systemic lupus erythematosus ( sle ) is a chronic , multisystem , autoimmune disease that can affect any part of the human body . ocular manifestations in sle are fairly common and should be taken seriously as they may lead to significant morbidity and visual deterioration . in addition to that , ocular manifestations could represent the disease activity of the lupus ( especially in the presence of retinal signs ) and serve as an indicator of adequacy of treatment in control of the disease.1 common ocular signs that could lead to visual blinding complications include retinal involvement ( central retinal artery occlusion [ crao ] , central retinal vein occlusion [ crvo ] , severe vaso - occlusive retinopathy , and retinal detachment ) and optic nerve involvement ( optic neuritis and ischemic optic neuropathy).2 milder spectrum includes keratoconjunctivitis sicca , episcleritis , scleritis , and anterior uveitis . antiphospholipid antibodies ( apa ) have been recognized as a risk factor for complications in sle and their clinical significance in ocular presentation have been associated with poorer prognosis.3 clinicians will usually raise their suspicion of antiphospholipid syndrome ( aps ) in sle patients if there is large vessel involvement such as crao or crvo . we are reporting two cases of aps in sle patients that presented with crvo ( case 1 ) and vaso - occlusive lupus retinopathy ( case 2 ) . a 19-year - old girl with known sle for 3 years and was on maintenance oral prednisolone 10 mg daily presented in the ophthalmology clinic at the hospital raja permaisuri bainun , ipoh , malaysia with sudden , painless loss of vision in the left eye of 1 day s duration . visual acuity was hand movement in the left eye with the presence of relative afferent pupillary defect ( rapd ) , and was 6/6 in the right eye . anterior segment examinations of both eyes were unremarkable . left fundoscopy showed extensive flame - shaped intraretinal hemorrhages , dilated and tortuous retinal veins with swollen and hyperemic disc suggestive of crvo ( figure 1 ) . fundus fluorescein angiography ( ffa ) of the left eye revealed delayed venous filling and prolonged arteriovenous transit time with extensive capillary nonperfusion affecting 360 of peripheral retina with macular ischemia ( figure 2 ) . ffa of the right eye was unremarkable . in view of the ocular vascular thrombotic event , she was screened for laboratory criteria for aps , which consisted of lupus anticoagulant , anticardiolipin antibody , and -2 glycoprotein-1 antibody . the screening revealed that the anticardiolipin antibody was positive at a moderate titer ( igg : 73.85 gpl units , igm : 8.62 mpl units ) . she was given intravenous methylprednisolone 500 mg daily for 3 days followed by oral prednisolone 55 mg ( 1 mg / kg daily ) for 2 weeks . oral prednisolone was tapered down 10 mg every 2 weeks until 30 mg , and then further tapered down 5 mg weekly until maintenance of 5 mg daily . the patient was also treated with oral hydroxychloroquine ( hcq ) 200 mg daily and oral warfarin 5 mg daily . she was planned for long - term treatment of hcq and oral anticoagulant . in view of the extensive capillary nonperfusion area in the left eye , laser panretinal photocoagulation ( prp ) the left fundus showed pallor disc with resolving retinal hemorrhages with extensive sclerosed retinal vessels ( figure 3 ) . she is still on maintenance treatment with oral prednisolone 5 mg daily , oral hcq 200 mg daily , and oral warfarin 5 mg daily . a 16-year - old girl with known sle and lupus nephritis for 10 months , on maintenance oral prednisolone 20 mg daily , oral mycophenolate mofetil ( mmf ) 750 mg daily , and oral hcq 200 mg twice daily . her ultrasound doppler showed thrombosis of the right external iliac and common femoral vein and was started on oral warfarin for 6 months duration . she presented to the ophthalmology clinic in hospital raja permaisuri bainun , ipoh , malaysia with bilateral central scotomas of 4 days duration . fundoscopy showed multiple cotton wool spots , intraretinal hemorrhages , and macular edema bilaterally ( figure 4a and b ) . ffa showed perifoveal vasculitis with macular ischemia and capillary nonperfusion at superior , temporal , and inferior parts of the retina in both eyes ( the left eye more severe than the right eye ) ( figure 5a and b ) . apa