An International Journal of Otorhinolaryngology Clinics

Register      Login

VOLUME 5 , ISSUE 3 ( Number, 2013 ) > List of Articles

CASE REPORT

Isolated IgG4-related Disease of Sphenoid Sinus Manifesting as Blindness

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

Keywords : IgG4-related disease,Sphenoid sinus,Optic neuritis

Citation Information : Mathew V, Chandrasekharan R, Ashish G, Tyagi AK, Job AK. Isolated IgG4-related Disease of Sphenoid Sinus Manifesting as Blindness. Int J Otorhinolaryngol Clin 2013; 5 (3):178-181.

DOI: 10.5005/jp-journals-10003-1139

Published Online: 01-08-2014

Copyright Statement:  Copyright © 2013; The Author(s).


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.

How to cite this article

Chandrasekharan R, Mathew V, Ashish G, Tyagi AK, Job AK. Isolated IgG4-related Disease of Sphenoid Sinus Manifesting as Blindness. Int J Otorhinolaryngol Clin 2013;5(3):178-181.


PDF Share
  1. Immunoglobulin G4-related sclerosing disease of the paranasal sinus. Head Neck 2013 Oct;35(10):E321–324.
  2. Th2 and regulatory immune reactions are increased in immunoglobin G4-related sclerosing pancreatitis and cholangitis. Hepatol Baltim Md 2007 Jun;45(6):1538–1546.
  3. Consensus statement on the pathology of IgG4-related disease. Modern Pathology 2012;25:1181–1192.
  4. A novel clinical entity, IgG4-related disease (IgG4RD): general concept and details. Mod Rheumatol 2012;22:1–14.
  5. Clinical and pathological differences between serum immunoglobulin G4-positive and -negative type 1 autoimmune pancreatitis. World J Gastroenterol 2013 July;19(25):4031–4038.
  6. Autoimmune pancreatitis. Curr Opin Gastroenterol 2008 Sep;24(5):591–596.
  7. Autoimmune pancreatitis in Japan: overview and perspective. J Gastroenterol 2009;44(6):503–517.
  8. IgG4 immunostaining of duodenal papillary biopsy specimens may be useful for supporting a diagnosis of autoimmune pancreatitis. Gastrointest Endosc 2010 May;71(6):960–966.
  9. Chronic sclerosing dacryoadenitis: part of the spectrum of IgG4-related Sclerosing disease? Am J Surg Pathol 2007 Apr;31(4):643–645.
  10. IgG4-related perineural disease. Int J Rheumatol 2012; 2012:401890.
PDF Share
PDF Share

© Jaypee Brothers Medical Publishers (P) LTD.