眶部嗜酸性血管中心纤维化1例并文献复习  

Orbital eosinophilic angiocentric fibrosis:a case report with literature review

作　　者：梁城晔 徐玥彤 王天琪[1] 孙炎 乔鹏岗 刘燕鹰 Liang Chengye;Xu Yuetong;Wang Tianqi;Sun Yan;Qiao Penggang;Liu Yanying(Department of Rheumatology,Beijing Friendship Hospital,Capital Medical University,Beijing 100050,China;Department of Otolaryngology,Head and Neck Surgery,Beijing Tongren Hospital,Capital Medical University,Beijing 100005,China;Department of Radiology,Beijing Friendship Hospital,Capital Medical University,Beijing 100050,China)

机构地区：[1]首都医科大学附属北京友谊医院风湿内科,北京100050 [2]首都医科大学附属北京友谊医院耳鼻咽喉头颈外科,北京100050 [3]首都医科大学附属北京友谊医院放射科,北京100050

出　　处：《中华风湿病学杂志》2025年第3期219-224,I0002,共7页Chinese Journal of Rheumatology

摘　　要：目的分析总结眶部嗜酸性血管中心纤维化(EAF)的临床特点和治疗。方法分析1例眶部EAF患者并回顾性分析已经发表的眶部EAF病例报道。结果共纳入34例眶部EAF,其中15例单独累及眼部,19例合并其他部位受累(其中鼻部受累有17例)。首发症状以眼部肿胀性改变(18例)、溢泪(4例)等为突出表现。EAF病理表现以血管周围大量嗜酸性粒细胞浸润和血管中心性洋葱皮状纤维化为典型特征,本组病例均符合上述典型病理特征。20例患者行组织IgG4免疫组化染色,其中16例IgG4免疫组织化学染色阳性,4例阴性。未见闭塞性静脉炎、席纹状纤维化的表现。对IgG4染色阳性组与阴性组的发病年龄、性别、病变部位(单发或多发)等进行分析。2组的发病年龄、性别、病变部位差异均无统计学意义(P>0.05)。结论当出现眼部肿胀、溢泪等症状,伴或不伴鼻部病变,应考虑到EAF,可通过组织病理学确定诊断。部分EAF病例组织IgG4染色阳性,但未见席纹状纤维化、闭塞性静脉炎等表现,可与IgG4相关性疾病鉴别。ObjectiveTo analyze the clinical characteristics and treatment of eosinophilic angiocentric fibrosis(EAF)involving the orbit.MethodsWe described a case and review the literature of EAF involving the orbit.ResultsThe literature review has shown 34 similar cases.Nineteen patients combined with other site involvement(17 cases had nasal involvement),whereas 15 had primary orbital involvement.Ocular swelling(18 cases)and epiphora(4 cases)were the most common initial presenting symptoms.The typical histopathologic findings include a perivascular,eosinophil-rich infiltrate and a"onion-skin"type of fibrosis concentrated around small vessels and all cases in this group conformed the above typical characteristics.In this series,20 patients provided immunohistochemical results for IgG4,among them,16 cases were positive while 4 cases were negative.No manifestations of obliterative phlebitis and storiform fibrosis were observed.The age,gender,and lesion locations(single or multiple)of the IgG4 staining positive group and the negative group were analyzed.There was no statistically significant difference in the age of onset,gender ratio and lesion the two groups(P>0.05).ConclusionFor patients presented with ocular swelling,epiphora,with or without nasal lesions,EAF should be considered.The diagnosis of EAF is based largely on histopathologic findings.Although some cases were positive for IgG4 by immunohistochemistry,storiform fibrosis and obliterative phlebitis is not seen in our series,which aid in distinguishing EAF from IgG4-related disease.

关 键 词：眶疾病 IGG4相关性疾病 嗜酸性血管中心纤维化 首发症状 

分 类 号：R73[医药卫生—肿瘤]