[Vasculitis of the retina : Clinical aspects, diagnostics, management and treatment]. / Vaskulitis der Retina : Klinik, Diagnostik, Management und Therapie.

Inflammatory changes in the retinal vessels can be attributed to a wide range of etiologies. These include infections, intraocular and systemic autoimmune processes, general diseases and iatrogenic factors. As the endothelium of the retinal capillaries forms the inner blood-retinal barrier, a disruption of this structure is directly associated with consequences for the fluid electrolyte balance of the retina. Clinical sequelae can include leakage of the retinal vessels and macular edema, which are often functionally threatening and significantly reduce the quality of life of patients. As the eye can be affected as an "index organ", a work-up of the patient by the ophthalmologist is of great importance. In the age of "precision medicine", efforts are being made to gain new insights into the pathogenetic mechanisms of vasculitis through "omics" in order to develop innovative treatment concepts.

IRVAN syndrome: A retrospective review of 9 cases from Tunisia.

PURPOSE: To describe clinical features, relevant imaging findings, disease course, and response to treatment in 9 patients (18 eyes) with idiopathic retinal vasculitis, aneurysms, and neuroretinitis (IRVAN) syndrome. METHODS: Retrospective review of the charts of nine patients (18 eyes) diagnosed with IRVAN syndrome at Fattouma Bourguiba University Hospital, Monastir, Tunisia, from January 1, 2011 to January 1, 2022. RESULTS: Nine patients were included with bilateral involvement in all cases. Mean initial best-corrected visual acuity (VA) was 20/32 (range, 20/1600-20/20). Clinical findings at presentation included vitreous cells (10 eyes, 55.6%), peripapillary exudates (12 eyes, 66.7%), partial or complete macular star (11 eyes, 61.1%), and vascular sheathing (11 eyes, 61.1%). Fluorescein angiography showed arteriolar aneurysms (18 eyes, 100%), areas of peripheral capillary non-perfusion (16 eyes, 88.9%), and retinal neovascularization (6 eyes, 33.3%). Optical coherence tomography showed macular edema in 5 eyes (27.8%). Optical coherence tomography angiography of the optic disc demonstrated papillary aneurysms in 4 eyes of 2 patients. Indocyanine green angiography showed retinal arteriolar aneurysmal dilatations in 4 eyes of 2 patients. Ten eyes (55.6%) had stage 2 disease, 6 eyes (33.3%) had stage 3, and 2 eyes (11.1%) had stage 1. Treatment modalities included peripheral photocoagulation (16 eyes, 88.9%), intravitreal bevacizumab (4 eyes, 22.2%), and intravitreal triamcinolone acetonide (1 eye, 5.6%). Mean final best-corrected VA was 20/32 (range, 20/600-20/20). Ocular complications included vitreous hemorrhage in 3 eyes (16.7%), branch retinal artery occlusion in 2 eyes (11.1%) and submacular fibrosis in 3 eyes (16.7%). CONCLUSION: IRVAN syndrome should be highly suspected in patients with peripapillary exudates associated with vascular sheathing and vitreous cells. Multimodal imaging confirms the diagnosis by showing retinal macroaneurysms. Early treatment of macular edema and/or peripheral retinal non-perfusion is mandatory to improve prognosis.

Occlusive retinal vasculitis: novel insights into causes, pathogenesis and treatment.

PURPOSE OF REVIEW: Occlusive retinal vasculitis (ORV) has a large differential diagnosis and varied therapeutic approaches. This review highlights existing and novel causes and treatment options for ORV. RECENT FINDINGS: Mutations in CAPN5, TREX1, and TNFAIP3 have been associated with dominantly inherited forms of ORV. Various intraocular therapeutics have been shown to occasionally cause ORV; the most recent medications associated with ORV are vancomycin and brolucizumab. In cases of ORV linked to Behçet's disease, clinical trials support the use of tumor necrosis factor alpha inhibitors. SUMMARY: Identification of the underlying etiology of ORV is critical to help guide treatment. Treatment in ORV involves both treatment any underlying infection or autoimmune condition, cessation of the any offending causative agent and local treatment of neovascular complications.

