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Eosinophilic angiocentric fibrosis of the nose and sinuses

Published online by Cambridge University Press:  17 November 2014

J Rimmer ,
P Andrews  and
V J Lund
J Rimmer*
Affiliation:
Department of Otolaryngology, Royal National Throat Nose and Ear Hospital, London, UK
P Andrews
Affiliation:
Department of Otolaryngology, Royal National Throat Nose and Ear Hospital, London, UK
V J Lund
Affiliation:
Department of Otolaryngology, Royal National Throat Nose and Ear Hospital, London, UK Ear Institute, University College London, UK
*
Address for correspondence: Ms J Rimmer, Royal National Throat Nose and Ear Hospital, 330 Gray's Inn Road, London WC1X 8DA, UK E-mail: [email protected]
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Abstract

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.

Conclusion:

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.

Keywords

Eosinophilic Angiocentric FibrosisIgG4-related DiseaseEosinophilsFibrosisNoseParanasal Sinuses
Type
Main Articles
Information
The Journal of Laryngology & Otology , Volume 128 , Issue 12 , December 2014 , pp. 1071 - 1077
Copyright
Copyright © JLO (1984) Limited 2014 

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