Immunoglobulin G4-related sclerosing disease with orbital inflammation in a 12-year-old girl.

Abstract

Immunoglobulin G4-related sclerosing disease is a rare but potentially debilitating cause of orbital inflammation, with a predilection for older males. We report the case of a 12-year-old African girl with immunoglobulin G4-related sclerosing disease, including possible extraorbital involvement. Because of an escalating severity of illness leading to oculomotor nerve palsy and cavernous sinus thrombosis that was resistant to steroids, systemic immunosuppressive therapy with rituximab was used to achieve disease remission. The diagnosis was histologically confirmed with a tissue biopsy.

• Authors: Mona Sane, James Chelnis, Rafal Kozielski, Airaj Fasiuddin
• Journal: Journal of AAPOS : the official publication of the American Association for Pediatric Ophthalmology and Strabismus (J AAPOS) Vol. 17 Issue 5 Pg. 548-50 (Oct 2013) ISSN: 1528-3933 [Electronic] United States
• PMID: 24160980 (Publication Type: Case Reports, Journal Article)
• Copyright: Copyright © 2013 American Association for Pediatric Ophthalmology and Strabismus. Published by Mosby, Inc. All rights reserved.

Chemical References

• Immunoglobulin G

Topics

• Cavernous Sinus Thrombosis (diagnosis)
• Child
• Female
• Humans
• Immunoglobulin G (immunology)
• Oculomotor Nerve Diseases (diagnosis)
• Scleroderma, Systemic (diagnosis, immunology)