HEMORRHAGIC OCCLUSIVE RETINAL VASCULITIS AFTER INTRACAMERAL VANCOMYCIN USE IN CATARACT SURGERY AFTER INTRAVENOUS EXPOSURE.

PURPOSE: To report a case of hemorrhagic occlusive retinal vasculitis after cataract surgery. METHODS: A 74-year-old woman presented with blurry vision and distorted vision, which started 2 days after an uncomplicated cataract surgery in the left eye. Intracameral vancomycin was injected during the case. The patient reported being treated with systemic vancomycin in the past. RESULTS: The visual acuity was 20/80 in the left eye. She had trace cells in the anterior chamber with no hypopyon and intraocular lens implant within the capsular bag in the left eye. Dilated fundus examination revealed no vitritis, There were large patches of peripheral retinal hemorrhages and retinal ischemia. The patient was diagnosed with hemorrhagic occlusive retinal vasculitis likely secondary to hypersensitivity reaction to intracameral vancomycin. The patient was started on oral prednisone, and the topical difluprednate course was escalated. Within 3 weeks, vision improved to 20/30 in the left eye. She underwent pan retinal photocoagulation targeting the ischemic areas in the periphery. CONCLUSION: The patient had previous exposure to systemic vancomycin, which may have sensitized her immune system. Later on, the hypersensitivity reaction took place after exposure to intracameral vancomycin during cataract surgery. Our hemorrhagic occlusive retinal vasculitis case had a favorable visual outcome, and recognition of this entity will ensure that vancomycin will not be used for infection prophylaxis in the fellow eye at the time of cataract surgery.

TREATMENT OF IDIOPATHIC RETINAL VASCULITIS, ANEURYSMS, AND NEURORETINITIS (IRVAN) WITH PHOTOCOAGULATION IN COMBINATION WITH SYSTEMIC IMMUNOSUPPRESSION.

BACKGROUND: Idiopathic retinal vasculitis, aneurysms, and neuroretinitis syndrome is a very rare primary retinal vasculitis. It seems to evolve in stages, where there is initially a florid vasculitis associated with aneurysm formation. Neuroretinitis and macula edema are common features. Subsequently, retinal ischemia ensues, leading to neovascularization. If untreated, further sight-threatening complications occur, including traction retinal detachment and secondary glaucoma. METHODS AND RESULTS: Here, we describe a patient with early idiopathic retinal vasculitis, aneurysms, and neuroretinitis syndrome, who was treated with a combination of systemic immunosuppression and localized photocoagulation. There was substantial regression of the aneurysms and improvement of the macular edema. Treatment of the disorder should be based on the clinical stage and complications of the disorder. CONCLUSIONS: As shown here, a multidisciplinary approach can be very helpful in managing patients with this severe sight-threatening disorder.

Wide-field angiography for diagnosis and follow-up of Eales disease: a case series / Angiografia de amplo campo para diagnóstico e monitoramento da doença de Eales: série de casos

ABSTRACT Wide-field angiography enables assessing peripheral areas with better quality and gives greater deep focus, which improves the image periphery. Some studies have proposed the usefulness of these angiographic systems in inflammatory diseases of the retina. However, few studies have evaluated this technique in Eales disease. We present a case series in which 5 eyes of 3 patients with Eales disease were evaluated by using retinal fluorescein angiography with 30º, 50º, and 150º lenses in a laser-scanning ophthalmoscope. These cases highlight the usefulness of wide-field fluorescein angiography in the diagnosis and follow-up of peripheral ischemic retinal areas in Eales disease, which enables better follow-up than possible with conventional fluorescein angiography images.

