Atopic Dermatitis Associated Retinal Detachment and Retinal Vasculitis: A Case Report.

Atopic dermatitis is usually associated with various ocular complications. We report a 21-year-old Chinese male who presented to our ophthalmology clinic with bilateral retinal detachment and cataracts. The patient had a clear medical history of atopic dermatitis, which had been diagnosed eight years earlier and had been treated with loratadine and pimecrolimus. Cataract surgery was performed for both eyes, combined with scleral buckling for the right eye and pars plana vitrectomy for the left eye. During postoperative follow-up, fundus fluorescein angiography showed retinal vasculitis in both eyes and macular edema in the left eye, which coincided with an exacerbation of atopic dermatitis. Macular edema improved after four months of regular dupilumab treatment in the dermatology department. The ocular condition remained stable three years postoperatively.

[Diagnostic modalities of a Behçet uveitis]. / Comment j'explore une uvéite de Behçet.

Behçet disease is a chronic multisystemic inflammatory condition. The ocular disease mainly manifests as a bilateral, non-granulomatous, posterior uveitis or panuveitis, associated with occlusive retinal vasculitis. Uveitis is frequent in Behçet disease, it may be severe, and visually threatening. Early diagnosis and aggressive treatment are mandatory to preserve visual function. Therefore, the goal of this review is to describe the new diagnosis tools and therapeutic guidelines allowing for a significant improvement of the visual prognosis.

La maladie de Behçet est une pathologie inflammatoire chronique multisystémique. L'atteinte oculaire se caractérise le plus souvent par une panuvéite ou uvéite postérieure bilatérale récidivante, non granulomateuse, associée à une vascularite rétinienne occlusive. L'uvéite associée à la maladie de Behçet est fréquente, sévère et potentiellement cécitante en l'absence d'un diagnostic précoce et d'un traitement adapté. L'objectif de cette revue est de décrire les outils diagnostiques et thérapeutiques ayant permis une amélioration significative du pronostic visuel.

CLINICOPATHOLOGICAL ANALYSIS OF SECONDARY RETINAL VASOPROLIFERATIVE TUMOR/REACTIVE RETINAL ASTROCYTIC TUMOR SUCCESSFULLY TREATED BY ENDORESECTION.

PURPOSE: To report the clinicopathological findings of retinal vasoproliferative tumor/reactive retinal astrocytic tumor (VPT/RRAT) with retinal vasculitis, treated by tumor resection. METHODS: A retrospective single case report. PATIENT: A 29-year-old Japanese woman was referred with cystoid macular edema and retinal vasculitis in the right eye. Best-corrected visual acuity was 0.9. Results of fundus examination, optical coherence tomography, and fluorescein angiography demonstrated VPT/RRATs in the temporal retina surrounded by a subretinal exudate, serous retinal detachment and macular edema, and retinal vasculitis. Despite 3 months of oral prednisolone treatment, a full-thickness macular hole developed. Pars plana vitrectomy and endoresection of the VPT/RRATs were performed. Pathologic and immunohistochemical analyses with anti-CD34 antibody, antiglial fibrillary acidic protein antibody, anti-Ki67 antibody, and anti-vascular endothelial growth factor antibody were performed on the excised tissue. Inflammation was evaluated by immunohistological staining with leukocyte common antigen (LCA), anti-CD3 antibody, and anti-CD20 antibody. RESULTS: After surgery, the macular hole closed, best-corrected visual acuity improved to 1.2, retinal vasculitis was ameliorated, and retinal exudate disappeared. There was no recurrence of VPT/RRAT or retinal vasculitis. Pathologic examination showed that antiglial fibrillary acidic protein and anti-vascular endothelial growth factor were widely expressed, irrespective of the distribution of blood vessels. Ki67-positive proliferating cells were detected in the perivascular area. Leukocyte common antigen-positive leukocytes and CD3-positive T cells were detected throughout the samples, whereas CD20-positive B cells were rarely detected. CONCLUSION: Endoresection of VPT/RRAT could be a good treatment option for secondary VPT/RRAT accompanied by retinal vasculitis. Pathologic findings revealed for the first time that inflammatory cells infiltrate the tissue in secondary VPT/RRAT.

