Vaso-occlusive Retinopathy Research Articles

Chorioretinal Findings in a Patient with New-Onset Systemic Lupus Erythematosus and Antiphospholipid Syndrome after Stillbirth Associated with Pre eclampsia

ABSTRACT Purpose To describe chorioretinal findings in a patient with new-onset systemic lupus erythematosus (SLE) and antiphospholipid syndrome (APS) after a stillbirth associated with preeclampsia. Study design Case report. Results We report a patient with new-onset SLE and APS after pregnancy, who had a history of preeclampsia and intrauterine death that presented with bilateral visual loss after a seizure. Clinical findings of a unilateral vaso-occlusive retinopathy and choroidopathy associated with intraocular inflammation, serous retinal detachment, and vasculitis are presented, which responded well to immunosuppressive therapy. Conclusion New-onset systemic lupus erythematosus (SLE) during or after pregnancy could occur, especially when complicated with preeclampsia, making it difficult to diagnose accurately. Pregnancy-induced hypertension retinopathy and choroidopathy, as well as chorioretinal manifestations of SLE and APS, can share similar ocular manifestations that can overlap and coexist in the same patient, and it is important to recognize them for an adequate management and follow-up.

Retinal Arteriogenesis after Vaso-Occlusive Lupus Retinopathy

A 19-year-old woman presented with vaso-occlusive retinopathy associated with systemic lupus erythematosus. Baseline fluorescein angiography revealed capillary nonperfusion (Fig A). At 3 months, OCT angiography showed arteriolar occlusion, which had been previously perfused (Fig A, B, arrows). At 8 months, arteriogenesis was initiated in a direction different to that at baseline (Fig C, arrowheads). At 24 months, the arteriogenesis continued to extend, and the new arteriole formed an anastomosis with a pre-existing arteriole and a new bifurcation (Fig D, magnified).

Case Report: Recurrent retinal vein occlusion as the first clinical manifestation of systemic lupus erythematosus in a male patient

Systemic lupus erythematosus (SLE) is a chronic, autoimmune disease characterized by widespread clinical manifestations and immunological disorders. A myriad of ocular manifestations can be seen in patients with SLE. The most vision-threatening complication is vaso-occlusive retinopathy including retinal vein occlusion (RVO). RVO associated with SLE is well described in the literature and its association with antiphospholipid antibodies is recognized. However, RVO as the initial manifestation of SLE is scarcely reported. Herein, we report the first case of recurrent RVO as the revealing manifestation of SLE in a 40-year-old male patient. He had two consecutive episodes of decreased vision. Ophthalmologic examination disclosed a branch retinal vein occlusion the first time and a central retinal vein occlusion the second time. The diagnosis of SLE was established based on clinical and immunological criteria. He was prescribed antiplatelet therapy, hydroxychloroquine at 5.5 mg/kg/day, and intravitreal anti-vascular endothelial growth factor (VEGF) antibodies regimen. He slowly improved under treatment.

Case Report: Recurrent retinal vein occlusion as the first clinical manifestation of systemic lupus erythematosus in a male patient

Systemic lupus erythematosus (SLE) is a chronic, autoimmune disease characterized by widespread clinical manifestations and immunological disorders. A myriad of ocular manifestations can be seen in patients with SLE. The most vision-threatening complication is vaso-occlusive retinopathy including retinal vein occlusion (RVO). RVO associated with SLE is well described in the literature and its association with antiphospholipid antibodies is recognized. However, RVO as the initial manifestation of SLE is scarcely reported. Herein, we report the first case of recurrent RVO as the revealing manifestation of SLE in a 40-year-old male patient. He had two consecutive episodes of decreased vision. Ophthalmologic examination disclosed a branch retinal vein occlusion the first time and a central retinal vein occlusion the second time. The diagnosis of SLE was established based on clinical and immunological criteria. He was prescribed antiplatelet therapy, hydroxychloroquine at 5.5 mg/kg/day, and intravitreal anti-vascular endothelial growth factor (VEGF) antibodies regimen. He slowly improved under treatment.

Rheological Impact of GBT1118 Cessation in a Sickle Mouse Model.

