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Sequential Bilateral Vision Loss in a Woman With Scalp Tenderness and Jaw Claudication

Educational Objective
Based on this clinical scenario and the accompanying image, understand how to arrive at a correct diagnosis.
1 Credit CME

A 67-year-old woman with fibromyalgia presented with sequential bilateral vision loss. Vision decreased suddenly in the right eye 3 to 4 weeks prior and in the left eye 5 days prior. She reported left-sided scalp tenderness; jaw claudication; neck, shoulder, and low back pain; and a recent 6.8-kilogram weight loss. Visual acuity was 20/200 in the right eye (OD) and hand motion in the left eye (OS). She had a left relative afferent pupillary defect, moderate dry-eye syndrome, and mild cataracts. The right optic nerve had a cup-disc ratio of 0.55 with temporal pallor and mild optic disc edema, and the left nerve had a cup-disc ratio of 0.50 with mild edema without pallor.

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C. Neuromyelitis optica spectrum disorder associated with Sjögren syndrome

The initial clinical suspicion for GCA was high given the patient’s classic presentation—a woman older than 65 years with sequential bilateral vision loss accompanied by scalp tenderness, jaw claudication, weight loss, and musculoskeletal pain suggestive of polymyalgia rheumatica. Expedited evaluation and treatment are critical in suspected GCA. Initial laboratory workup should include ESR, CRP, and platelet count tests. Chan et al1 found that these 3 tests have similar sensitivity (range, 65%-71%) and specificity (range, 57%-62%) for GCA. The gold standard of GCA diagnosis is TAB, but the sensitivity is only 77%.2 Empirical treatment should not be delayed for TAB because the positivity rate is similar after up to 4 weeks of systemic corticosteroid therapy.3 In this clinically suspicious patient with ESR elevation but normal CRP level and platelet count and negative TAB results, GCA remained a plausible diagnosis to justify continued corticosteroid therapy.

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Article Information

Corresponding Author: Brooke T. Johnson, DO, Department of Ophthalmology and Visual Sciences, University of Illinois at Chicago, 1855 W Taylor St, Ste 3.158, Chicago, IL 60612 (bjohn30@uic.edu).

Published Online: May 28, 2021. doi:10.1001/jamaneurol.2021.0757

Conflict of Interest Disclosures: Dr Johnson reported receiving grants from the National Eye Institute outside the submitted work. No other disclosures were reported.

Additional Contributions: We thank the patient for granting permission to publish this information.

References
1.
Chan  FLY , Lester  S , Whittle  SL , Hill  CL .  The utility of ESR, CRP and platelets in the diagnosis of GCA.   BMC Rheumatol. 2019;3:14. doi:10.1186/s41927-019-0061-z PubMedGoogle ScholarCrossref
2.
Rubenstein  E , Maldini  C , Gonzalez-Chiappe  S , Chevret  S , Mahr  A .  Sensitivity of temporal artery biopsy in the diagnosis of giant cell arteritis.   Rheumatology (Oxford). 2020;59(5):1011-1020. doi:10.1093/rheumatology/kez385 PubMedGoogle ScholarCrossref
3.
Narváez  J , Bernad  B , Roig-Vilaseca  D ,  et al.  Influence of previous corticosteroid therapy on temporal artery biopsy yield in giant cell arteritis.   Semin Arthritis Rheum. 2007;37(1):13-19. doi:10.1016/j.semarthrit.2006.12.005 PubMedGoogle ScholarCrossref
4.
Liu  GT , Glaser  JS , Schatz  NJ , Smith  JL .  Visual morbidity in giant cell arteritis.   Ophthalmology. 1994;101(11):1779-1785. doi:10.1016/S0161-6420(94)31102-X PubMedGoogle ScholarCrossref
5.
Madani  G , Beale  T .  Inflammatory conditions of the salivary glands.   Semin Ultrasound CT MR. 2006;27(6):440-451. doi:10.1053/j.sult.2006.09.005 PubMedGoogle ScholarCrossref
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Ruiz-Gaviria  R , Baracaldo  I , Castañeda  C , Ruiz-Patiño  A , Acosta-Hernandez  A , Rosselli  D .  Specificity and sensitivity of aquaporin 4 antibody detection tests in patients with neuromyelitis optica.   Mult Scler Relat Disord. 2015;4(4):345-349. doi:10.1016/j.msard.2015.06.003 PubMedGoogle ScholarCrossref
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Miller  NR , Arnold  AC .  Current concepts in the diagnosis, pathogenesis and management of nonarteritic anterior ischaemic optic neuropathy.   Eye (Lond). 2015;29(1):65-79. doi:10.1038/eye.2014.144PubMedGoogle ScholarCrossref
8.
Shahmohammadi  S , Doosti  R , Shahmohammadi  A ,  et al.  Autoimmune diseases associated with neuromyelitis optica spectrum disorders.   Mult Scler Relat Disord. 2019;27:350-363. doi:10.1016/j.msard.2018.11.008 PubMedGoogle ScholarCrossref
9.
Weber  MS , Derfuss  T , Metz  I , Brück  W .  Defining distinct features of anti-MOG antibody associated central nervous system demyelination.   Ther Adv Neurol Disord. 2018;11:1756286418762083. doi:10.1177/1756286418762083PubMedGoogle Scholar
10.
Wu  Y , Zhong  L , Geng  J .  Neuromyelitis optica spectrum disorder.   Mult Scler Relat Disord. 2019;27:412-418. doi:10.1016/j.msard.2018.12.002 PubMedGoogle ScholarCrossref
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