Bilateral Retinal Vasculitis as Initial Presentation of Systemic Lupus Erythematosus with Secondary Antiphospholipid Syndrome.

BACKGROUND Systemic lupus erythematosus (SLE) is a multisystem autoimmune disease of undefined etiology with a relapsing and remitting course. Lupus retinopathy is reported in around 10% of patients with SLE; however, it is rarely the initial presenting feature of the disease. We report a unique case of bilateral retinal vasculitis as the initial presentation of SLE with secondary antiphospholipid syndrome (APS). CASE REPORT A 34-year-old man, previously healthy, presented to the eye clinic for the first time with painless reduced vision for 3 weeks. A review of systems revealed generalized fatigue, myalgia, arthralgias, and weight loss of around 10 kg in the last 3 months. On ophthalmic examination, his visual acuity was reduced bilaterally, more in the right eye. A fundus exam revealed bilateral diffuse multiple cotton-wool spots, dot and blot hemorrhage covering the posterior pole, and venous congestion and beading. In addition, there was cystoid macular edema (CME) in the fovea of both eyes, and fundus fluorescein angiography (FFA) showed bilateral areas of peripheral and macular hypo-fluorescence, multiple hyper-fluorescent knob-like aneurysmal dilatations, and vascular leaking and staining. He was diagnosed with SLE by the rheumatology team based on the clinical presentations and laboratory investigations. The patient was managed with intravenous methylprednisolone and discharged on oral prednisone with a tapering regimen. Eighteen months after, he reported significant improvement in his vision with regular follow-ups. CONCLUSIONS Ocular manifestations can be the initial presentation of SLE and can lead to serious ocular complications. Early diagnosis and proper management are essential and require cooperation between rheumatologists and ophthalmologists.

Rescue Treatment with Infliximab for a Bilateral, Severe, Sight Threatening Frosted Branch Angiitis Associated with Concomitant Acute Onset of Presumed Dermatomyositis.

PURPOSE: To report a severe bilateral frosted branch angiitis associated with acute onset of systemic dermatomyositis. METHOD: History and clinical examination, laboratory evaluation, fundus' and skin's color pictures, fluorescein angiography (FA), optical coherence tomography (OCT). RESULTS: A 32-year-old female was referred for a bilateral frosted branch angiitis and vitreitis, with skin rash, muscle pain, weakness, severe headache, compatible with dermatomyositis. After an initial improvement following an aggressive treatment by systemic steroids and mycophenolate mofetil, she was switched to intravenous infliximab (RemicadeTM, Janssen Biotech, Inc. USA) due to lack of efficacy. Nine months later, her visual acuity was improved up to 20/20 in OU, with both retinal vasculitis and vitreitis being solved. CONCLUSION: Dermatomyositis is a severe multiorgan disease which might severely involve eye structures. Anti-TNF-Alpha agents, particularly Infliximab, might offer a rapid control and long-term remission.

Anti-CD20 therapy for multiple sclerosis-associated uveitis: A case series.

BACKGROUND AND PURPOSE: Approximately 1% of patients with multiple sclerosis (MS) have uveitis, but data on the effects of immunotherapies for MS on MS-associated uveitis are scarce. The aim of this study was to investigate the ophthalmological outcomes in patients with MS-associated uveitis treated with anti-CD20 therapy. METHODS: A retrospective study of 12 eyes of six patients with MS-associated uveitis, refractory to previous immunotherapies, was conducted. Uveitis activity was assessed before initiation of anti-CD20 therapy and at regular follow-up visits. Primary outcome measures were: vitreous haze score; retinal vasculitis score, determined on fluorescein angiography images; macular edema, as quantified by central retinal thickness (CRT) on optical coherence tomography; and visual acuity (VA). Secondary outcomes included number of annualized uveitis or MS relapses, disease activity on cerebral magnetic resonance imaging (cMRI) and Expanded Disability Status Scale (EDSS) score. RESULTS: After a median (interquartile range [IQR]) treatment time of 28.5 (8-43) months, anti-CD20 therapy was associated with an improvement of vitreous haze score (p = 0.002), retinal vasculitis score (p = 0.001), CRT (p = 0.002), and VA (p = 0.007). The median (IQR) annualized uveitis relapse rate declined from 0.59 (0.56-0.94) before to 0 (0-0.49) after the start of anti-CD20 therapy. The median (IQR) annualized MS relapse rate declined from 0.62 (0.26-2.84) before to 0 (0-0) after the start of anti-CD20 therapy. After initiation of anti-CD20 therapy, there was no disease activity on cMRI, and EDSS score improved (n = 2) or remained stable (n = 4). No severe adverse events were observed. CONCLUSION: These findings suggest that anti-CD20 therapy may be a valuable treatment option for MS-associated uveitis.