In sickle cell disease (SCD), higher whole blood viscosity is a risk factor for vaso-occlusive crisis, avascular necrosis, and proliferative retinopathy. Blood viscosity is strongly impacted by hemoglobin (Hb) levels and red blood cell (RBC) deformability. Voxelotor is a hemoglobin S (HbS) polymerization inhibitor with anti-sickling properties that increases the Hb affinity for oxygen, thereby reducing HbS polymerization. In clinical trials, voxelotor increased Hb by an average of 1g/dl, creating concern that this rise in Hb could increase viscosity, particularly when the drug was cleared. To investigate this potential rebound hyperviscosity effect, we treated SCD mice with GBT1118, a voxelotor analog, and stopped the treatment to determine the effect on blood viscosity and RBC deformability under a range of oxygen concentrations. GBT1118 treatment increased Hb, improved RBC deformability by increasing the elongation index under normoxic (EImax) and hypoxic conditions (EImin), and decreased the point of sickling (PoS) without increasing blood viscosity. The anti-sickling effects and improvement of RBC deformability balanced the effect of increased Hb such that there was no increase in blood viscosity. Forty-eight hours after ceasing GBT1118, Hb declined from the rise induced by treatment, viscosity did not increase, and EImin remained elevated compared to control animals. Hb and PoS were not different from control animals, suggesting a return to native oxygen affinity and clearance of the drug. RBC deformability did not return to baseline, suggesting some residual rheological improvement. These data suggest that concerns regarding viscosity rise above pre-treatment levels upon sudden cessation of voxelotor are not warranted.

Radiation retinopathy masquerading diabetic retinopathy

Background: Radiation retinopathy (RR) can occur years after irradiation as a chronic and progressive retinal micro-angiopathy. The presentations of RR are similar to other retinal vaso-occlusive disorders like diabetic retinopathy, hypertensive retinopathy. The patient was referred to us as a case of diabetic retinopathy who had received intravitreal injections for macular edema without any improvement. A thorough evaluation revealed it to be a case of Radiation retinopathy. RR treatment includes laser photocoagulation, intravitreal anti-vascular endothelial growth factor (anti-VEGF) injections, intravitreal steroid injections, and surgery. Case presentation: We report a case of a 47-year-old diabetic male patient in whom RR developed following 30Gy of radiation therapy for Ethmoidal sinus carcinoma masquerading as Diabetic retinopathy. FFA was showing extensive ischemia of the retina, and OCT showing Macular edema. He received Pan retinal photocoagulation in both eyes followed by multiple intravitreal Anti-VEGF injections on a PRN basis and underwent Pars plana vitrectomy in the right eye for non-resolving vitreous hemorrhage. Conclusion: The patient's vision improved only after treating the retinal ischemia with photocoagulation and later treating the macular edema. This report emphasizes the importance of differentiating this disease entity from other vaso-occlusive retinopathy by eliciting a detailed patient history and planning further management accordingly.

Case of catastrophic antiphospholipid syndrome presenting as neuroretinitis and vaso-occlusive retinopathy

BackgroundOcular involvement in catastrophic antiphospholipid syndrome (CAPS), a rare, life-threatening form of antiphospholipid syndrome (APS) that results in multiorgan failure and a high mortality rate, has rarely been reported.Case presentationA 15-year-old girl presented with sudden vision blurring in both eyes. She had marked optic disc swelling and macular exudates in the right eye and intra-arterial white plaques, a few retinal blot hemorrhages, and a white ischemic retina in the left eye. Systemic examination revealed she had acute kidney injury with thrombotic microangiopathy (TMA), multiple cerebral infarcts, valvular dysfunction, and a high titer of triple aPL. Thus, she was diagnosed with CAPS involving the brain, eyes, heart, and kidneys. Plasma exchange and the administration of glucocorticoids, immunoglobulin, warfarin, and rituximab brought a sustained recovery of the TMA, visual symptoms, and echocardiographic findings.ConclusionsOcular involvement of both vaso-occlusive retinopathy, an APS-related thrombotic microangiopathy, and neuroretinitis, a non-thrombotic microangiopathy, can occur as an initial presentation of CAPS.

