Rimmer J, Andrews P, Lund V J
Department of Otolaryngology,Royal National Throat Nose and Ear Hospital,London,UK.
J Laryngol Otol. 2014 Dec;128(12):1071-7. doi: 10.1017/S0022215114002552. Epub 2014 Nov 17.
Eosinophilic angiocentric fibrosis is a rare benign disorder of the upper respiratory tract. It is slow growing and progressive, with characteristic histological appearances.
We report the largest single-institution case series of sinonasal eosinophilic angiocentric fibrosis to date, comprising nine patients. The current literature is reviewed, showing emerging evidence that this condition may belong to the immunoglobulin G4-related disease spectrum.
The series comprised five female and four male patients, with a mean age at presentation of 53 years. All were treated surgically. Six patients had no signs of recurrent disease after an average of 8.5 years. One patient went on to develop granulomatosis with polyangiitis (Wegener's granulomatosis), which required immunosuppressive therapy.
The first-line management of this rare condition is complete surgical excision. Chronic granulomatous conditions, including granulomatosis with polyangiitis, should be excluded before a diagnosis is made, and patients should be carefully followed.
嗜酸性粒细胞性血管中心性纤维化是一种罕见的上呼吸道良性疾病。它生长缓慢且呈进行性发展，具有特征性的组织学表现。
我们报告了迄今为止最大的单机构鼻窦嗜酸性粒细胞性血管中心性纤维化病例系列，共9例患者。对当前文献进行了综述，显示有新证据表明该疾病可能属于免疫球蛋白G4相关疾病谱。
该系列包括5名女性和4名男性患者，就诊时的平均年龄为53岁。所有患者均接受了手术治疗。平均8.5年后，6例患者无复发迹象。1例患者后来发展为肉芽肿性多血管炎（韦格纳肉芽肿），需要免疫抑制治疗。
这种罕见疾病的一线治疗方法是完整的手术切除。在做出诊断之前，应排除包括肉芽肿性多血管炎在内的慢性肉芽肿性疾病，并且应对患者进行仔细随访。