RESUMO A angiografia de campo amplo permite avaliar áreas periféricas com melhor qualidade e proporciona maior foco profundo, melhorando a imagem da periferia. Alguns estudos têm proposto a utilidade desses sistemas angiográficos nas doenças inflamatórias da retina. No entanto, poucos estudos avaliaram esta técnica na doença de Eales. Apresentamos uma série de casos em que 5 olhos de 3 pacientes com doença de Eales foram avaliados usando angiografia de fluoresceína da retina com lentes de 30º, 50º e 150º em um oftalmoscópio de varredura a laser. Esses casos destacam a utilidade da angiografia com fluoresceína de campo amplo no diagnóstico e no acompanhamento das áreas isquêmicas periféricas da retina na doença de Eales, permitindo um melhor acompanhamento do o possível com imagens por angiofluoresceinografia convencional.

Wide-field angiography for diagnosis and follow-up of Eales disease: a case series.

Wide-field angiography enables assessing peripheral areas with better quality and gives greater deep focus, which improves the image periphery. Some studies have proposed the usefulness of these angiographic systems in inflammatory diseases of the retina. However, few studies have evaluated this technique in Eales disease. We present a case series in which 5 eyes of 3 patients with Eales disease were evaluated by using retinal fluorescein angiography with 30º, 50º, and 150º lenses in a laser-scanning ophthalmoscope. These cases highlight the usefulness of wide-field fluorescein angiography in the diagnosis and follow-up of peripheral ischemic retinal areas in Eales disease, which enables better follow-up than possible with conventional fluorescein angiography images.

[Retinal vasculitis and systemic diseases]. / Vascularites rétiniennes et maladies systémiques.

Retinal vasculitis (RV) is an inflammation of retinal blood vessels that can be associated with uveitis or be isolated, and can induce vascular occlusion and retinal ischemia. Visual acuity can be severely affected in case of macular involvement or neovessel formation. The diagnosis relies on fundoscopy and fluorescein angiography. Systemic diseases may be associated with RV, the most frequently encountered are Behçet's disease, sarcoidosis or multiple sclerosis, all predominantly associated with venous involvement, whereas systemic lupus erythematosus and necrotizing vasculitis are less frequently observed and predominantly associated with arterial or mixed vasculitis. Treatments are usually aggressive in order to preserve a good visual acuity and to reduce retinal inflammation and chronic ischemia. Steroids, immunosuppressive drugs, retinal laser photocoagulation, intravitreal anti-VEGF injections are usual treatments and more recently, anti-TNFalpha monoclonal therapeutic antibodies have been shown to be very successful.

Pediatric Eales Disease: An Indian Tertiary Eye Center Experience.

PURPOSE: To report the clinical profiles, etiologies, treatment modalities, and outcomes for Eales disease in patients younger than age 16 years in India. METHODS: Retrospective review of medical records of patients with Eales disease who had a minimum 5-year follow-up period. RESULTS: A total of 25 eyes of 13 patients were included. Of these 13 patients, 12 (94%) had bilateral Eales disease and 11 (84.6%) were men. Mean patient age was 14.1 years (range: 11 to 16 years). Diminution of vision (36%) was the most common presenting complaint, followed by both diminutions of vision and floaters (32%). Sclerosed vessels were seen in all eyes, and 21 (84%) eyes had active periphlebitis at presentation. Neovascularization elsewhere was seen in 20 (80%) eyes and neovascularization of the optic disc was seen in 1 (4%) eye. Veno-venous shunts were found in 12 (48%) eyes, and 18 (72%) eyes had vitreous hemorrhage. All eyes received photocoagulation; 84.6% of patients received oral steroids, with 7.7% of patients treated with azathioprine and 38.4% treated with anti-tubercular therapy. Vitrectomy was performed in 36% of eyes for non-clearing vitreous hemorrhage and tractional retinal detachment. Vision improved in 7 (28%) eyes, was stable in 12 (48%) eyes, and worsened in 6 (24%) eyes. Recurrence of the disease more than five times during the 5-year follow-up period occurred in 20% of patients. CONCLUSIONS: Recurrent vasculitis and vitreous hemorrhage in children should raise the suspicion of pediatric Eales disease. [J Pediatr Ophthalmol Strabismus. 2018;55(4):270-274.].