Bilateral Occlusive Retinal Vasculitis in a Patient with Dermatomyositis.

A 48-year-old woman with a history of dermatomyositis (DMS) presented with 2 weeks of worsening myalgias, weakness, and diffuse edema following cessation of her systemic immunosuppression and subsequently developed severe bilateral vision loss consistent with bilateral frosted branch angiitis. Multimodal imaging was performed, and the patient was successfully treated with pulse-dose steroids and intravenous immunoglobulin, as well as intravitreal aflibercept. Ophthalmic involvement of DMS is typically limited to episcleritis, conjunctivitis, and uveitis. We present an uncommon case of bilateral occlusive retinal vasculitis with frosted branch angiitis in a patient with DMS. The significant improvement anatomically and in visual acuity in our patient suggests a role of combined anti-vascular endothelial growth factor and systemic immunosuppression in the management of DMS -related frosted branch angiitis. We suggest that retinal vasculitis should be considered in patients with known DMS and acute vision loss, with prompt referral for ophthalmologic evaluation.

Subretinal Hemorrhage Complicating Retinal Angiomatous Proliferation in Tubercular Retinal Vasculitis.

PURPOSE: To report a patient with submacular hemorrhage due to retinal angiomatous proliferation (RAP) in tubercular retinal vasculitis (TRV). METHODS: Case report. RESULTS: We report a 33-year-old Asian Indian patient of TRV presenting with capillary non-perfusion areas, submacular hemorrhage and venous loops. The patient presented with sudden onset decrease in vision in the right eye. Multimodal imaging revealed presence of retinal vascular anastomosis and stage 2 RAP. Systemic examination was within normal limits. Laboratory evaluation revealed positive Mantoux and interferon gamma release assay. He underwent right eye intravitreal injection of recombinant tissue plasminogen activator (12.5µg/0.1ml) with 100% sulphur hexafluoride (SF6) tamponade. The patient had successful displacement of the submacular hemorrhage with some improvement in visual acuity. CONCLUSION: This case highlights that rare vascular alterations such as RAP can develop in subjects with ocular tuberculosis.

EFFICACY OF INTRAVITREAL AFLIBERCEPT INJECTIONS IN THE TREATMENT OF IDIOPATHIC RETINAL VASCULITIS, ANEURYSMS, AND NEURORETINITIS SYNDROME.

PURPOSE: To present a case of idiopathic retinal vasculitis, aneurysms, and neuroretinitis syndrome that was successfully managed with serial intravitreal aflibercept injections. METHODS: Ophthalmic imaging and visual acuity were used to monitor disease state and track treatment methods to determine the most valuable combination of treatment medication and treatment interval. RESULTS: A 28-year-old woman with idiopathic retinal vasculitis, aneurysms, and neuroretinitis syndrome status after panretinal photocoagulation of both eyes presented with bilateral cystoid macular edema. We demonstrate successful management of retinal cystoid macular edema associated with idiopathic retinal vasculitis, aneurysms, and neuroretinitis syndrome using serial intravitreal aflibercept injections. CONCLUSION: Intravitreal aflibercept has a useful role in managing the potential retinal complications associated with idiopathic retinal vasculitis, aneurysms, and neuroretinitis syndrome and provides further insights into treatment of the later stages of this rare disease.

