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 Table of Contents  
Year : 2019  |  Volume : 16  |  Issue : 2  |  Page : 113-115

Importance of atherothrombosis or thrombophilia in acute retinal vein occlusion

1 Department of Cardiology, Antalya Private Life Hospital, Antalya, Turkey
2 Department of Diet and Nutrition and Medical Ethics, Afyon Kocatepe University, Afyon, Turkey

Date of Submission19-Aug-2018
Date of Decision28-Dec-2018
Date of Acceptance08-Feb-2019
Date of Web Publication11-Nov-2019

Correspondence Address:
Dr. Yalcin Boduroglu
Department of Cardiology, Ahi Evran University Education and Research Hospital, Kervansaray Mah, 2019. Sok D: 1, 40200 Kirsehir
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/njc.njc_22_18

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Retinal vein occlussion (RVO) is one of the retinal vascular disease which may occur due to atherosclerosis in elderly, but young patients may have connective tissue diseases (such as systemic lupus erythematosus [SLE]) or thrombophilia. Antiphospholipid antibodies (lupus anticoagulant [LA], anticardiolipin [anti-CL]) are well-known risk factors for thrombotic complications in SLE. We present a case of SLE with RVO.
A 57-year-old woman was admitted with decreasing of visual acuity (VA) in the right eye. She did not have diabetes mellitus or hypertension. Malar rash was found in the face. The ophthalmoscopy and fluorescein angiography showed retinal hemorrhages. LA antibody and antinuclear antibodies were positive, and level of anti-CL was normal with high level of homocysteine. Because of detecting widespread capillary nonperfusion in her retina, VA did not change during follow-up after treatment. RVO in SLE patients has been described to be caused by thrombophilic conditions.

Keywords: Antiphospholipid antibodies, retinal vein occlusion, systemic lupus erythematosus

How to cite this article:
Boduroglu Y, Son N. Importance of atherothrombosis or thrombophilia in acute retinal vein occlusion. Nig J Cardiol 2019;16:113-5

How to cite this URL:
Boduroglu Y, Son N. Importance of atherothrombosis or thrombophilia in acute retinal vein occlusion. Nig J Cardiol [serial online] 2019 [cited 2023 May 29];16:113-5. Available from: https://www.nigjcardiol.org/text.asp?2019/16/2/113/270685

  Introduction Top

Systemic lupus erythematosus (SLE) is a chronic, autoimmune disease of the connective tissue. Ocular manifestations are observed in up to one-third of patients with SLE.[1] The most common ocular manifestation is dry eye syndrome.[2] Other common ocular signs include retinal involvement (central retinal artery occlusion [CRAO], central retinal vein occlusion [CRVO], and severe vaso-occlusive retinopathy) and optic nerve involvement.[3] RVO is a common retinal vascular disease, and CRVO and branch RVO (BRVO) are the most common subtypes. The causes of RVO are quite different in elderly and young patients. Elderly patients may have atherosclerotic risk factors, but young patients may have connective tissue diseases (such as [SLE]) or thrombophilia.[4] Antiphospholipid antibodies (APAs) containing lupus anticoagulant (LA), anticardiolipin (anti-CL), and anti-β2 glycoprotein-1 antibodies are recognized as risk factors for thrombotic complications, which is called antiphospholipid syndrome (APS). Several studies have also reported a potential association between the APAs and risk of RVO, especially in young patients, so it should usually raise the suspicion of diagnosis of APS if there is large vessel involvement such as CRAO or CRVO. APS is an autoimmune disorder that frequently affects young women and leads to recurrent venous or arterial thrombosis and/or fetal loss. Diagnosis of APS requires one out of two clinical criteria (vascular thrombosis or pregnancy morbidity) and one laboratory criteria that include either of APAs.[3],[4],[5] In a systemic review and meta-analysis which was conducted by Zhu et al., APAs, especially antiCL antibody but not LA was found to be risk factor for CRVO and BRVO.[4]

In another report, it was indicated that without anti-CL antibody, other APAs such as anti-phosphatidylcholine (anti-PC), antiphosphatidylethanolamine (anti-PE), antiphosphatidylinositol (anti-PI), and antiphosphatidylserine (anti-PS) antibodies increase the risk of CRVO.[6] We hereby present a case of SLE with RVO.

  Case Report Top

A 57-year-old woman was admitted with sudden decrease of visual acuity (VA) in her right eye with normal vision in the left eye. After obtaining inform constent, there was no history of diabetes mellitus (DM), hypertension, or smoking. On physical examination, the patient presented with normal respiratory, heart rate, and blood pressure but malar rash was found in the face. The uncorrected VA was 18/20 in the left and 6/20 in the right eye. After obtaining informed consent from the patient, the initial ophthalmoscopy of the right eye showed normal optic disc cup, flame-shaped retinal hemorrhages with normal view of the left eye. The fluorescein angiography (FA) showed abnormal vessel permeability in the fundus with edema along the vessels and occlusion of vein branches with leakage of the dye in these areas with flame-shaped retinal hemorrhages in the right eye [Figure 1], [Figure 2], [Figure 3]. The laboratory studies of the bleeding time were in the normal range. An immunologic study revealed 5.22 U/mL IgG anti-CL (normal <10 U/mL), 4,11 U/mL IgM anti-CL (normal <12.5 U/mL), and positive LA, antinuclear antibody (ANA) with granular pattern, and anti-ds DNA antibody – 327,4 IU/ml (normal <200 IU/mL). Homocysteine was 13.4 mmol/L (normal <12), low-density lipoprotein cholesterol – 175 mg/dL, and triglyceride – 292 mg/dL. Anti-RO/La and protein C and S antigens were in the normal range. Chest X-rays were normal. Magnetic resonance imaging of the head documented small nonspecific gliotic changes in the brain. Serologic tests for Chlamydia trachomatis, syphilis, Varicella–Zoster virus, human immunodeficiency virus 1, 2, hepatitis B surface antigen, and anti-hepatitis C virus were negative. She was started on warfarin and high-dose prednisolone (1 mg/kg/day) and hydroxychloroquine treatment for a month at first. Prednisolone treatment was continued with tapering doses in the later month. Because of detecting widespread capillary nonperfusion due to vaso-occlusion in her retina, VA did not change during follow-up after treatment.
Figure 1: Fundus photograph of the left eye without marked lesions

