Cilioretinal Artery Occlusion in Antiphospholipid Syndrome and the Decision to Anticoagulate

Overview

Journal BMJ Case Rep

Specialty General Medicine

Date 2022 Nov 17

PMID 36384882

Authors

Arash Delavar

Sally L Baxter

Affiliations

Soon will be listed here.

Abstract

A patient in her late 50s with antiphospholipid syndrome presented to general ophthalmology clinic for annual hydroxychloroquine retinopathy screening. She had taken 400 mg hydroxychloroquine daily for over a decade. She denied any visual changes and visual acuity was 20/20. Her examination and fundus photos were normal, but macular optical coherence tomography of the right eye demonstrated inner retinal atrophy and visual field tests revealed a corresponding paracentral scotoma, consistent with a prior cilioretinal artery occlusion. Prior testing from visits with other ophthalmologists revealed that this occlusion had occurred previously, but she had only been informed of not having hydroxychloroquine retinopathy. The possibility of vision loss prompted her to reconsider her prior decision to discontinue anticoagulation. This case demonstrates how anchoring bias may lead clinicians astray, and how the risk of blindness is a strong motivator for patients regarding anticoagulation.

References

Cohen D, Berger S, Steup-Beekman G, Bloemenkamp K, Bajema I . Diagnosis and management of the antiphospholipid syndrome. BMJ. 2010; 340:c2541. DOI: 10.1136/bmj.c2541. View

Arachchillage D, Efthymiou M, Mackie I, Lawrie A, Machin S, Cohen H . Rivaroxaban and warfarin achieve effective anticoagulation, as assessed by inhibition of TG and in-vivo markers of coagulation activation, in patients with venous thromboembolism. Thromb Res. 2015; 135(2):388-93. DOI: 10.1016/j.thromres.2014.11.037. View

Utz V, Tang J . Ocular manifestations of the antiphospholipid syndrome. Br J Ophthalmol. 2010; 95(4):454-9. DOI: 10.1136/bjo.2010.182857. View

Zuo Y, Barbhaiya M, Erkan D . Primary Thrombosis Prophylaxis in Persistently Antiphospholipid Antibody-Positive Individuals: Where Do We Stand in 2018?. Curr Rheumatol Rep. 2018; 20(11):66. DOI: 10.1007/s11926-018-0775-8. View

Espinosa G, Cervera R . Antiphospholipid syndrome: frequency, main causes and risk factors of mortality. Nat Rev Rheumatol. 2010; 6(5):296-300. DOI: 10.1038/nrrheum.2010.47. View

Scott A, Bressler N, Ffolkes S, Wittenborn J, Jorkasky J . Public Attitudes About Eye and Vision Health. JAMA Ophthalmol. 2016; 134(10):1111-1118. DOI: 10.1001/jamaophthalmol.2016.2627. View

Dufrost V, Risse J, Zuily S, Wahl D . Direct Oral Anticoagulants Use in Antiphospholipid Syndrome: Are These Drugs an Effective and Safe Alternative to Warfarin? A Systematic Review of the Literature. Curr Rheumatol Rep. 2016; 18(12):74. DOI: 10.1007/s11926-016-0623-7. View

Lewis A, Mireskandari K . Hemiretinal arterial supply by the cilioretinal artery. Eye (Lond). 2004; 19(11):1239-40. DOI: 10.1038/sj.eye.6701750. View

Yusuf I, Sharma S, Luqmani R, Downes S . Hydroxychloroquine retinopathy. Eye (Lond). 2017; 31(6):828-845. PMC: 5518824. DOI: 10.1038/eye.2016.298. View

10.

Hussain A, Oestreicher J . Clinical decision-making: heuristics and cognitive biases for the ophthalmologist. Surv Ophthalmol. 2017; 63(1):119-124. DOI: 10.1016/j.survophthal.2017.08.007. View

11.

Gumbinger C, Holstein T, Stock C, Rizos T, Horstmann S, Veltkamp R . Reasons underlying non-adherence to and discontinuation of anticoagulation in secondary stroke prevention among patients with atrial fibrillation. Eur Neurol. 2015; 73(3-4):184-91. DOI: 10.1159/000371574. View

12.

Martinelli I, Abbattista M, Bucciarelli P, Tripodi A, Artoni A, Gianniello F . Recurrent thrombosis in patients with antiphospholipid antibodies treated with vitamin K antagonists or rivaroxaban. Haematologica. 2018; 103(7):e315-e317. PMC: 6029544. DOI: 10.3324/haematol.2017.185132. View

13.

Pengo V, Denas G, Zoppellaro G, Padayattil Jose S, Hoxha A, Ruffatti A . Rivaroxaban vs warfarin in high-risk patients with antiphospholipid syndrome. Blood. 2018; 132(13):1365-1371. DOI: 10.1182/blood-2018-04-848333. View

14.

Abdou J, Auyeung V, Patel J, Arya R . Adherence to long-term anticoagulation treatment, what is known and what the future might hold. Br J Haematol. 2016; 174(1):30-42. DOI: 10.1111/bjh.14134. View

15.

Garcia D, Erkan D . Diagnosis and Management of the Antiphospholipid Syndrome. N Engl J Med. 2018; 378(21):2010-2021. DOI: 10.1056/NEJMra1705454. View

16.

Salmasi S, Loewen P, Tandun R, Andrade J, De Vera M . Adherence to oral anticoagulants among patients with atrial fibrillation: a systematic review and meta-analysis of observational studies. BMJ Open. 2020; 10(4):e034778. PMC: 7245382. DOI: 10.1136/bmjopen-2019-034778. View

17.

Froom P, Saffuri-Elias E, Rozenberg O, Barak M . Triple positive antiphospholipid antibody profile in outpatients with tests for lupus anticoagulants. Clin Chem Lab Med. 2014; 53(1):53-6. DOI: 10.1515/cclm-2014-0571. View

18.

Marmor M, Kellner U, Lai T, Melles R, Mieler W . Recommendations on Screening for Chloroquine and Hydroxychloroquine Retinopathy (2016 Revision). Ophthalmology. 2016; 123(6):1386-94. DOI: 10.1016/j.ophtha.2016.01.058. View

19.

Dufrost V, Risse J, Reshetnyak T, Satybaldyeva M, Du Y, Yan X . Increased risk of thrombosis in antiphospholipid syndrome patients treated with direct oral anticoagulants. Results from an international patient-level data meta-analysis. Autoimmun Rev. 2018; 17(10):1011-1021. DOI: 10.1016/j.autrev.2018.04.009. View

20.

Dori D, Gelfand Y, Brenner B, Miller B . Cilioretinal artery occlusion: an ocular complication of primary antiphospholipid syndrome. Retina. 1997; 17(6):555-7. DOI: 10.1097/00006982-199706000-00014. View