Retinal Detachment associated with Viral Retinitis

Viral retinitis of which incidence is 0.63/1000000 per year, is necrotizing, vaso-occlusive retinopathy. In practice, 90 % of cases are acute retinal necrosis in which etiological agents are Varicella-Zoster Virus and Herpes Simplex Virus and Cytomegalovirus retinitis. 65 % of patients with viral retinitis develop a retinal detachment. Retinal detachment due to viral retinitis is almost-always rhegmatogenous and tractional and associated with proliferative vitreoretinopathy. Pars plana vitrectomy combined with silicone oil injection is the most commonly used techniques for the treatment of retinal detachment associated with viral retinitis. Optic atrophy, chronic cystoid macular edema, epiretinal membrane, proliferative vitreoretinopathy, and hypotony are the most commonly seen complications that are limiting functional improvement.

Vaso-occlusive retinopathy and stage IV Lupus Nephritis; Primary manifestation of a 30 year old female

The diagnosis of systemic lupus erythematosus (SLE) depends on clinical evidence of renal, rheumatologic, cutaneous, and neurologic involvement, supported by serological markers. Ocular involvement is not included in diagnostic criteria of SLE. Vaso-occlusive retinopathy, as the primary manifestation of systemic lupus erythematosus (SLE), is relatively rare. We report a 30-year-old female, who suddenly suffered bilateral visual loss. Fundus examination revealed bilateral occlusive retinal vasculitis. She fulfilled the American College of Rheumatology criteria for diagnosing SLE; she had arthralgia, photosensitivity, positive ANA, high titre of anti-dsDNA, and proteinuria. A renal biopsy showed stage IV lupus nephritis. Treatment was initiated with pulse steroid and immunosuppressant. Best corrected visual acuity of both eye improved to finger counting after treatment. This case demonstrates that in patients with severe vaso-occlusive retinopathy, a generalized immunological disorder, like SLE, should be suspected.Bangladesh J Medicine Jan 2018; 29(1) : 36-40

Vaso-occlusive retinopathy by systemic lupus erythematosus associated with the antiphospholipid antibody syndrome

Resumo Relatar um caso de paciente com Retinopatia vaso-oclusiva por Lupus Eritematoso Sistemico (LES) associado a Sindrome do Anticorpo Antifosfolipideo (SAF), que se iniciou com um quadro de anemia hemolitica autoimune acompanhado por baixa visual subita monocular. Poucos casos foram descritos na literatura nacional e mundial em que o LES se manifeste primeiramente com alteracoes oculares. O screening dos Anticorpos antifosfolipideos (APAs) e de suma importância para pacientes com retinopatia lupica para que seja instituida a terapia imediata com anticoagulantes como forma de prevenir a trombose vascular, o que piora o prognostico visual.

Systemic lupus erythematosus and ocular involvement: an overview.

Systemic lupus erythematosus (SLE) is a multisystem autoimmune disease of undefined etiology and with remarkably heterogeneous clinical features. Virtually any organ system can be affected, including the eye. SLE-related eye involvement can be diagnosed in approximately one-third of the patients and is usually indicative of disease activity. An early diagnosis and the adoption of suitable therapeutic measures are necessary to prevent sight-threatening consequences, especially in patients with juvenile SLE. Periocular lesions, such as eyelid involvement and orbital inflammation, are relatively rare and, in case of orbital masses, may require a biopsy control. Keratoconjunctivitis sicca or secondary Sjögren's syndrome is the most frequent ophthalmic manifestation of SLE. According to its variable severity, lubricating tear drops may be sufficient in mild cases, whereas cyclosporine-A ophthalmic solution, glucocorticoids (GCs), methotrexate, and/or other immunosuppressive drugs may be required in the more severe cases. Partial occlusion of the lacrimal punctum by thermal cautery is rarely applied. Although uncommon, episcleritis and scleritis can sometimes be detected as an initial finding of SLE and reveal themselves as moderate to intense ocular pain, redness, blurred vision, and lacrimation. Unilateral or more often bilateral retinopathy is responsible for visual loss of variable severity and is ascribed to vasculitis of the retinal capillaries and arterioles. In addition to the combined treatment suitable for all patients with active SLE, intravitreal bevacizumab should be considered in cases of severe vaso-occlusive retinopathy and laser photocoagulation in cases of neovascularization. Purtscher-like retinopathy is likely ascribable to the formation of microemboli that results in retinal vascular occlusion and microvascular infarcts. Choroidal disease is characterized by monolateral or bilateral blurred vision. Because of the choroidal effusion, retinal detachment and secondary angle-closure glaucoma may occur. Ischemic optic neuropathy is characterized by acute-onset and progressive binocular visual impairment as a consequence of occlusion of the small vessels of the optic nerves due to immune complex vasculitis. Intravenous GC boluses followed by oral GCs and/or, in case of recurrence, intravenous cyclophosphamide and/or rituximab are commonly employed. Neovascularization can be treated by intravitreal bevacizumab and progression of retinal ischemic areas by retinal laser photocoagulation. Ocular adverse events (AE) have been described following the long-term administration of one or more of the drugs presently used for the treatment of SLE patients. Posterior subcapsular cataracts and secondary open-angle glaucoma are common AE of the prolonged GC administration. The long-term administration of hydroxychloroquine (HCQ) sulfate is well known to be associated with AE, such as vortex keratopathy and in particular the often irreversible and sight-threatening maculopathy. Length of administration>5years,>1000g total HCQ consumption,>6.5mg/kg daily dosing, coexistence of renal disease, and preexisting maculopathy are all considered risk factors for HCQ-induced retinopathy. Ocular AE of additional immunosuppressive and biological agents are still poorly known, given the worldwide more limited experience with their long-term use. A thorough ophthalmological control is strongly recommended at closer intervals for all SLE patients, in step with the total length of exposure to the drugs and the cumulative dose administered.

