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A patient in their late 70s with a history of Crohn disease, coronary artery disease, hypercholesterolemia, and transient ischemic attack presented with sudden, painless, left-sided vision loss. They denied headache, scalp tenderness, and jaw claudication. The erythrocyte sedimentation rate (ESR) was 103 mm/h and a magnetic resonance angiographic examination of the brain was negative for mass, infarction, and hemorrhage. Examination results of a right, superficial, temporal artery biopsy specimen showed chronic, nongranulomatous inflammation surrounding adventitial vessels and extensive subintimal fibrosis, without giant cells or segmental loss of the internal elastic lamina, which was interpreted as compatible with temporal arteritis. High-dose corticosteroid therapy was initiated (1 g methylprednisolone intravenously for 3 days followed by 60 mg/d of oral prednisone).
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Systemic amyloidosis in the setting of lymphoplasmacytic lymphoma (Waldenström macroglobulinemia)
C. Order serum and urine protein electrophoresis
An older patient presenting with visual loss and pallid disc edema arouses suspicion for an anterior ischemic optic neuropathy. As the most common vasculitis of older adults in North America, giant cell arteritis (GCA) has become the poster child of the arteritic form of anterior ischemic optic neuropathy. Well established as visually morbid—with an approximate 8% rate of permanent vision loss and a 50% rate of fellow eye involvement—and responsive to corticosteroid therapy, GCA has a low threshold for diagnosis and treatment.1 However, the prevailing criteria for diagnosing GCA (the 1990 American College of Rheumatology classification) is relatively nonspecific, having a specificity of 91.3%.2 Other vasculitides, including eosinophilic granulomatosis with polyangiitis, granulomatosis with polyangiitis, hypersensitivity vasculitis, Henoch-Schönlein purpura, polyarteritis nodosa, and Takayasu arteritis, are all cited as misclassifications for GCA in the original article by Hunder et al.2
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Corresponding Author: Ralph C. Eagle Jr, MD, Department of Neurology and Ophthalmology, Wills Eye Hospital, Sidney Kimmel Medical College of Thomas Jefferson University, 840 Walnut St, Ste 1410, Philadelphia, PA 19107 (firstname.lastname@example.org).
Published Online: March 18, 2021. doi:10.1001/jamaophthalmol.2020.4638
Conflict of Interest Disclosures: None reported.
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