Eosinophilic angiocentric fibrosis of the nose and sinuses.
BACKGROUND
Eosinophilic angiocentric fibrosis is a rare benign disorder of the upper respiratory tract. It is slow growing and progressive, with characteristic histological appearances.


METHODS
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.


RESULTS
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.


CONCLUSIONS
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.
Department of Otolaryngology,Royal National Throat Nose and Ear Hospital,London,UK.
J
J
Rimmer
Department of Otolaryngology,Royal National Throat Nose and Ear Hospital,London,UK.
P
P
Andrews
Department of Otolaryngology,Royal National Throat Nose and Ear Hospital,London,UK.
V J
VJ
Lund
D000328:Adult; D000368:Aged; D004802:Eosinophilia / Q000473:pathology* / Q000601:surgery; D005260:Female; D005355:Fibrosis / Q000473:pathology / Q000601:surgery; D006801:Humans; D008297:Male; D008875:Middle Aged; D009296:Nasal Cavity / Q000473:pathology / Q000601:surgery; D009666:Nose / Q000473:pathology* / Q000601:surgery; D009668:Nose Diseases / Q000473:pathology* / Q000601:surgery; D010256:Paranasal Sinuses / Q000473:pathology* / Q000601:surgery