嗜中性球細胞質抗體相關血管炎：單一醫學中心病例回顧

Anti-neutrophil Cytoplasmic Antibody-associated Vasculitis: Experience from a Single Institute

施凱翔(Kai-Hsiang Shih) ； 梁凱莉(Kai-Li Liang) ； 顏廷廷(Ting-Ting Yen)

《台灣耳鼻喉頭頸外科雜誌》 55卷2期 (2020/06) Pp. 69-75

https://doi.org/10.6286/jtohns.202006_55(2).069

摘要

背景：嗜中性球細胞質抗體相關血管炎經常影響上下呼吸道且表徵多變，因此，耳鼻喉科醫師常是患者第一位求診的對象。血管炎在台灣本土少見，本研究的目的在了解台灣地區血管炎之患者臨床表現。方法：本研究主要回顧本院近13年中，以耳鼻喉症狀為初期表現之血管炎患者，分析其臨床表現、診斷與治療結果。結果：2004年3月到2017年3月間，本部門共診斷11名血管炎病患。所有病人皆有鼻部症狀；包括鼻塞、鼻出血、膿鼻涕或馬鞍鼻。5名病人有耳部症狀；包括耳漏、聽力減退、耳鳴、或顏面神經麻痺。此外，2名患者有聲音沙啞和3名有唾液腺腫脹之頭頸或喉部病徵。所有患者中，10名診斷有鼻竇炎，5名有中耳炎，5名有下呼吸道之侵犯，4名有腎臟侵犯，5名有粘膜皮膚侵犯，5名有眼侵犯，4名有中樞神經或中樞侵犯，1名有腸胃潰瘍性穿孔。所有患者於診斷後接受治療，其中1名於診斷後4年因敗血性休克併呼吸衰竭死亡，其他10名至本研究結束時持續接受治療。結論：台灣人血管炎類疾病發生率非常低，且初期表現多樣性，較難與一般發炎性疾病區分，但都有耳鼻喉症狀表現，耳鼻喉科醫師在患者臨床表現不典型時應納入鑑別診斷。

關鍵字

anti-neutrophil cytoplasmic antibody ； vasculitis（嗜中性球細胞質抗體 ； 血管炎）

並列摘要

BACKGROUND: Anti-neutrophil cytoplasmic antibody-associated vasculitis (AAV) is a rare autoimmune disease usually presenting with otorhinolaryngological manifestations. The otolaryngologist is the first physician contacted by the majority of AAV patients. METHODS: A retrospective chart review from 2004 to 2017 was conducted at the study hospital. Patients diagnosed with AAV according to the criteria of the 2012 Chapel Hill Consensus Conference were enrolled. Their clinical presentation, and diagnostic and treatment results were analyzed. RESULTS: A total of 11 AAV patients were enrolled in this study. All patients presented with sinonasal symptoms including nasal obstruction, epistaxis, nasal purulence, and saddle nose. Five presented with otorrhea, hearing impairment, tinnitus, and facial palsy. Two had a husky voice, and 3 had swollen salivary glands. Our AAV patients were diagnosed with sinusitis (n=10), otitis media (n=5), lung diseases (n=5), renal diseases (n=4), mucocutaneous involvement (n=5), orbital involvement (n=5), cranial nerve/central nervous system involvement (n=4), and intestinal perforation (n=1). All 11 patients received treatment afterwards including corticosteroid, cyclophosphamide, and rituximab. One patient died of septic shock and respiratory failure because of pneumonia 4 years after diagnosis. CONCLUSIONS: AAV patients in this study presented with a great variety of symptoms. Differentiating this disease from other inflammatory diseases is difficult. The otolaryngologist is one of the first of several physicians to be contacted by the majority of AAV patients. The diagnosis of AAV must be taken into consideration in patients with atypical symptoms or those who are unresponsive to standard treatment.

並列關鍵字

anti-neutrophil cytoplasmic antibody ； vasculitis