Refractory Giant Cell Arteritis Complicated by Vision Loss From Optic Atrophy and Maculopathy Associated With Pachymeningitis

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Title Refractory Giant Cell Arteritis Complicated by Vision Loss From Optic Atrophy and Maculopathy Associated With Pachymeningitis
Creator Jorge A. Uribe, MD; Ishita Aggarwal, MD; Juthamat Witthayaweerasak, MD; Y. Joyce Liao, MD, PhD; Gerald J. Berry, MD; Umesh K. Sab, MD; Cornelia M. Weyand, MD, PhD
Affiliation Departments of Rheumatology (JAU, IA, CMW), Ophthalmology (JW, YJL), and Pathology (GJB), Stanford University School of Medicine, Stanford, California; and Department of Rheumatology (US), Sequoia Hospital, Redwood City, California
Abstract Background: We describe a 75-year-old woman who experienced vision loss in her left eye due to biopsy-proven giant cell arteritis (GCA). She subsequently developed pachymeningitis causing refractory headaches and bilateral optic neuropathy and maculopathy. Methods: Case report with literature review. Results: Eighteen months after the initial diagnosis of GCA, imaging studies in our patient demonstrated pachymeningeal enhancement, and meningeal biopsy confirmed lymphoplasmacytic tissue infiltrates with low frequencies of IgG4+ plasma cells. Laboratory investigation revealed the presence of 3 antiretinal antibodies and antimyeloperoxidase antibodies, consistent with autoimmune retinopathy. Treatment with B-cell-depleting anti-CD20 antibodies suppressed meningeal inflammation and prevented further vision loss. Conclusions: This case illustrates that bilateral vision loss and chronic headaches in patients with GCA may result from retina-directed autoimmunity and pachymeningitis.
Subject Giant Cell Arteritis; Optic Atrophy; Maculopathy; Pachymeningitis
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Date 2018-03
Language eng
Format application/pdf
Type Text
Publication Type Journal Article
Source Journal of Neuro-Ophthalmology, December 2018, Volume 38, Issue 1
Collection Neuro-Ophthalmology Virtual Education Library: Journal of Neuro-Ophthalmology Archives: https://novel.utah.edu/jno/
Publisher Lippincott, Williams & Wilkins
Holding Institution Spencer S. Eccles Health Sciences Library, University of Utah
Rights Management © North American Neuro-Ophthalmology Society
ARK ark:/87278/s6pp3jvk
Setname ehsl_novel_jno
ID 1404071
Reference URL https://collections.lib.utah.edu/ark:/87278/s6pp3jvk
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