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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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CME Disclosure Statement: Unless noted, all individuals in control of content reported no relevant financial relationships. If applicable, all relevant financial relationships have been mitigated.
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 (email@example.com).
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.
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