Chinese Medical Sciences Journal ›› 2021, Vol. 36 ›› Issue (4): 342-345.doi: 10.24920/003871

• 病例报告 • 上一篇    下一篇

1例严重肺及下肢血管栓塞并轻微肾损害的髓过氧化物酶阳性的抗中性粒细胞胞浆抗体相关性血管炎:病例报告

廖中华1,冯俊涛2,*(),唐家乐1,罗丽颖1,李晓照1,*()   

  1. 1中南大学湘雅医院,肾病内科,湖南,长沙,410008
    2中南大学湘雅医院,呼吸与危重症医学科,湖南,长沙,410008
  • 收稿日期:2020-12-22 出版日期:2021-12-31 发布日期:2022-01-06
  • 通讯作者: 冯俊涛,李晓照 E-mail:jtfeng1976@hotmail.com;lixiaozhao@csu.edu.cn

Severe Pulmonary Embolism, Thrombosis of Lower Extremity, Unexpected Mild Renal Disorder in MPO-ANCA Associated Vasculitis: A Case Report

Zhonghua Liao1,Juntao Feng2,*(),Jiale Tang1,Liying Luo1,Xiaozhao Li1,*()   

  1. 1Department of Nephrology, National Key Clinical Specialty, Branch of National Clinical Research Center for Respiratory Disease, Xiangya Hospital, Central South University, Changsha 410008, China
    2Department of Respiratory and Critical Care Medicine, National Key Clinical Specialty, Branch of National Clinical Research Center for Respiratory Disease, Xiangya Hospital, Central South University, Changsha 410008, China
  • Received:2020-12-22 Published:2021-12-31 Online:2022-01-06
  • Contact: Juntao Feng,Xiaozhao Li E-mail:jtfeng1976@hotmail.com;lixiaozhao@csu.edu.cn

摘要:

髓过氧化物酶阳性的抗中性粒细胞胞浆抗体相关性血管炎(myeloperoxidase antineutrophil cytoplasmic antibody- associated vasculitis,MPO-AAV)是一类以严重的多器官损害为特征的自身免疫性疾病,尤以急性肾功能衰竭为突出表现。本文报道了1例65岁的老年女性,因6月来进展性的呼吸困难,发热、咯血痰、下肢疼痛并间歇性跛行2天入院。通过一系列详尽检查发现严重的肺动脉栓塞及动静脉血栓,肾脏损害相对轻微,核周型抗中性粒细胞胞浆抗体(perinuclear antineutrophil cytoplasmic antibody,P-ANCA)及抗髓过氧化物酶抗体(myeloperoxidase antineutrophil cytoplasmic antibody, MPO-ANCA)呈阳性。排除其他因素导致多器官损伤及血栓形成可能性,此患者被诊断为MPO-AAV。给予糖皮质激素,环磷酰胺及抗凝治疗后,患者的临床症状及肺栓塞明显改善。

关键词: ANCA相关性血管炎, 髓过氧化物酶, 肺栓塞, 动脉栓塞

Abstract:

Myeloperoxidase antineutrophil cytoplasmic antibody (MPO-ANCA) associated vasculitis is an autoimmune disease usually with severe multiple dysfunction syndrome, especially prominent acute renal failure. A 65-year-old woman was admitted with progressive dyspnoea for six months and fever, sputum with blood, pain of the lower extremities and intermittent claudication for two days, indicating multiple organ involvement (respiratory system, blood vessels). The renal involvement was relatively mild, presenting with microscopic haematuria. The chest computed tomography demonstrated multiple pulmonary embolisms. Ultrasound and computed tomography angiography for the lower extremity vessels showed venous and arterial thrombosis. Exclusion of other diseases that can cause multiple organ damage and thrombosis, the positive perinuclear ANCA and MPO-ANCA strongly support the diagnosis of MPO-ANAC-associated vasculitis. The patient’s physical condition has been greatly improved by treatment with corticosteroids and anticoagulation.

Key words: antineutrophil cytoplasmic antibody-associated vasculitis, myeloperoxidase, pulmonary embolism, arterial embolism

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