Relevance of Brain MRI in Patients with Uveitis: Retrospective Cohort on 402 Patients.

AIM: To assess the diagnostic value of brain magnetic resonance imaging (bMRI) for the etiological diagnosis of uveitis and to establish predictive factors associated with its advantageous use. METHODS: Retrospective study on all patients with de novo uveitis who were referred to our tertiary hospital and who underwent a bMRI between 2003 and 2018. RESULTS: bMRI was contributive in 19 out of 402 cases (5%), among patients with a contributive bMRI, 68% had neurological signs. Univariate analysis established that neurological signs (p < .001), granulomatous uveitis (p = .003), retinal vasculitis (p = .002), and intermediate uveitis (p < .001) were all significantly associated with a contributive bMRI. Multivariate analysis confirms the significant association of neurological signs (p < .001) and intermediate uveitis (p = .01). CONCLUSION: bMRI appears to be a relevant exam in specific cases; intermediate/posterior uveitis or panuveitis accompanied by neurological signs, retinal vasculitis, or in patients older than 40, to rule out an oculocerebral lymphoma. ABBREVIATIONS: ACE: Angiotensin-Converting Enzyme; bMRI: Magnetic Resonance Imaging; CBC: Complete Blood cell Count; BMRI: Brain Magnetic Resonance Imaging; CT: Computerized Tomography; MS: Multiple Sclerosis; NS: Neurological Signs; OCL: Oculocerebral Lymphoma; RIS: Radiologically Isolated Syndrome.

Vitrectomy Due to Vitreous Hemorrhage and Tractional Retinal Detachment Secondary to Eales' Disease

Objectives: To investigate visual and anatomical outcomes of vitreoretinal surgeries in patients with Eales' disease. Materials and Methods: In this retrospective study, 22 eyes of 21 patients with vitreous hemorrhage (VH) or tractional retinal detachment (TRD) secondary to Eales' disease who underwent vitreoretinal surgery between January 1997 and December 2015 and had at least 1 year of follow-up were included. Results: The mean best corrected visual acuity (BCVA) was significantly higher at final visit (0.6±0.9 logMAR) than the preoperative values (1.8±1.1 logMAR) (p<0.001). After surgery, BCVA was stable in 4 eyes (18.2%), increased in 16 eyes (72.7%), and decreased in 2 eyes (9.1%). Although the mean BCVA was better in the VH group (0.3±0.34 logMAR) than the TRD group (0.9±1.1 logMAR), the difference was not statistically significant (p=0.1). Multivariable linear regression analyses revealed that final BCVA was negatively associated with preoperative or postoperative proliferative vitreoretinopathy grade C (PVR-C), preoperative retinal detachment involving the macula, postoperative neovascular glaucoma, and long preoperative duration of disease, and positively associated with preoperative BCVA. Final BCVA was not associated with preoperative retinal and disc neovascularization, rubeosis iridis, total posterior hyaloid detachment, preoperative retinal laser photocoagulation, indication of surgery, diameter of sclerotomy (20 or 23 gauge), preoperative lens status, preoperative or postoperative epimacular membrane, peroperative iatrogenic retinal breaks, postoperative hypotony, cystoid macular edema, and new or recurrent retinal detachment. The primary anatomic success rate was 81.8% and the final anatomic success rate was 90.9%. Conclusion: In Eales' disease, good visual results can be obtained with vitreoretinal surgery if the detachment area does not involve the macula and PVR-C does not develop pre- or postoperatively.

Secondary Coats' response to retinal vasculitis managed with bevacizumab.

