such as sclerosing pancreatitis and sclerosing sialadenitis. In this report, we describe a case of IgG4-related retroperitoneal and mediastinal fibroses. A 52-year-old man presented with dull back pain and was found to have a continuously Surrounding paraaortic mass. A biopsy specimen taken from the retroperitoneum showed a diffuse lymphoplasmacytic infiltration intermixed with fibrosis. Many IgG4-positive plasma cells were demonstrated oil immunostaining. His serum IgG4 concentration was 392 mg/dL (reference range, < 70). We treated this patient with a corticosteroid, which markedly diminished the paraaortic mass along with lowering of his serum IgG4 concentration. The possible involvement of IgG4 was suggested in the pathogeneses of retroperitoneal and mediastinal fibroses in this patient. IgG4 might be useful in the clinical management of retroperitoneal or mediastinal fibrosis to differentiate them from malignant tumors and predict steroid sensitivity. (c) 2006 Elsevier Inc. All rights reserved.