Macular capillary recovery in systemic lupus erythematosus complicated by Kikuchi–Fujimoto disease

Few case reports have described vaso-occlusive retinopathy in systemic lupus erythematosus (SLE) using optical coherence tomography (OCT) angiography. Here we report the clinical features of a patient with SLE, complicated by Kikuchi-Fujimoto disease, who developed vaso-occlusive retinopathy. We then describe the subsequent recovery of the macular capillaries as assessed by OCT angiography. A 16-year-old male was referred to us with fever, a 1-month history of violaceous red papules and erythematous plaques on his face and a painful nodule in his right neck. We diagnosed him with SLE complicated by Kikuchi-Fujimoto disease through physiological assessment and histology from his neck lymph node and chin skin. Systemic steroids were prescribed as treatment. After remission, his fever and cervical lymph node swelling with pain recurred and he developed blurred inferior vision in his left eye. His best-corrected visual acuities were 1.0 and 0.1 in the right and left eyes, respectively. Extensive cotton wool spots were observed in the right fundus, and retinal capillary occlusions were detected by OCT angiography of the left eye. We diagnosed this case as vaso-occlusive retinopathy with SLE and increased immunosuppressive treatment together with anticoagulation therapy. Macular capillaries, observed by OCT angiography, gradually recovered function following assessment at 7 and 16months post-onset of the vaso-occlusive retinopathy. We reported a 1½-year course of vaso-occlusive retinopathy in a patient with SLE complicated by Kikuchi-Fujimoto disease. Occlusion of the retinal vasculature and the subsequent recovery of circulation are clearly observed by OCT angiography.

Antiphospholipid syndrome in lupus retinopathy.

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.

Bilateral Intravitreal Ranibizumab Injection and Panretinal Photocoagulation in a 16-Year-Old Girl with Severe Vaso-Occlusive Lupus Retinopathy

A 16-year-old girl with fever of unknown origin and bilateral vaso-occlusive retinopathy with retinal neovascularization, preretinal hemorrhage, and serous macular detachment was treated with single bilateral 0.5 mg intravitreal ranibizumab injection prior to aggressive PRP with success. No systemic steroids or immunosuppressive therapy was employed at that time. She received the diagnosis of “systemic lupus erythematosus” five years after this episode with further systemic symptoms. In certain cases with vaso-occlusive type of lupus retinopathy, anti-VEGF agents may be administered in addition to panretinal photocoagulation to achieve better visual and anatomic outcome.

