The Clinical Characters and Outcome of Antineutrophil Cytoplasmic Antibody-associated Vasculitis: A Retrospective Single-Center Study

抗嗜中性球細胞質抗體相關血管炎患者之臨床表現與治療反應：單一醫學中心之回顧性研究

呂政勳(Cheng-Hsun Lu) ； 沈玠妤(Chieh-Yu Shen) ； 吳政翰(Cheng-Han Wu) ； 余家利(Chia-Li Yu)

《Formosan Journal of Rheumatology》 27卷2期 (2013/12) Pp. 27-36

https://doi.org/10.6313/FJR.2013.27(02).02

摘要

目的：探討北部一家醫學中心之抗嗜中性球細胞質抗體相關血管炎患者之臨床表現、治療反應與預後。方法：蒐集2007年1月至2013年3月間，利用酵素免疫分析法檢驗抗嗜中性球細胞質抗體陽性，並於國立台灣大學附設醫院接受治療之患者。以病例回顧方式記錄患者臨床表現、侵犯器官範圍、相關檢驗結果，且評估患者之伯明翰血管炎活動指數。並統計分析患者之治療方式與成果，以及疾病復發。結果：本研究總共納入了26例新診斷抗嗜中性球細胞質抗體相關血管炎患者，當中19位為白血細胞髓過氧化酶抗體陽性，7位為蛋白酶3抗體陽性。其中百分之42為顯微型多血管炎，是最常見之診斷。本研究的患者中，腎臟與肺部是最常被侵犯的器官，分別占了百分之73與百分之69。白血細胞髓過氧化酶抗體陽性之患者中，百分之53在確診血管炎之前已有慢性腎病變。在追蹤期間共有6位患者於接受免疫調節治療後死亡（26%），感染是最常見之死因，共有五名患者死於感染併發症。而肺腎症候群、較高之伯明翰血管炎活動指數或腎臟受侵犯患者之日本血管炎活動指數，皆與患者之存活有關。結論：與過去東亞族群之研究相似，在本研究中，白血細胞髓過氧化酶抗體陽性血管炎比蛋白酶3抗體陽性血管炎更為常見，疾病分類則以顯微型多血管炎為最常見。肺腎症候群、伯明翰血管炎活動指數與日本血管炎活動指數皆可預測患者存活。及早診斷與治療，以及減少感染應可改善預後。

關鍵字

抗嗜中性球細胞質抗體(ANCA) ； 血管炎 ； 台灣 ； ANCA

並列摘要

Objective: To retrospectively review the classification, clinical course, treatment response and investigated prognostic factors. of patients with antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV). Methods: Patients with positive ANCA that received treatment at the National Taiwan University Hospital (NTUH) between January 1, 2007 and March 31, 2013 were enrolled. The range of involved organs was based on medical records, images and laboratory data. The Birmingham vasculitis activity score (BVAS) was evaluated for each patient. The treatments, clinical outcome and disease recurrences were recorded for statistical analyses. Results: Nineteen myeloperoxidase (MPO)-ANCA-positive patients and seven proteinase 3 (PR3)-ANCApositive patients had newly diagnosed AAV during the study period. Microscopic polyangiitis (MPA) was the most common classification (42% overall). Kidneys and lungs were the most frequently involved organs, with an incidence rate of 73% and 69%, respectively. Moreover, 53% of patients with MPO-AAV had chronic kidney disease before diagnosis. In addition, the all-cause mortality rate in this study was 26% and infection was the most common cause of death. Of the six expired patients, 5 died due to infectious complications. The higher BVAS, Japanese vasculitis activity score (JVAS) for patients with renal vasculitis, or involvement of both lung and kidney, has the higher all-cause mortality. Conclusions: AAV is not common, but does cause considerable morbidity and mortality. MPO-AAV was more common than PR3-AAV in this study, and the majority of the diagnoses were MPA. Both BVAS and JVAS predicted survival outcome. Early diagnosis combined with adequate treatment, and measures to prevent, reduce or monitor infection, may help with overall survival.

並列關鍵字

Antineutrophil cytoplasmic antibodies (ANCA) ； vasculitis ； Taiwan ； ANCA