[IgG4相关性腹膜后纤维化的临床特征]

[Clinical characteristics of IgG4-related retroperitoneal fibrosis].

作者信息

Chen Hua, Lin Wei, Yan Linyi, Zhang Haiting, Wu Qingjun, Wang Li, Fei Yunyun, Su Jinmei, Wang Qian, Li Jing, Zhang Wen, Tian Xinping, Zhang Xuan, Zhao Yan, Zeng Xiaofeng, Zhang Fengchun

机构信息

Department of Immunology & Rheumatology, Peking Union College Hospital & Chinese Academy of Medical Sciences, Key Laboratory of Immunological & Rheumatologic Diseases under Ministry of Education, Beijing 100032, China.

出版信息

Zhonghua Yi Xue Za Zhi. 2014 Oct 28;94(39):3079-81.

PMID:25549682

Abstract

OBJECTIVE

To explore the clinical characteristics of IgG4-related retroperitoneal fibrosis (RPF) in China.

METHODS

From December 2010 to September 2013, a total of 107 IgG4-related disease (IgG4-RD) patients were reviewed prospectively. Their clinical, laboratory and histopathological features were summarized. And the treatment responses were analyzed.

RESULTS

Among them, 22 RPF cases were identified. The male-to-female ratio and age were 4.5: 1 and 63 ± 11 years respectively. The follow-up period was 3-28 months. The most common onset symptom was low back pain (55%). Multiple organ involvements were documented in 20 patients (91%), including lymphadenopathy (n = 9), aortitis (n = 8), sialadenitis (n = 7), dacryoadenitis (n = 5) and autoimmune pancreatitis (n = 4). Allergic histories were reported by10 subjects (46%). Eosinophilia was present in 6 subjects (27%). Serum IgG4 levels were significantly elevated in all subjects. Serum total IgE level was elevated in 12/13 tested subjects. Tissue biopsies of 12 subjects revealed massive lymphocytic infiltration and obvious tissue fibrosis. Immunohistochemical staining showed a massive infiltration of IgG4-positive plasma cell. Glucocorticoid was administrated in all subjects. Most subjects received combined therapy with immunosuppressive agents or tamoxifen.

CONCLUSION

IgG4-related RPF is a systemic autoimmune disease with favorable responses to the treatments of glucocorticoid and immunosuppressive agents.

摘要

目的

探讨中国IgG4相关性腹膜后纤维化（RPF）的临床特征。

方法

回顾性分析2010年12月至2013年9月期间前瞻性纳入的107例IgG4相关性疾病（IgG4-RD）患者。总结其临床、实验室及组织病理学特征，并分析治疗反应。

结果

其中确诊为RPF的患者22例。男女比例为4.5∶1，年龄为（63±11）岁。随访时间为3～28个月。最常见的首发症状为腰痛（55%）。20例患者（91%）存在多器官受累，包括淋巴结病（9例）、主动脉炎（8例）、涎腺炎（7例）、泪腺炎（5例）和自身免疫性胰腺炎（4例）。10例患者（46%）有过敏史。6例患者（27%）有嗜酸性粒细胞增多。所有患者血清IgG4水平均显著升高。13例接受检测的患者中12例血清总IgE水平升高。12例患者的组织活检显示大量淋巴细胞浸润及明显的组织纤维化。免疫组化染色显示IgG4阳性浆细胞大量浸润。所有患者均接受糖皮质激素治疗。大多数患者接受了免疫抑制剂或他莫昔芬的联合治疗。