A Case of Severe Vaso-Occlusive Retinopathy as the First Manifestation Associated with Systemic Lupus Erythematosus

A Case of Severe Vaso-Occlusive Retinopathy as the First Manifestation Associated with Systemic Lupus Erythematosus

Vaso-occlusive Retinopathy in a Patient with Systemic Lupus Erythematosus

Vaso-occlusive Retinopathy in a Patient with Systemic Lupus Erythematosus

Inaugural severe vaso-occlusive retinopathy in systemic lupus erythematosus

Purpose To report the case of a patient with an inaugural severe bilateral vaso-occlusive retinopathy due to systemic lupus erythematosus. Method Clinical examination, fundus pictures and fluorescein angiography were performed. Results A 26-year-old, healthy, African man presented with a meningo-encephalitic syndrome and a severe bilateral visual impairment. The fundus examination revealed multiple retinal vascular occlusions, and a fluorescein angiography showed retinal and choroidal ischemia bilaterally. In addition, based on the neurologic disorders, a pleuritis, a renal disorder and a hematologic disorder, systemic lupus erythematosus, was diagnosed. Conclusions Severe vaso-occlusive retinopathy in a 26-year-old man resulting in a significant visual loss as the initial manifestation of systemic lupus erythematosus. Central nervous system involvement during lupus might be more frequent in patients when serious retinal changes occur.

Branch Retinal Artery Occlusion Secondary to Antiphospholipid Syndrome

To demonstrate through a case report a common ocular manifestation of antiphospholipid syndrome (APS). APS is an autoimmune disorder characterized by arterial or venous thrombosis that can affect any organ system. Antibodies associated with the syndrome include anticardiolipin antibodies, lupus anticoagulant, and antibodies directed against beta-2-glycoprotein 1. These molecules all play a role in inhibiting the coagulation cascade, promoting thrombosis, and causing a hypercoagulable state. The most common ocular manifestation is vaso-occlusive retinopathy. A 54-year-old man presented with complaints of a transient ‘‘fog’’ over the vision in his right eye, lasting 2 to 3 minutes for 6 months. Dilated fundus examination revealed a branch retinal artery occlusion (BRAO) along the superior temporal arcade right eye (OD). Given the patients age, a hematology consult was obtained to rule out hypercoagulable disorders. Laboratory testing confirmed a diagnosis of APS. Ocular findings can often be the first presenting sign of APS; therefore, it is imperative that primary eye care providers include this disorder in the differential diagnosis of vaso-occlusive disease. This case report reviews APS as the etiology of a BRAO. In addition, common systemic manifestations, ocular manifestations, and treatment and management options for APS are discussed.

Pulsed intravenous cyclophosphamide for sle retinal vasculitis.

To report a case of systemic lupus erythematosus vaso-occlusive retinopathy with severe visual loss treated with intravenous pulsed cyclophosphamide. Retrospective interventional case report. A 20-year-old Cambodian woman with newly diagnosed systemic lupus erythematosus presented with acute visual loss. Fluorescein fundus angiography demonstrated occlusive retinal vasculitis. Treatment with pulsed intravenous cyclophosphamide, intravenous methylprednisolone, and anticoagulation resulted in recovery of vision from count fingers to 6/6 in both eyes. Early aggressive immunosuppression and anticoagulation for systemic lupus erythematosus retinal vasculitis can be beneficial in preventing disease progression and restoring vision. Further studies are needed to compare dosage regimens.

Intravitreal bevacizumab for severe vaso-occlusive retinopathy in systemic lupus erythematosus

Severe vaso-occlusive retinopathy is a relatively rare form of retinopathy in systemic lupus erythematosus (SLE). We report two patients with severe vaso-occlusive retinopathy in SLE who were treated with intravitreal bevacizumab (IVB). (Patient 1) A 35-year-old woman presented with left visual loss and was diagnosed with SLE after systemic evaluation. Despite systemic immunosuppressive therapy, retinal vascular obstruction progressed and neovascularization of the disk (NVD) developed. The patient was treated with IVB and pan retinal photocoagulation. The progression of vascular obstruction ceased and regressed. (Patient 2) A 24-year-old man with SLE presented with left visual loss. There was retinal vascular obstruction with macular edema in both eyes, and then the patient was treated with IVB. One month after injection, minimal capillary nonperfusion increased to 10 disk area, and 5 months later, neovascularization elsewhere (NVE) developed in the right eye. Six months after injection, vitreous hemorrhage with florid NVE and NVD developed in the left eye. In selected severe vaso-occlusive retinopathy in SLE patients, IVB may be an adjuvant option for treatment.