Orbital eosinophilic angiocentric fibrosis: a case report with literature review
10.3760/cma.j.cn141217-20240529-00173
- VernacularTitle:眶部嗜酸性血管中心纤维化1例并文献复习
- Author:
Chengye LIANG
1
;
Yuetong XU
;
Tianqi WANG
;
Yan SUN
;
Penggang QIAO
;
Yanying LIU
Author Information
1. 首都医科大学附属北京友谊医院风湿内科，北京 100050
- Publication Type:Journal Article
- Keywords:
Orbital diseases;
Immunoglobulin G4-related disease;
Eosinophilic angiocentric fibrosis;
Initial manifestation
- From:
Chinese Journal of Rheumatology
2025;29(3):219-224
- CountryChina
- Language:Chinese
-
Abstract:
Objective:To analyze the clinical characteristics and treatment of eosinophilic angiocentric fibrosis (EAF) involving the orbit.Methods:We described a case and review the literature of EAF involving the orbit.Results:The 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). Conclusion:For 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.
