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

Update Item Information
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
OCR Text Show
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
Publisher Lippincott, Williams & Wilkins
Holding Institution Spencer S. Eccles Health Sciences Library, University of Utah, 10 N 1900 E SLC, UT 84112-5890
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
Back to Search Results