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A 42-year-old woman was referred for ophthalmologic evaluation after developing blurry vision in the right eye 30 days prior. She had a history of hyperlipidemia, hypertension, and migraine headaches. Her medications included atorvastatin, carvedilol, and norethindrone. Dilated-fundus examination of the right eye showed a single cotton-wool spot (indicating local retinal ischemia), with intraretinal hemorrhage in the inferotemporal quadrant. Ultrawide-field imaging showed a cotton-wool spot on the left fundus (Figure 1A, right) and a peripheral arterial occlusion in the right eye (Figure 1A, left), and fluorescein angiographic study of the right eye demonstrated an area of blockage corresponding to intraretinal hemorrhage and venous hyperfluorescence (Figure 1B). The patient was afebrile, with pulse 110 beats/min and blood pressure of 188/99 mm Hg. Auscultation over the heart apex revealed a holosystolic ejection murmur (grade 2/6). Laboratory testing showed normal complete blood cell count and thyrotropin level and negative results for autoantibodies against Sm, SSA, SSB, Scl-70, Jo-1, centromere, chromatin, ribonucleoprotein, and antineutrophil cytoplasmic antibodies. Erythrocyte sedimentation rate was mildly elevated (30 mm/h). Results of testing for antinuclear antibodies were positive at low titer (1:40, homogenous pattern) with positive dsDNA (5.0 IU/mL; negative if <4.0 IU/mL). Blood cultures were negative for bacterial or fungal growth after 5 days. An echocardiogram revealed pedunculated and mobile aortic valve vegetations.
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Nonbacterial thrombotic endocarditis (NBTE) associated with primary antiphospholipid syndrome (APS)
D. Check antiphospholipid antibody levels
The key to the correct diagnosis was the presence of systemic features of a prothrombotic state and endothelial injury involving more than 1 anatomical site (ie, retinal artery thrombosis and aortic valve findings). Although a cotton-wool spot with surrounding hemorrhage (Roth spot) has been classically associated with bacterial endocarditis, it is a nonspecific finding and has also been associated with APS, autoimmune disease, hematologic malignancy, diabetic retinopathy, preeclampsia, and shaken baby syndrome. Intravenous antibiotics for infectious endocarditis (choice A) would not be appropriate in the absence of fevers, markedly elevated inflammatory marker levels, or abnormal blood cultures. While a prothrombotic state may be associated with autoimmune disease, empirical treatment with systemic corticosteroids (choice B) or anticoagulation (choice C) should not be initiated without additional workup, including antiphospholipid antibody testing (choice D).
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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: Miroslav Sekulic, MD, MA, Department of Pathology and Cell Biology, Columbia University Irving Medical Center, 630 W 168th St, VC14-238C, New York, NY 10032 (email@example.com).
Conflict of Interest Disclosures: None reported.
Additional Contributions: We thank the patient for providing permission to share her information.
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