Peripheral Retinal Vasculitis Research Articles

Peripheral occlusive retinal vasculitis and associated hemi-central retinal vein occlusion in the same eye with suspected neuroretinitis: A rare case report

We describe a case of an 18-year-old male with decreased vision in the left eye (LE) over two weeks. At presentation, his uncorrected visual acuity was 6/6 in the right eye and light perception (PL+ve) in the LE. On ophthalmic examination, both eye’s anterior segments were unremarkable except a relative afferent pupillary defect in the LE. Posterior segment of the LE revealed peripheral active occlusive retinal vasculitis and hemi-central retinal vein occlusion superiorly along with suspected neuroretinitis. The diagnosis was confirmed with fundus fluorescein angiography and optical coherence tomography macula. He was not known to have any systemic illness previously and after relevant systemic investigations, he was treated empirically with intravenous methylprednisolone, oral steroids, anti-tubercular therapy, and anti-toxoplasma therapy. Retinal laser photocoagulation was done to the capillary non-perfusion areas. After three months from the day of presentation, visual acuity improved to 6/6 (partial), N6 in his LE.

Multiple sclerosis-associated uveitis - A series from South India.

To report clinical profiles of multiple sclerosis (MS)-associated uveitis in seven cases from a single tertiary eye care center in South India. Retrospective case series 2013-2023. Seven cases of MS-associated uveitis were retrieved from our databases. There were five females and two males. The diagnosis of MS was made by the neurologist in all cases. Bilaterality was seen in all cases. Intermediate uveitis was the most common presentation (five cases). It was associated with peripheral retinal vasculitis (two cases) and disc pallor (two cases). Fuchs heterochromic iridocyclitis (one case) and incomplete Vogt-Koyanagi-Harada (VKH)-like presentation with a peripapillary choroidal neovascular membrane (one case) were the other presentations. In the case with incomplete VKH-like presentation, whole genome sequencing revealed a heterozygous non-synonymous variation (c.1228C>T) in exon 10 of TNFRSF1A, suggestive of susceptibility to multiple sclerosis 5 due to mutation in the TNFRS1A gene on chromosome 12p13.31. All cases received systemic steroids. Azathioprine (three cases) and rituximab (three cases) were the commonly used immunomodulatory drugs. The visual outcome was good in all cases at the last follow-up. MS-associated uveitis is underreported in India. This series highlights the clinical profile of MS-associated uveitis in India.

Different Cases, Different Manifestations of Post-COVID-19 Retinal Artery Occlusion: A Case Series.

Coronavirus disease 2019 (COVID-19) is a procoagulant disease that increases the risk of clinically evident thrombotic complications. Herein we present 3 cases with different retinal artery occlusions that emerged soon after the diagnosis of COVID-19. The first patient had central retinal artery occlusion (CRAO) that resulted in visual loss in one eye. The second patient had inflammatory peripheral retinal artery occlusion, vasculitis, and uveitis which did not affect vision. The third patient presented with CRAO following the progression from orbital cellulitis to orbital apex syndrome. Interestingly, CRAO progressed to internal carotid artery occlusion in this case within days and resulted in monocular visual loss. Variations in the underlying pathophysiology and the characteristics of individual immune responses in patients with COVID-19 may be factors that determine differences in clinical manifestations. This article aims to describe different presentations of COVID-19-related retinal artery occlusions and discuss possible pathophysiological aspects.

Ophthalmic Manifestations of ANCA-Associated Vasculitis

Ocular manifestations in antineutrophil cytoplasmic antibody-associated vasculitis can be associated with the general or limited form of disease and can even occur in the absence of systemic disease. Ocular manifestations of associated vasculitis can be the first symptom of previously not manifested or undiagnosed systemic disease, allowing ophthalmologists to contribute to the diagnosis. Although its ocular findings are variable and nonspecific, the presence of necrotizing changes and peripheral corneal involvement accompanying scleral inflammation are important clues suggesting systemic vasculitis, especially associated vasculitis. The disease may affect all layers of the eye; scleritis and orbital involvement being the most common. Conjunctivitis, episcleritis, peripheral ulcerative keratitis, uveitis and retinal vasculitis are other ocular findings that may be observed during the disease course.
 Ocular involvement is most commonly seen in granulomatosis with polyangiitis followed by eosinophilic granulomatosis with polyangiitis and microscopic polyangiitis. Due to the high morbidity and mortality of associated vasculitis, it is very important to recognize the ocular manifestations of vasculitis as a sign of the underlying systemic disease and an indicator for the disease activity. Treatment varies depending on the location and severity of the ocular involvement. Although localized medical and surgical treatments can help to manage associated ocular disease, systemic immunosuppressive medications are often required to control the underlying disease. With the increasing availability and use of biological agents, prognosis has improved in patients with severe ocular complications. Rituximab appears to be useful in inducing remission and controlling relapses in patients with ocular involvement of associated vasculitis, particularly in cyclophosphamide resistant cases. A multidisciplinary approach in the diagnosis, treatment and follow-up of patients with associated vasculitis is required in order to achieve successful results.

