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. 2024 Aug;76(4):3661-3665.
doi: 10.1007/s12070-024-04669-w. Epub 2024 Apr 2.

Sino-Orbital IgG4 Related Disease- An Uncommon Entity

Pookamala Sathasivam  1 , Subramanian Nallasivan  2 , Ramapriyadharshini  3 , Jegan C  4 , Vishnu  5
Affiliations

Sino-Orbital IgG4 Related Disease- An Uncommon Entity

Pookamala Sathasivam et al. Indian J Otolaryngol Head Neck Surg. 2024 Aug.

Abstract

IgG4 related disease (IgG4-RD) is an auto immune fibro-inflammatory condition, characterised by presence of IgG4 positive lymphoplasmacytic infiltrates and extensive fibrosis of the involved organ. It commonly affects pancreas, biliary tract and salivary glands. Sino-orbital involvement is a relatively rare presentation. There is extensive fibrosis of the involved organ. Biopsy is often diagnostic and it shows extensive necrosis and lymphoplasmocytic infiltrates. They show dramatic response to steroid therapy. Here we present three cases of IgG4-RD disease involving orbit and para nasal sinuses who were evaluated and treated in a tertiary care teaching hospital over a period of 2 years.

Keywords: IgG4 related disease; Orbital apex syndrome; Orbital pseudo tumour; Proptosis; Sino-orbital IgG4 disease.

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Conflict of interest statement

Conflict of InterestThe authors declare no conflicts of interest.

Figures

Fig. 1
Fig. 1
HPE of orbital biopsy showing inflammatory cell infiltrates
Fig. 2
Fig. 2
MRI of case no- 2 showing pan orbital involvement (left side) with extension into infra temporal fossa (arrows)
See this image and copyright information in PMC

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