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VOLUME 6 , ISSUE 3 ( Number, 2014 ) > List of Articles

CASE REPORT

Isolated IgG4-related Disease of Sphenoid Sinus Manifesting as Blindness

Vivek Mathew, Gaurav Ashish, Amit Kumar Tyagi, Anand Kamalam Job, Ramanathan Chandrasekharan

Citation Information : Mathew V, Ashish G, Tyagi AK, Job AK, Chandrasekharan R. Isolated IgG4-related Disease of Sphenoid Sinus Manifesting as Blindness. Int J Otorhinolaryngol Clin 2014; 6 (3):19-22.

DOI: 10.5005/aijoc-6-3-19

License: CC BY-NC 3.0

Published Online: 01-07-2014

Copyright Statement:  Copyright © 2014; Jaypee Brothers Medical Publishers (P) Ltd.


Abstract

Introduction

IgG4 related disease is a rare entity which can affect almost all parts of the body. The available literature of this entity related to paranasal sinuses is very limited.

Presentation of case

We present a case of isolated sphenoid sinus affliction by IgG4 related sclerosing disease manifesting as blindness, which recovered fully with pulsed steroid therapy.

Discussion

We present this case in view of developing insights toward diagnosis and management of IgG4 related sclerosing disease in paranasal sinuses, specifically the sphenoid sinus.

Conclusion

IgG4 disease involving paranasal sinuses (PNS) is rare entity and is managed with pulse methylprednisolone and immunomodulator drugs. Considering the limit of understanding of etiopathogenesis of this disease, surgeon needs to keep this differential diagnosis in mind. It may present atypically like isolated sinus involvement and optic neuritis.


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