- 期刊
Granulomatosis with Polyangiitis Initially Presenting with Sinonasal Tumor: A Case Report
以鼻竇腫瘤為最初表現的肉芽腫併多發性血管炎：病例報告
摘要
韋格納氏肉芽腫（Wegener's granulomatosis）於2011年1月被三家學會建議更名為肉芽腫併多發性血管炎（granulomatosis with polyangiitis）。耳鼻及喉部侵犯是最常見的臨床表現，副鼻竇炎是很常見的症狀，緊接著是發燒、關節痛、咳嗽、鼻炎、咳血、耳炎及眼球發炎。此類病人初期可能僅表現上呼吸道症狀，例如鼻腔阻塞、流鼻血，所以可能造成延遲診斷及治療。我們提出的一個46歲中年男性因起初以慢性臉部疼痛及麻與流鼻血持續約3週，被發現有右鼻竇腫瘤。胸腔X光檢查呈現左上肺葉開洞腫塊。這個病人的診斷是由右側鼻病灶病理切片呈現壞死性肉芽腫，及陽性抗嗜中性白血球細胞質抗體確立診斷。病人在脈衝式療法（pulse therapy with high dose corticosteroid）後又再度復發，選擇標靶治療藥物rituximab（anti-CD 20）當作替代式療法，接著使用低劑量類固醇併hydrochloroquine 及mycophenolate mofetil當作維持性療法（maintenance therapy），且經過三個月後，上呼吸道及臉部症狀改善且左上葉開洞病灶也幾乎消失。
並列摘要
Ear, nose and throat involvement is the most common clinical manifestation of granulomatosis with polyangiitis (GPA) or Wegener's granulomatosis, and sinusitis is the most frequent presenting symptom, followed by fever, arthralgia, cough, rhinitis, hemoptysis, otitis, and ocular inflammation. Patients with GPA may present upper respiratory symptoms such as nasal obstruction and epistaxis initially. However, this would lead to a delayed diagnosis and treatment. A 46-year-old man presented with chronic facial pain, numbness, and epistaxis for 3 weeks. Nasal inspection showed an ulcerating tumor in the right osteomeatal complex region. Chest film showed a cavitary mass in the left upper lobe. GPA was confirmed by evidence of necrotizing granulomatous inflammation of nasal tissue and positive cytoplasmic antineutrophil cytoplasmic antibody (c-ANCA). Flare-up of GPA occurred after pulse therapy with high-dose corticosteroid followed by low-dose steroid; anti-CD 20 target therapy with rituximab combined with pulse therapy was then prescribed as an alternative initial induction therapy, followed by low-dose steroid plus hydroxychloroquine. Mycophenolate mofetil as maintenance therapy controlled the progression of GPA and led to remission of the facial and nasal symptoms and the lung mass 3 months after diagnosis of GPA.
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