13例嗜酸性肉芽肿性多血管炎临床特点观察
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(中国人民解放军总医院第一医学中心呼吸与危重症医学科,北京市 100853)

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张歆刚,硕士,副主任医师,研究方向为呼吸系统感染及少见疑难病,E-mail为zhangxg301@126.com。

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全军后勤科研计划重点课题(BWS14c051)


Clinical characteristics of 13 cases of eosinophilic granulomatosis with polyangiitis
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Department of Respiratory and Critical Care Medicine, First Medical Center, General Hospital of Chinese People's Liberation Army, Beijing 100853, China)

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    摘要:

    目的 探讨嗜酸性肉芽肿性多血管炎(EGPA)的临床表现、病理及影像学特征,以提高临床诊治水平。方法 回顾性分析2006年1月—2021年4月于解放军总医院确诊的13例EGPA患者的临床资料,包括基本信息、临床表现、受累器官情况、血液学及影像学检查、病理检查和治疗、随访情况。结果 13例EGPA患者中男7例,女6例;年龄27~60岁,中位年龄37岁。受累器官包括呼吸系统、神经系统、消化系统、心血管系统、泌尿系统、皮肤、耳鼻喉眼等。10例白细胞升高,11例嗜酸性粒细胞(Eos)升高。10例IgE升高。6例抗中性粒细胞胞质抗体阳性。10例行病理检查,显示Eos浸润明显。所有患者均接受激素和免疫抑制剂治疗。随访13例,4例复发,1例死亡。结论 EGPA主要临床特点为外周血Eos增多及病理组织Eos浸润,病变累及多个系统,以呼吸系统最常见。病理活检可协助诊断。激素及免疫抑制剂治疗EGPA有效。

    Abstract:

    Aim To investigate the clinical manifestations, pathological and imaging features of eosinophilic granulomatosis with polyangiitis (EGPA) in order to improve the level of clinical diagnosis and treatment. Methods The clinical data of 13 patients with EGPA diagnosed in General Hospital of Chinese People's Liberation Army from January 2006 to April 2021 were analyzed retrospectively, including basic information, clinical manifestations, conditions of involved organs, hematology and imaging examinations, pathological examinations and treatments, and follow-up happening.Results Among the 13 patients with EGPA, 7 were males and 6 were females; aged from 27 to 60 years old, with a median age of 37 years. The involved organs include respiratory system, nervous system, digestive system, cardiovascular system, urinary system, skin, ear, nose, throat, eyes, and etc. Leukocytosis was found in 10 patients, and eosinophils (Eos) increasing was found in 11 patients. Elevated IgE was found in 10 patients. 6 cases were positive for antineutrophil cytoplasmic autoantibody. 10 cases of pathological examination showed obvious Eos infiltration. All patients were treated with hormone and immunosuppressant. 13 cases were followed up, 4 cases recurred and 1 case died. Conclusions The main clinical features of EGPA are increased Eos in peripheral blood and Eos infiltration in pathological tissues, involving multiple systems, especially the respiratory system. Pathological biopsy is helpful for diagnosis. Hormone and immunosuppressant are effective in the treatment of EGPA.

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张歆刚,张明月,韩国敬.13例嗜酸性肉芽肿性多血管炎临床特点观察[J].中国动脉硬化杂志,2021,29(8):713~717.

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  • 收稿日期:2021-05-19
  • 最后修改日期:2021-05-29
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  • 在线发布日期: 2021-08-10