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IgG4-related orbitopathy as an important differential diagnosis of advanced silent sinus syndrome

IgG4-assoziierte Orbitopathie als wichtige Differenzialdiagnose des fortgeschrittenen Silent-Sinus-Syndroms

Abstract

Background

Immunoglobulin (Ig)G4-related disease is classified as an immune-mediated disease. The etiology of this condition has not been explained to date. Manifestations of the disease are diverse, and simultaneous involvement of multiple organs is not unusual.

Case report

We report the case of a patient referred to us after multiple unsuccessful paranasal sinus operations who presented with enophthalmos and a resultant migratory keratitis with a suspected diagnosis of silent sinus syndrome. Preservation of the orbit was no longer feasible. After five years without a definitive diagnosis, we ascertained that this was a case of IgG4-related disease.

Discussion

IgG4-related disease represents an important element in the differential diagnosis of chronic advanced diseases of the orbit and paranasal sinuses. The diagnosis should be considered in the case of unclear disease presentations. Typical histological findings include a storiform pattern of fibrosis, vasculopathy, and tissue infiltration by IgG4 plasma cells.

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Author information

Correspondence to Dr. med. univ. M. Jurkov.

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Conflict of interest

M. Jurkov, H. Olze, F. Klauschen, E. Bertelmann, U. Schneider and P. Arens declare that they have no competing interests.

For this article no studies with human participants or animals were performed by any of the authors. All studies performed were in accordance with the ethical standards indicated in each case. Additional written informed consent was obtained from all individual participants or their legal representatives for whom identifying information is included in this article.

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Jurkov, M., Olze, H., Klauschen, F. et al. IgG4-related orbitopathy as an important differential diagnosis of advanced silent sinus syndrome. HNO (2020) doi:10.1007/s00106-019-00799-8

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Keywords

  • IgG4-related disease
  • Immunoglobulin G
  • Orbit
  • Eosinophilic angiocentric fibrosis
  • Chronic sinusitis