We report a case of 22-year-old young male who presented with a 3-month history of gradual and painless decrease of vision in his right eye (RE). On ophthalmological examination, best-corrected visual acuity in his RE was counting finger close to the face. Left eye ophthalmic examination was unremarkable. RE indirect ophthalmoscopy revealed multiple telangiectatic vessels, aneurysmal dilations, extensive yellow (lipid) exudation over the posterior pole along with perivascular exudation, and multiple sclerosed vessels in peripheral retina. Fluorescein angiography also revealed progressive leakage from telangiectasias, multiple aneurysmal outpouchings, extensive capillary dropouts, and vascular communicating channels in all quadrants. Spectral-domain-optical coherence tomography of macula demonstrated marked intraretinal fluid. Based on the above findings, a diagnosis of healed retinal vasculitis with Coats'-like response was made. The patient received 4-weekly two intravitreal bevacizumab injections which resulted in stabilization of the retinal findings and improvement in visual acuity.

Vasculitis retinal / Retinal vasculitis

La vasculitis retinal es una enfermedad inflamatoria que amenaza la visión y afecta los vasos retinales (capilares, venas y arterias). Puede ocurrir como una condición idiopática o como complicación de enfermedades infecciosas y neoplásicas o en asociación con enfermedades sistémicas inflamatorias. Sus manifestaciones clínicas típicas incluyen revestimiento perivascular, envainamiento, filltración vascular y oclusión. Puede estar asociada con signos de isquemia retinal (puntos algodonosos y hemorragias intraretinales). Es clasificada en diferentes estadíos: inflamación, isquemia, neovascularización y complicaciones. En su diagnóstico es importante la biomicroscopia, la oftalmoscopia del segmento posterior y la prueba de angiografía fluoresceínica. Se presenta un paciente de 30 años con buena historia de salud anterior. El paciente comenzó con enrojecimiento del ojo izquierdo y la observación de flotadores en ambos ojos. Fue examinado y los signos clínicos fueron descritos en el examen oftalmológico. El examen del fondo de ojo con lente de 90 dioptrias y el oftalmoscopio binocular indirecto fue realizado para un cuidadoso examen. La retinografía del fondo y las imágenes del test de angiografía y otros estudios complementarios indicados permitieron conocer que presentaba una vasculopatía obliterativa retinal ideopática (Enfermedad de Eales), y el diagnóstico diferencial fue discutido. El tratamiento indicado fue la fotocoagulación con láser y evaluar la indicación de los estudios bioquímicos del cultivo del vítreo, por antígenos de leucocito humano, factor de crecimiento endotelial vascular e interleukinas que han sido observados en este examen(AU)

Retinal vasculitis is an inflammatory disease that threatens vision and affects retinal vessels (capillaries, veins and arteries). It may occur as an isolated idiopathic condition and as a complication of infective or neoplastic disorders, in association with systemic inflammatory diseases. Typical clinical manifestations including perivascular sheathing or cuffing, vascular leakage and occlusion. It may be associated with signs of retinal ischemia (cotton-wool spots and intra-retinal hemorrhage). It is classified into different stages: lnflamation, ischemia, neovascularization and complications. In the diagnosis, biomicroscopy and ophtalmoscopic of the posterior segment and fluorescein angiography test are important. A case report is presented of a 30-year-old male patient with a history of good health. In October 2015 the patient had started out with reddening of his left eye and the viewing of floaters with both eyes. Funduscopy was performed with a 90-dioptric lens and a binocular indirect ophthalmoscope. Fundus retinography, angiographic imaging and other complementary studies revealed an idiopathic obliterative retinal vasculopathy (Eales disease). The treatment indicated was photocoagulation, as well as a recommendation to evaluate the biochemical studies of the vitreous culture, since the test had found human leukocyte antigens, vascular endothelial growth factor and interleukins(AU)

[Retinal vasculopathy with cerebral leukoencephalopathy carrying TREX1 mutation diagnosed by the intracranial calcification: a case report].

