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2 results

  • Eosinophilic angiocentric fibrosis of the nose and sinuses

  • J Rimmer, P Andrews, V J Lund
  • Journal:
    The Journal of Laryngology & Otology / Volume 128 / Issue 12 / December 2014
    Published online by Cambridge University Press:
    17 November 2014, pp. 1071-1077
    Print publication:
    December 2014
  • 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.

  • Nasal fibrosis: long-term follow up of four cases of eosinophilic angiocentric fibrosis

  • Santdeep Paun, Valerie J Lund, Andrew Gallimore
  • Journal:
    The Journal of Laryngology & Otology / Volume 119 / Issue 2 / February 2005
    Published online by Cambridge University Press:
    16 March 2006, pp. 119-124
    Print publication:
    February 2005

  • Eosinophilic angiocentric fibrosis is a rare, benign cause of submucosal thickening and fibrosis within the upper respiratory tract. It predominantly affects the nose although cases have been reported in the subglottis. We describe four cases of the disease centred around the nasal cavity, with widespread infiltration of the facial soft tissues and orbit in three of the four patients. Each underwent long term follow up. Multiple surgical resections were required with two of our patients and, to date, medical therapy has been of limited help. The disease process, with its clinical and characteristic histopathological findings, is described. We also discuss the management of the disease following a comprehensive review of, and comparison with, the few prior reported cases.