screening showed positive anticardiolipin antibody ( igg:12.6 gpl units ) and lupus anticoagulant ( ratio between 1.2 to 1.5 ) . she was given intravenous methylprednisolone 500 mg daily for 3 days followed by oral prednisolone 45 mg daily ( 1 mg / kg daily ) , then tapered down 10 mg every 2 weeks until maintenance of 10 mg daily . oral anticoagulant ( she was planned for long - term oral anticoagulant . in view of vaso - occlusive retinopathy at peripheral retina , laser prp was given in both eyes at superior , temporal , and inferior parts of the retina in three sessions to prevent neovascularization . at 12 weeks follow - up , her visual acuity was 6/12 in the right eye and 6/24 in the left eye with persistent central scotomas bilaterally . both fundus showed resolving of cotton wool spots and intraretinal hemorrhages with the presence of hard exudate at the macular area ( figure 6a and b ) . she is still on maintenance treatment with oral prednisolone 10 mg daily , oral warfarin 5 mg daily , oral mmf 750 mg daily , and oral hcq 200 mg twice daily . a 19-year - old girl with known sle for 3 years and was on maintenance oral prednisolone 10 mg daily presented in the ophthalmology clinic at the hospital raja permaisuri bainun , ipoh , malaysia with sudden , painless loss of vision in the left eye of 1 day s duration . visual acuity was hand movement in the left eye with the presence of relative afferent pupillary defect ( rapd ) , and was 6/6 in the right eye . anterior segment examinations of both eyes were unremarkable . left fundoscopy showed extensive flame - shaped intraretinal hemorrhages , dilated and tortuous retinal veins with swollen and hyperemic disc suggestive of crvo ( figure 1 ) . fundus fluorescein angiography ( ffa ) of the left eye revealed delayed venous filling and prolonged arteriovenous transit time with extensive capillary nonperfusion affecting 360 of peripheral retina with macular ischemia ( figure 2 ) . ffa of the right eye was unremarkable . in view of the ocular vascular thrombotic event , she was screened for laboratory criteria for aps , which consisted of lupus anticoagulant , anticardiolipin antibody , and -2 glycoprotein-1 antibody . the screening revealed that the anticardiolipin antibody was positive at a moderate titer ( igg : 73.85 gpl units , igm : 8.62 mpl units ) . she was given intravenous methylprednisolone 500 mg daily for 3 days followed by oral prednisolone 55 mg ( 1 mg / kg daily ) for 2 weeks . oral prednisolone was tapered down 10 mg every 2 weeks until 30 mg , and then further tapered down 5 mg weekly until maintenance of 5 mg daily . the patient was also treated with oral hydroxychloroquine ( hcq ) 200 mg daily and oral warfarin 5 mg daily . she was planned for long - term treatment of hcq and oral anticoagulant . in view of the extensive capillary nonperfusion area in the left eye , laser panretinal photocoagulation ( prp ) the left fundus showed pallor disc with resolving retinal hemorrhages with extensive sclerosed retinal vessels ( figure 3 ) . she is still on maintenance treatment with oral prednisolone 5 mg daily , oral hcq 200 mg daily , and oral warfarin 5 mg daily . a 16-year - old girl with known sle and lupus nephritis for 10 months , on maintenance oral prednisolone 20 mg daily , oral mycophenolate mofetil ( mmf ) 750 mg daily , and oral hcq 200 mg twice daily . her ultrasound doppler showed thrombosis of the right external iliac and common femoral vein and was started on oral warfarin for 6 months duration . she presented to the ophthalmology clinic in hospital raja permaisuri bainun , ipoh , malaysia with bilateral central scotomas of 4 days duration . fundoscopy showed multiple cotton wool spots , intraretinal hemorrhages , and macular edema bilaterally ( figure 4a and b ) . ffa showed perifoveal vasculitis with macular ischemia and capillary nonperfusion at superior , temporal , and inferior parts of the retina in both eyes ( the left eye more severe than the right eye ) ( figure 5a and b ) . apa screening showed positive anticardiolipin antibody ( igg:12.6 gpl units ) and lupus anticoagulant ( ratio between 1.2 to 1.5 ) . she was given intravenous methylprednisolone 500 mg daily for 3 days followed by oral prednisolone 45 mg daily ( 1 mg / kg daily ) , then tapered down 10 mg every 2 weeks until maintenance of 10 mg daily . she was planned