We present the case of a 41-year-old Caucasian woman with established accompanying antiphospholipid syndrome (APS) in the course of systemic lupus erythematosus (SLE). The diagnosis of APS / SLE was based on clinical findings (neuropsychiatric: seizures and transient ischemic attack; renal: proteinuria and thrombotic microangiopathy on kidney biopsy) and immunological findings (presence of anti-double-stranded deoxyribonucleic acid antibody, anti-Smith antibody, lupus anticoagulant, as well as anti-beta-2 glycoprotein and anti-cardiolipin antibodies, low complement component 3). These findings were consistent with both the revised Sapporo APS Classification Criteria and the European League Against Rheumatism / American College of Rheumatology Classification Criteria for SLE.1,2 The patient was primarily treated with warfarin, glucocorticoids, and chloroquine.3,4 The treatment with the latter became eventually the reason for the ophthalmological consultation. Ophthalmological examination showed full visual acuity in both eyes (20/20), normal intraocular pressure, good color vision in the right eye, and improper in the left. Anterior segments of both eyes were unremarkable. The results of other ophthalmological tests are shown in Figure 1A–1H. An electrophysiological evaluation was contraindicated due to epilepsy.
Despite retaining full visual acuity, the patient presented with bilateral active severe retinopathy. The severe retinal damage was supposedly a manifestation of accompanying APS and vasculitis associated with SLE.5 Mycophenolate mofetil was initiated, warfarin and chloroquine continued, and the dose of glucocorticoids was gradually tapered down until withdrawal. Partial renal remission was achieved (a decrease in serum creatinine level and proteinuria, normal urine sediment). As the preexisting ischemia of the temporal quadrants was found in the left eye, the patient was scheduled for retinal photocoagulation.
In conclusion, a regular ophthalmological follow-up is warranted in patients with APS / SLE treated with chloroquine or glucocorticoids, as these drugs may produce side effects in the eye. Thromboembolic and / or vasculitic retinal damage, although still underrecognized, should be considered a sight-threatening condition.
Even with full visual acuity, a patient with APS / SLE may suffer from severe visual function loss and require prompt treatment by both rheumatologist and ophthalmologist.
Diana A. Dmuchowska, MD, PhD, Ophthalmology Department, University Teaching Hospital of Białystok, ul. M. Skłodowskiej-Curie 24a, 15-276 Białystok, Poland, phone: +48 85 746 83 72, email: diana.dmuchowska@umb.edu.pl
June 15, 2020.
July 5, 2020.
July 14, 2020.
None declared.
Dmuchowska DA, Grochowski ET, Goździkiewicz-Łapińska J, et al. Full visual acuity may be misleading in accompanying antiphospholipid syndrome: a case of bilateral fovea-sparing retinopathy in a patient with systemic lupus erythematosus. Pol Arch Intern Med. 2020; 130: 887-890. doi:10.20452/pamw.15508
- 1.
- 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: 295-306.Crossref
- 2.
- Aringer M, Costenbader K, Daikh D, et al. 2019 European League Against Rheumatism/American College of Rheumatology Classification Criteria for Systemic Lupus Erythematosus. Arthritis Rheumatol. 2019; 71: 1400-1412.Crossref
- 3.
- Durcan L, O’Dwyer T, Petri M. Management strategies and future directions for systemic lupus erythematosus in adults. Lancet. 2019; 393: 2332-2343.Crossref
- 4.
- Negrini S, Pappalardo F, Murdaca G, et al. The antiphospholipid syndrome: from pathophysiology to treatment. Clin Exp Med. 2017; 17: 257-267.Crossref
- 5.
- Au A, O’Day J. Review of severe vaso-occlusive retinopathy in systemic lupus erythematosus and the antiphospholipid syndrome: associations, visual outcomes, complications and treatment. Clin Exp Ophthalmol. 2004; 32: 87-100.Crossref