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Figure 2: Fundus photograph of the right eye with showing extensive flame-shaped intraretinal hemorrhages

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Figure 3: Fluorescein angiography of the right eye with showing extensive flame-shaped intraretinal hemorrhages

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  Discussion Top

RVO is the second most common retinal vascular disease after diabetic retinopathy. RVO is classified into CRVO and BRVO based on the site of vascular occlusion. Although RVO is commonly seen in the elderly, it can also occur in younger patients. While most elderly patients may have a history of atherosclerotic disease, hypertension, and DM, the causes in young patients have been still unclear which may contain thrombophilia, connective tissue diseases (such as SLE), APS, hyperhomocysteinemia, and hyperviscosity syndrome.[7] Although our case did not have any central nervous system (CNS) involvement, some studies support the strong association between occlusive ocular vascular disease and CNS in SLE.[8] There is a strong association between APAs and RVO disease, but the prevalence of APAs was found to be 23.4% in a study which included 368 patients with ophthalmic occlusive diseases.[9] APS is an autoimmune disorder that could lead to recurrent venous or arterial thrombosis, and the diagnosis requires including either of APAs.[4] APAs contain LA, anti-CL, and anti-β2 glycoprotein-1 antibodies, and in some cases, other APAs such as anti-PC, PE, PS, and PI may play important role in the pathogenesis of thrombotic complications such as CRVO or CRAO, which is called APS.[10] In many cases with CRVO, main APAs are expected to rise in laboratory studies, but rarely, minor components of APAs such as anti-PI, PE, and PC could be traced without increase of anti-CL and LA.[9] In our cases, we did not find the increasing of anti-CL and LA, and further examination was required to find minor components of APAs but our facility was not capable to study that examination. We aimed to remind here the importance of APAs and maybe we have to do further examination to find the minor components of APAs while initial studies confirmed no rising level of anti-CL and LA.

  Conclusion Top

Retinal vascular changes are frequently seen in patients with SLE, and vaso-occlusive retinopathy in these patients is often associated with APAs and APS, which merits prompt treatment with anticoagulation and corticosteroids.

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Conflicts of interest

There are no conflicts of interest.

  References Top

Monov S, Hristova R, Dacheva R, Toncheva R, Shumnalieva R, Shoumnalieva-Ivanova V, et al. Acute necrotizing retinal vasculitis as onset of systemic lupus erythematosus: A case report. Medicine (Baltimore) 2017;96:e5754.  Back to cited text no. 1
Bajwa A, Khurana G, Kimpel D, Reddy AK. Incidental retinal vascular occlusions on hydroxychloroquine screening in patients with systemic lupus erythematosus. Int Med Case Rep J 2015;8:121-6.  Back to cited text no. 2
Hong-Kee N, Mei-Fong C, Azhany Y, Zunaina E. Antiphospholipid syndrome in lupus retinopathy. Clin Ophthalmol 2014;8:2359-63.  Back to cited text no. 3
Zhu W, Wu Y, Xu M, Wang JY, Meng YF, Gu Z, et al. Antiphospholipid antibody and risk of retinal vein occlusion: A systematic review and meta-analysis. PLoS One 2014;10:e0122814.  Back to cited text no. 4
Kim JK, Kim MY, Yu HS, Jong HK, Hwang IS, Lee CW, et al. Retinal vein occlusion in two patients with primary antiphospholipid syndrome. Korean J Intern Med 2001;16:274-6.  Back to cited text no. 5
Seigo K, Hironori G, Chika G, Ryoko O, Toshiaki K, Tatsuro I. CASE REPORT central retinal vein occlusion in a pediatric patient with SLE and antiphospholipid antibodies without anti-cardiolipin or anti-β2 glycoprotein I antibodies. BMC Pediatr 2014;14:116.  Back to cited text no. 6
Sinawat S, Bunyavee C, Ratanapakorn T, Sinawat S, Laovirojjanakul W, Yospaiboon Y, et al. Systemic abnormalities associated with retinal vein occlusion in young patients. Clin Ophthalmol 2017;11:441-7.  Back to cited text no. 7
Song YH, Kim CG, Kim SD, Kim YY, Choe JY. Systemic lupus erythematosus presenting earlier as retinal vaso-occlusion. Korean J Intern Med 2001;16:210-3.  Back to cited text no. 8
Palmowski-Wolfe AM, Denninger E, Geisel J, Pindur G, Ruprecht KW. Antiphospholipid antibodies in ocular arterial and venous occlusive disease. Ophthalmologica 2007;221:41-6.  Back to cited text no. 9
Suvajac G, Stojanovich L, Milenkovich S. Ocular manifestations in antiphospholipid syndrome. Autoimmun Rev 2007;6:409-14.  Back to cited text no. 10


  [Figure 1], [Figure 2], [Figure 3]


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