for long - term oral anticoagulant . in view of vaso - occlusive retinopathy at peripheral retina , laser prp was given in both eyes at superior , temporal , and inferior parts of the retina in three sessions to prevent neovascularization . at 12 weeks follow - up , her visual acuity was 6/12 in the right eye and 6/24 in the left eye with persistent central scotomas bilaterally . both fundus showed resolving of cotton wool spots and intraretinal hemorrhages with the presence of hard exudate at the macular area ( figure 6a and b ) . she is still on maintenance treatment with oral prednisolone 10 mg daily , oral warfarin 5 mg daily , oral mmf 750 mg daily , and oral hcq 200 mg twice daily . 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 includes either lupus anticoagulant , anticardiolipin antibody in medium or high titer , or anti-2 glycoprotein-1 antibody . the presence of these autoantibodies causes imbalance to the hemostasis , which favors the prothrombotic state , thus leading to arterial and venous thrombosis , and is associated with more severe retinopathy and vascular occlusion.4 apa is often associated with a major vascular occlusion like crvo or crao . crvo cases are more likely than controls to have high anticardiolipin antibody levels with an odds ratio of 6.26.5 the first patient presented with crvo , which involved major vascular occlusion and illustrates a classical presentation of aps ; thus , she was screened for apa , which turned out to be positive and was started on an anticoagulant with oral warfarin . standard treatment for initial venous thrombotic event is early initiation with oral warfarin.6 mild lupus retinopathy consists of cotton wool spots , retinal hemorrhages , and vascular tortuosity , where as severe lupus retinopathy consists of occlusion of retinal arterioles leading to retinal infarction , hence termed vaso - occlusive retinopathy or retinal vasculitis . although the second patient only presented with features of vaso - occlusive lupus retinopathy , screening for aps was done in view of recent history of dvt , which turned out to be positive as well . initial management of lupus retinopathy includes systemic immunosuppression ( usually intravenous corticosteroids followed by oral steroid ) , which is then supplemented or replaced by other immunosuppressive agents . however , if apa is present , prompt use of anticoagulant is beneficial , as apa is linked to focal thrombosis.7 therefore , screening for aps in the second patient prevented a delay in initiating the anticoagulant and worsening of her condition . we have highlighted two cases of sle whereby the first case with crvo illustrates a classical presentation of aps and the second case with features of active vaso - occlusive lupus retinopathy . however , aps can be present in sle regardless of the nature of retinopathy . hence , screening for aps is useful , as prompt treatment with anticoagulants is important to prevent further vascular thrombosis and worsening visual prognosis . although systemic steroids and antithrombotic agent were given in these cases , visual prognosis was not good as a result of macular ischemia , leading to permanent visual disability . nevertheless , the most important issue now is to prevent a similar condition in the fellow eye , therefore antiinflammatory and antithrombotic need to be given for a longer duration .
systemic lupus erythematosus ( sle ) is a chronic , multisystem , autoimmune disease that can affect any part of the human body including the eyes . 
 common blinding ocular manifestations include central retinal artery occlusion ( crao ) , central retinal vein occlusion ( crvo ) , severe vaso - occlusive retinopathy , and optic nerve involvement . 
 antiphospholipid syndrome ( aps ) in lupus is usually associated with large vessel occlusions and needs prompt treatment with anticoagulant . 
 we are reporting two cases of aps in sle patients that presented with crvo ( case 1 ) and vaso - occlusive lupus retinopathy ( case 2 ) . 
 both cases were positive for antiphospholipid antibody ( apa ) and were treated with immunosuppression , anticoagulant , and laser treatment . 
 thus , screening for apa is vital in sle patients with lupus retinopathy , as prompt treatment with anticoagulants is important to prevent further vascular thrombosis , which worsens the visual prognosis .