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Retinal Artery Thrombosis and Aortic Valve Vegetations

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

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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Article Information

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 (ms6105@cumc.columbia.edu).

Conflict of Interest Disclosures: None reported.

Additional Contributions: We thank the patient for providing permission to share her information.

Miyakis  S , Lockshin  MD , Atsumi  T ,  et al.  International consensus statement on an update of the classification criteria for definite antiphospholipid syndrome (APS).   J Thromb Haemost. 2006;4(2):295-306. doi:10.1111/j.1538-7836.2006.01753.xPubMedGoogle ScholarCrossref
Corban  MT , Duarte-Garcia  A , McBane  RD , Matteson  EL , Lerman  LO , Lerman  A .  Antiphospholipid syndrome: role of vascular endothelial cells and implications for risk stratification and targeted therapeutics.   J Am Coll Cardiol. 2017;69(18):2317-2330. doi:10.1016/j.jacc.2017.02.058PubMedGoogle ScholarCrossref
Eiken  PW , Edwards  WD , Tazelaar  HD , McBane  RD , Zehr  KJ .  Surgical pathology of nonbacterial thrombotic endocarditis in 30 patients, 1985-2000.   Mayo Clin Proc. 2001;76(12):1204-1212. doi:10.4065/76.12.1204PubMedGoogle ScholarCrossref
Izzedine  H , Perazella  MA .  Thrombotic microangiopathy, cancer, and cancer drugs.   Am J Kidney Dis. 2015;66(5):857-868. doi:10.1053/j.ajkd.2015.02.340PubMedGoogle ScholarCrossref
Petri  M , Orbai  AM , Alarcón  GS ,  et al.  Derivation and validation of the Systemic Lupus International Collaborating Clinics classification criteria for systemic lupus erythematosus.   Arthritis Rheum. 2012;64(8):2677-2686. doi:10.1002/art.34473PubMedGoogle ScholarCrossref
Kolitz  T , Shiber  S , Sharabi  I , Winder  A , Zandman-Goddard  G .  Cardiac manifestations of antiphospholipid syndrome with focus on its primary form.   Front Immunol. 2019;10:941. doi:10.3389/fimmu.2019.00941PubMedGoogle ScholarCrossref
Demirci  FY , Küçükkaya  R , Akarçay  K ,  et al.  Ocular involvement in primary antiphospholipid syndrome.   Int Ophthalmol. 1998;22(6):323-329. doi:10.1023/A:1006305705080PubMedGoogle ScholarCrossref
Castañón  C , Amigo  MC , Bañales  JL , Nava  A , Reyes  PA .  Ocular vaso-occlusive disease in primary antiphospholipid syndrome.   Ophthalmology. 1995;102(2):256-262. doi:10.1016/S0161-6420(95)31028-7PubMedGoogle ScholarCrossref
Zmaili  MA , Alzubi  JM , Kocyigit  D ,  et al.  A contemporary 20-year Cleveland Clinic experience of nonbacterial thrombotic endocarditis: etiology, echocardiographic imaging, management, and outcomes.   Am J Med. 2021;134(3):361-369. doi:10.1016/j.amjmed.2020.06.047PubMedGoogle ScholarCrossref
AMA CME Accreditation Information

Credit Designation Statement: The American Medical Association designates this Journal-based CME activity activity for a maximum of 1.00  AMA PRA Category 1 Credit(s)™. Physicians should claim only the credit commensurate with the extent of their participation in the activity.

Successful completion of this CME activity, which includes participation in the evaluation component, enables the participant to earn up to:

  • 1.00 Medical Knowledge MOC points in the American Board of Internal Medicine's (ABIM) Maintenance of Certification (MOC) program;;
  • 1.00 Self-Assessment points in the American Board of Otolaryngology – Head and Neck Surgery’s (ABOHNS) Continuing Certification program;
  • 1.00 MOC points in the American Board of Pediatrics’ (ABP) Maintenance of Certification (MOC) program;
  • 1.00 Lifelong Learning points in the American Board of Pathology’s (ABPath) Continuing Certification program; and
  • 1.00 credit toward the CME [and Self-Assessment requirements] of the American Board of Surgery’s Continuous Certification program

It is the CME activity provider's responsibility to submit participant completion information to ACCME for the purpose of granting MOC credit.

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