COMBINED CENTRAL RETINAL VEIN AND ARTERY OCCLUSION IN SYSTEMIC LUPUS ERYTHEMATOSUS PATIENT Ho Sik Hwang, MD, Seungbum Kang, MD

Purpose: To describe combined central retinal vein occlusion and central retinal artery occlusion in systemic lupus erythematosus patient. Methods: An 18-year-old woman came to our clinic complaining of sudden marked visual loss in her right eye. Best-corrected visual acuity was only hand movement in the right eye. An examination of the right fundus revealed a pale and edematous retina with widespread flame hemorrhages, a swollen optic disk, engorged and tortuous retinal veins, and a cherry-red spot, leading to a diagnosis of combined central retinal vein occlusion and central retinal artery occlusion. Fluorescein angiography displayed disk edema and delayed arteriolar filling, with dilated veins that did not fill with fluorescein. Because of the presence of fever, she was referred to the department of internal medicine where she was diagnosed as having systemic lupus erythematosus and antiphospholipid syndrome. The patient received steroid and anticoagulant treatment. To prevent neovascular glaucoma, intense prophylactic panretinal photocoagulation was initiated 2 weeks later. Results: Despite these efforts, consequent retinal neovascularization and neovascular glaucoma were unavoidable. Filtering surgery was performed 1 month after prophylactic panretinal photocoagulation. Conclusion: Although rare, combined central retinal vein and artery occlusion can occur in systemic lupus erythematosus patient. RETINAL CASES & BRIEF REPORTS 6:187–188, 2012

Fluorescein angiography displayed disk edema and delayed arteriolar filling, with dilated veins that did

From the Department of Ophthalmology, Youngwol Medical Center, Korea; Department of Ophthalmology and Visual Sciences, Daejeon St. Mary’s Hospital, College of Medicine, The Catholic University of Korea, Seoul, Korea.

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n 18-year-old woman came to our clinic complaining of sudden marked visual loss in her right eye. Best-corrected visual acuity was 20/20 in the left eye and only hand movement in the right eye. An examination of the right fundus revealed a pale and edematous retina with widespread flame hemorrhages, a swollen optic disk, engorged and tortuous retinal veins, and a cherry-red spot, leading to a diagnosis of combined central retinal vein occlusion and central retinal artery occlusion (Figure 1). The authors have no financial interest or conflicts of interest. Reprint requests: Seungbum Kang, MD, Department of Ophthalmology and Visual Sciences, Daejeon St. Mary’s Hospital, College of Medicine, The Catholic University of Korea, Daeheungdong 520-2, Joong-gu, Daejeon, Korea; e-mail: john0730@ hanmail.net

Fig. 1. The right fundus photography shows a pale and edematous retina with widespread flame-shaped hemorrhages, a optic disk swelling, engorged and tortuous retinal veins, and a cherry-red spot, leading to a diagnosis of combined central retinal vein and artery occlusion.

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RETINAL CASES & BRIEF REPORTS´  2012  VOLUME 6  NUMBER 2

Fig. 2. Fluorescein angiography at 3 minutes after fluorescein dye injection shows delayed arteriolar filling, with dilated veins that did not fill with fluorescein dye because of combined central retinal vein and artery occlusion.

not fill with fluorescein (Figure 2). The left eye was unremarkable. Because of the presence of fever during examination, she was referred to the department of internal medicine, where she was diagnosed as having systemic lupus erythematosus and antiphospholipid syndrome (APS). She was diagnosed to have systemic lupus erythematosus on the basis of malar rashes, pleuritis, proteinuria, and abnormal titres of antinuclear antibodies, and anti–double-strand DNA antibodies. And she was diagnosed with APS based on positive serum anti–b2-glycoprotein antibody and retinal vessel thrombosis according to Sydney criteria.1 The patient received steroid and anticoagulant treatment. To prevent neovascular glaucoma, intense prophylactic panretinal photocoagulation was initiated 2 weeks later. Despite these efforts, consequent retinal neovascularization and neovascular glaucoma were unavoidable. Filtering surgery was performed 1 month after prophylactic panretinal photocoagulation. Systemic lupus erythematosus is an autoimmune disease in which organs and cells undergo damage mediated by tissue-binding autoantibodies and immune complexes. Retinopathy is the most common

ocular manifestation in systemic lupus erythematosus and usually consists of cotton wool spots with intraretinal hemorrhages. Occlusions of larger vessels including isolated central retinal artery occlusion or central retinal vein occlusion secondary to systemic lupus erythematosus have been reported in the literature2,3 and can be observed clinically. To our knowledge, however, there are few instances of combined central retinal artery occlusion and central retinal vein occlusion in systemic lupus erythematosus patients.3–6 Distinction between thrombotic and inflammatory etiology is very important during the acute phase of disease because treatment options can differ according to the cause of occlusion. The main pathogenesis in our case was thrombotic rather than vasculitic in origin, as determined based on a raised antiphospholipid antibody titer, absence of central nervous system lupus, unilateral eye involvement, and no signs of retinopathy or vasculitis in the left eye. Therefore, it is better to add an anticoagulant along with systemic steroid treatment to accommodate such cases. Key words: central retinal artery occlusion, central retinal vein occlusion, systemic lupus erythematosus. References 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. 2. 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 Experiment Ophthalmol 2004;32:87–100. 3. Fitzpatrick EP, Chesen N, Rahn EK. The lupus anticoagulant and retinal vaso-occlusive disease. Ann Ophthalmol 1990;22: 148–152. 4. Leibovitch I, Goldstein M, Loewenstein A, Barak A. Combined central retinal artery and vein occlusion in a patient with systemic lupus erythematosus. Rheumatology 2001;40: 1195–1196. 5. Durukan AH, Akar Y, Bayraktar MZ, et al. Combined retinal artery and vein occlusion in a patient with systemic lupus erythematosus and antiphospholipid syndrome. Can J Ophthalmol 2005;40:87–89. 6. Mendrinos E, Mavrakanas N, Kiel R, Pournaras CJ. Bilateral combined central retinal artery and vein occlusion in systemic lupus erythematosus resulting in complete blindness. Eye 2009; 23:1231–1232.

Combined central retinal vein and artery occlusion in systemic lupus erythematosus patient.

To describe combined central retinal vein occlusion and central retinal artery occlusion in systemic lupus erythematosus patient...
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