IgG4-Related Ophthalmic Disease Presenting as Meningitis and Panuveitis

Purpose. We report an uncommon case of immunoglobulin gamma 4-related ophthalmic disease (IgG4-ROD) presenting as meningitis and panuveitis. Observations. A 35-year-old male with no prior ophthalmic history presented with headaches, altered mental status, and fever of unknown origin. A lumbar punctu...

Full description

Saved in:
Bibliographic Details
Main Authors: Maria A. Mavrommatis, Sarah A. Avila, Richard France
Format: Article
Language:English
Published: Wiley 2019-01-01
Series:Case Reports in Ophthalmological Medicine
Online Access:http://dx.doi.org/10.1155/2019/5653282
Tags: Add Tag
No Tags, Be the first to tag this record!
_version_ 1832559380524957696
author Maria A. Mavrommatis
Sarah A. Avila
Richard France
author_facet Maria A. Mavrommatis
Sarah A. Avila
Richard France
author_sort Maria A. Mavrommatis
collection DOAJ
description Purpose. We report an uncommon case of immunoglobulin gamma 4-related ophthalmic disease (IgG4-ROD) presenting as meningitis and panuveitis. Observations. A 35-year-old male with no prior ophthalmic history presented with headaches, altered mental status, and fever of unknown origin. A lumbar puncture (LP) revealed an elevated white count with lymphocytic predominance, confirming a suspected meningitis. After an extensive work-up, he was discharged on oral acyclovir to cover for presumed aseptic meningitis. The patient initially improved, however, bilateral eye pain, redness, and photophobia 2 weeks after discharge prompted his first visit to the ophthalmology clinic. Exam at that time was consistent with bilateral anterior uveitis for which he was given topical prednisolone and cyclopentolate. In addition to the preceding work-up, quantitative immunoglobulin serology including IgG4 levels was added. At follow-up, he was found to have increased ocular inflammation with vitreitis, nerve head edema, and subclinical macular thickening. Visual acuity (VA) had decreased in both eyes. Serology titers for IgG had resulted in a significant elevation in IgG subclass 4 (IgG4). Optical coherence tomography (OCT) and fundus fluorescein angiography (FFA) confirmed posterior retinal involvement. The patient was diagnosed with presumed bilateral panuveitis secondary to IgG4-ROD. Conclusions and Importance. IgG4-RD can be a serious condition that requires careful consideration and intuition to diagnose. This report serves to encourage ophthalmologists to consider IgG4-ROD in cases of idiopathic systemic inflammation with ophthalmic involvement.
format Article
id doaj-art-dd45acefaa2e43219b5e253d38f24cfb
institution Kabale University
issn 2090-6722
2090-6730
language English
publishDate 2019-01-01
publisher Wiley
record_format Article
series Case Reports in Ophthalmological Medicine
spelling doaj-art-dd45acefaa2e43219b5e253d38f24cfb2025-02-03T01:30:14ZengWileyCase Reports in Ophthalmological Medicine2090-67222090-67302019-01-01201910.1155/2019/56532825653282IgG4-Related Ophthalmic Disease Presenting as Meningitis and PanuveitisMaria A. Mavrommatis0Sarah A. Avila1Richard France2Department of Ophthalmology, Icahn School of Medicine at Mount Sinai, 1 Gustave L. Levy Pl, New York, NY 10029, USADepartment of Ophthalmology, Icahn School of Medicine at Mount Sinai, 1 Gustave L. Levy Pl, New York, NY 10029, USADepartment of Ophthalmology, Icahn School of Medicine at Mount Sinai, 1 Gustave L. Levy Pl, New York, NY 10029, USAPurpose. We report an uncommon case of immunoglobulin gamma 4-related ophthalmic disease (IgG4-ROD) presenting as meningitis and panuveitis. Observations. A 35-year-old male with no prior ophthalmic history presented with headaches, altered mental status, and fever of unknown origin. A lumbar puncture (LP) revealed an elevated white count with lymphocytic predominance, confirming a suspected meningitis. After an extensive work-up, he was discharged on oral acyclovir to cover for presumed aseptic meningitis. The patient initially improved, however, bilateral eye pain, redness, and photophobia 2 weeks after discharge prompted his first visit to the ophthalmology clinic. Exam at that time was consistent with bilateral anterior uveitis for which he was given topical prednisolone and cyclopentolate. In addition to the preceding work-up, quantitative immunoglobulin serology including IgG4 levels was added. At follow-up, he was found to have increased ocular inflammation with vitreitis, nerve head edema, and subclinical macular thickening. Visual acuity (VA) had decreased in both eyes. Serology titers for IgG had resulted in a significant elevation in IgG subclass 4 (IgG4). Optical coherence tomography (OCT) and fundus fluorescein angiography (FFA) confirmed posterior retinal involvement. The patient was diagnosed with presumed bilateral panuveitis secondary to IgG4-ROD. Conclusions and Importance. IgG4-RD can be a serious condition that requires careful consideration and intuition to diagnose. This report serves to encourage ophthalmologists to consider IgG4-ROD in cases of idiopathic systemic inflammation with ophthalmic involvement.http://dx.doi.org/10.1155/2019/5653282
spellingShingle Maria A. Mavrommatis
Sarah A. Avila
Richard France
IgG4-Related Ophthalmic Disease Presenting as Meningitis and Panuveitis
Case Reports in Ophthalmological Medicine
title IgG4-Related Ophthalmic Disease Presenting as Meningitis and Panuveitis
title_full IgG4-Related Ophthalmic Disease Presenting as Meningitis and Panuveitis
title_fullStr IgG4-Related Ophthalmic Disease Presenting as Meningitis and Panuveitis
title_full_unstemmed IgG4-Related Ophthalmic Disease Presenting as Meningitis and Panuveitis
title_short IgG4-Related Ophthalmic Disease Presenting as Meningitis and Panuveitis
title_sort igg4 related ophthalmic disease presenting as meningitis and panuveitis
url http://dx.doi.org/10.1155/2019/5653282
work_keys_str_mv AT mariaamavrommatis igg4relatedophthalmicdiseasepresentingasmeningitisandpanuveitis
AT sarahaavila igg4relatedophthalmicdiseasepresentingasmeningitisandpanuveitis
AT richardfrance igg4relatedophthalmicdiseasepresentingasmeningitisandpanuveitis