A 40-year-old woman with renal dysfunction for 2 years was admitted to our hospital suffering from a headache. Family history revealed that her mother had a headache, renal dysfunction, and brain infarction in younger age. She had a retinal hemorrhage, a retinal atrophy, pitting edema in her lower extremities. Her neurological findings were unremarkable. Brain imaging showed multiple white matter lesions accompanied with calcifications and slightly enhancement. Kidney biopsy showed the thrombotic microangiopathy, Gene analysis demonstrated a causative mutation in three-prime repair exonuclease-1 (TREX1) gene, c.703_704insG (p.Val235GlyfsX6), thereby we diagnosed her as retinal vasculopathy with cerebral leukoencephalopathy (RVCL). RVCL is an autosomal dominant condition caused by C-terminal frame-shift mutation in TREX1. TREX1 protein is a major 3' to 5' DNA exonuclease, which are important in DNA repair. While TREX1 mutations identified in Aicardi-Goutieres syndrome patients lead to a reduction of enzyme activity, it is suggested that mutations in RVCL alter an intracellular location of TREX1 protein. There are no treatments based evidences in RVCL. We administered cilostazol to protect endothelial function, and her brain lesions and renal function have not become worse for 10 months after. It is necessary to consider RVCL associated with TREX1 mutation if a patient has retinal lesions, white matter lesions accompanied with calcifications, and multiple organ dysfunction.

A rare presentation of exudative macroaneurysms in unilateral Eales' disease.

BACKGROUND: A case of exudative macroaneurysms in unilateral Eales' disease is reported. 'Eales disease' is an idiopathic bilateral occlusive periphlebitis with neovascularisation and mostly presents with vitreous hemorrhage. CASE: A 30 year old male presented with diminution of vision in left eye (6/36) for 1 month. Slit Lamp examination of both eyes revealed normal anterior segment. Fundus examination of left eye with + 90 D lens revealed reddish lesions at the macula with surrounding circinate exudation. On peripheral examination hemorrhages were seen along with vasculitis in the superotemporal quadrant.The right eye fundus was normal.All laboratory investigations were found within normal limits. CONCLUSION: Eales' disease is mostly a bilateral condition but this case is rare as there is uniocular involvement with exudative macroaneurysms.Laser therapy was instituted which was effective in management of this condition with restoration of normal visual acuity.There was no relapse on follow up for 2 years.

Frosted branch angiitis and cerebral venous sinus thrombosis as an initial onset of neuro-Behçet's disease: a case report and review of the literature.

BACKGROUND: Frosted branch angiitis is a rare, severe condition. It can be either a primary or a secondary condition and is characterized by rapid deterioration of vision and fulminant retinal vasculitis that manifests as diffuse sheathing of retinal vessels, macular edema, papillitis, vitritis and anterior uveitis. We aimed to describe a case of frosted branch angiitis and cerebral venous sinus thrombosis as an initial neuro-Behçet's disease onset. Diagnosis of Behçet's disease was based on the current 2014 International Criteria for Behçet's Disease and the International consensus recommendation criteria for neuro-Behçet's disease. In addition, a literature review using search parameters of "frosted branch angiitis", "Behçet" and "neuro-Behçet" in the PubMed database is presented. CASE PRESENTATION: A 28-year-old Brazilian pardo woman presented to our hospital with abrupt bilateral vision loss associated with recurrent aphthous oral ulcers 6 months before visual symptom onset. A fundus examination showed bilateral widespread retinal vasculitis with venous and arterial white sheathing, optic disc swelling, macular edema, and retinal hemorrhages, leading to the diagnosis of frosted branch angiitis. An extensive systemic workup for retinal vasculitis was uneventful, except for brain magnetic resonance imaging demonstrating cerebral venous sinus thrombosis and lymphocytic aseptic meningitis. A diagnosis of neuro-Behçet's disease was made, and treatment was started with methylprednisolone therapy 1 g/day for 5 consecutive days, followed by oral mycophenolate mofetil and infliximab 5 mg/kg infusion. The patient's response was rapid, with improvement of visual acuity to hand movement and counting fingers by day 7 and final visual acuity of counting fingers and 20/130. CONCLUSIONS: Frosted branch angiitis may be associated with infectious, noninfectious, or idiopathic causes. An extensive workup should be done to exclude systemic vasculitis such as Behçet's disease. Treatment with systemic steroids must be promptly initiated in association with specific treatment aimed at inflammation control and blindness risk reduction.