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Vasculitis of the upper airways

机译:上呼吸道血管炎

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摘要

Systemic vasculitddes are rare and potentially life-threatening diseases. Granulomatosis with poly-angiitis (GPA; formerly Wegener's granulomatosis) and Churg Strauss syndrome (possibly to be renamed eosinophilic granulomatosis with polyangii-tis; EGPA) are the 2 chief-systemic vasculitides which may involve the upper respiratory tract. Chronic allergic rhinitis and nasal polyposis in EGPA, and recurrent sinusitis and/or otitis in both conditions, are not specific and can thus represent real diagnostic challenges if they are the first manifestations of the disease. Nasal septum perforation, saddle nose deformity and/or subglottic stenosis (SGS), although not totally specific, are more suggestive of GPA. Because upper airway manifestations often tend to be refractory to systemic therapy and/or to linger, local treatment represents a major aspect of management of the condition, especially for patients with SGS.
机译:系统性血管炎是罕见的,可能威胁生命的疾病。伴有多血管炎的肉芽肿病(GPA;以前是Wegener肉芽肿病)和Churg Strauss综合征(可能更名为伴有多血管炎的嗜酸性肉芽肿; EGPA)是可能涉及上呼吸道的2种主要系统性血管炎。 EGPA中的慢性过敏性鼻炎和鼻息肉以及这两种情况下的复发性鼻窦炎和/或中耳炎都不是特异性的,因此如果它们是该病的首发表现,则可能代表着真正的诊断挑战。鼻中隔穿孔,鞍鼻畸形和/或声门下狭窄(SGS)虽然不是完全特异性的,但更提示GPA。由于上呼吸道表现通常倾向于全身治疗和/或难治,因此局部治疗代表了病情管理的一个主要方面,特别是对于SGS患者。

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