Long-Term Outcomes of a Large Cohort of Patients with Eales' Disease.

PURPOSE: To analyze the long-term visual outcomes of Eales' patients. METHODS: A retrospective review of Eales' patients diagnosed between 1985 and 1995 with >10 years follow-up done. RESULTS: We included 500 patients (898 eyes) with mean follow-up duration of 15.8 years (10-25 years), and 81% patients had bilateral disease. Patients using oral steroid during acute stage disease had significantly better visual outcome at the final visit [0.42 logMar(6/18) ± 0.723 logMar(6/30)] compared to patients who did not receive oral steroids [(0.5907 logMar(6/24) ± 0.945 logMar(6/48)] (p = 0.004). Patients who received laser therapy had significantly better visual outcome at the final visit [(0.415 logMar(6/18) ± 0.66 logMar(6/30)] compared to those who did not undergo laser treatment [(0.9237 logMar(6/48) ± 1.31 logMar(6/120)] (p < 0.001). Poor presenting visual acuity and late stage of presentation were associated with poor visual outcome. Fifty-two percent of eyes had <5 recurrences over 10 years. CONCLUSION: Eales' disease is a bilateral recurrent retinal vasculitis disorder and timely treatment with steroid and/or laser photocoagulation result in better visual outcomes.

Dificultades en el manejo de los hemangiomas capilares retinianos asociados a enfermedad de von Hippel-Lindau / Difficulties in the management of retinal capillary haemangiomas associated with von Hippel Lindau disease

Caso clínico: Mujer de 29 años con hemangiomas capilares retinianos (HCR) bilaterales. Ante la sospecha clínica de enfermedad de von Hippel Lindau (VHL), se realizan estudios genéticos que son negativos en 2 ocasiones. Se produce progresión de los HCR del ojo izquierdo, con aparición de membrana epimacular, que son tratados con láser, ranibizumab intravítreo y vitrectomía. Finalmente, el tercer estudio genético detecta una mutación de novo en el gen VHL que permite confirmar el diagnóstico. Discusión: El síndrome de VHL causa una enfermedad ocular compleja y de difícil diagnóstico que requiere tratamiento precoz de los HCR para modificar el pronóstico visual (AU)

Clinical case: A 29-year-old female with bilateral retinal capillary haemangiomas (RCH). A genetic analysis was carried out due to the suspicion of von Hippel-Lindau (VHL) disease, with negative results on 2 occasions. There was progression of the RCH in the left eye, leading to a macular epiretinal membrane. The patient was treated with laser, intravitreal ranibizumab, and vitrectomy. Finally, a third genetic test detected a de novo mutation in the VHL gene, and led to the genetic diagnosis. Discussion: VHL syndrome causes a complex ocular disease with a difficult diagnosis that requires early treatment of the RCH in order to modify its visual prognosis (AU)

Ultra-widefield fundus fluorescein angiography in the diagnosis and management of retinal vasculitis.

PurposeTo quantify the additional information provided by ultra-widefield fluorescein angiography, compared with 7-field standard imaging, in patients with retinal vasculitis (RV).Patients and methodsRetrospective case series of 106 patients.ResultsRetinal vascular pathology was identified by UWF FFA, but not by standard ETDRS 7-field area, in 62 patients (58.5%) and in 79 eyes (43.4%). The pathology included active RV (47 eyes, 25.8% of eyes); retinal ischaemia, or infarction (53 eyes, 29.1%); and retinal neovascularization (7 eyes, 3.8%). A change to management was made in 36 patients (34%). Of these, 21 (20% of all patients undergoing angiography) were made after the identification of retinal vascular pathology, which was found only on UWF FFA, outside the ETDRS area.ConclusionUltra-widefield fluorescein angiography has clear advantages over standard multi-field imaging. It is currently the standard method of assessment for RV in this centre.