Pediatric Patient with Pars Planitis Associated with Lyme Disease: A Rare Presentation: Case Report

Lyme-associated uveitis is more common in adults, but very rare in children. Ophthalmological symptoms are seen in a minority of Lyme cases. Uveitis is seen in less than 1% and different clinical features can be seen. For the diagnosis of Lyme-associated uveitis, positive ELISA, immunoblot serology, exclusion of other causes by various tests in differential diagnosis, efficacy of antibiotics after primary resistance to steroid therapy alone, tick bite, living in an endemic area and the presence of systemic clinical symptoms should be investigated. In our report, we aimed to present a very rare Lyme disease-related intermediate uveitis in an 11-year-old male patient who had a history of tick bite 4 months ago and was diagnosed with pars planitis and peripheral retinal vasculitis. Recurrence was observed after being asymptomatic for 6 months after the treatment. However, our case was successfully treated with a new course of antibiotics in addition to steroid therapy.

Peripheral retinal vasculitis in chickenpox: A case report

Peripheral retinal vasculitis in chickenpox: A case report

Early anti-VEGF treatment for hemorrhagic occlusive retinal vasculitis as a complication of cataract surgery

BackgroundWe report a case of hemorrhagic occlusive retinal vasculitis (HORV) after prophylactic intracameral vancomycin use during an uneventful cataract surgery treated with early anti-VEGF treatment.Case presentationA 51-year-old female underwent uneventful cataract surgery with prophylactic intracameral vancomycin during the procedure. On the seventh post-operative-day, she presented with sudden painful, visual loss. Fundus examination revealed peripheral hemorrhagic retinal vasculitis. She received anti-VEGF therapy to prevent further vision loss and retinal neovascularization due to extensive retinal ischemia. At the 6-month follow-up visit, visual acuity was 20/20 with no sign of neovascularization.ConclusionsPostoperative HORV is a devastating condition that can occur after otherwise uncomplicated cataract surgery. The nature of this rare condition remains unknown. Early anti-VEGF administration seems to demonstrate favorable results.

PERIPHERAL RETINAL VASCULITIS: Analysis of 110 Consecutive Cases and a Contemporary Reappraisal of Tubercular Etiology.

Describe the clinical features and outcomes of patients with peripheral retinal vasculitis (RV) and describe clinical characteristics of presumed tubercular RV in a nonendemic setting. Retrospective cohort study of 110 consecutive patients with peripheral RV at a tertiary referral eye care center in the United Kingdom. Retinal vasculitis was defined as RV with vitritis associated with peripheral retinal ischemia. Patients who also had positive Quantiferon Gold in Tube test, positive tuberculin skin test, and/or other evidence of systemic tuberculosis such as biopsy were labeled with presumed tubercular RV. Treatment success was defined as resolution of inflammation, and successful tapering of oral corticosteroids to less than 10 mg/day or topical steroids to less than twice a day. Mean age of the study population was 42.69 ± 14.95 years. Patients were predominantly Asian (49.1%) and Male (67.0%). A total of 73.2% had bilateral involvement. Sixty-nine (62.72%) patients had presumed tubercular RV. A total of 52.8% patients received antitubercular therapy, 65.5% received oral corticosteroids, and 17.3% required steroid-sparing oral immunosuppressive agents. A total of 85.19% of patients with presumed tubercular RV achieved treatment success with concurrent antitubercular therapy as against 75.61% of patients with nontubercular RV. This is the largest study of the epidemiology, clinical features, and outcomes of both peripheral RV and presumed tubercular RV to date. Presumed tubercular RV commonly seems to affect young males of Asian descent and had vitreous hemorrhage as common clinical findings and also demonstrated a good treatment outcome with antitubercular therapy.

A Case of Lyme Disease Accompanied by Uveitis and White Dot Syndrome.

In this case report we aimed to present a case of Lyme disease presenting as peripheral retinal vasculitis, intermediate uveitis and multifocal white dots in the posterior pole. The patient exhibited vitritis and snowball opacities in both eyes. A diagnosis of Lyme disease was made based on clinical, angiographic and laboratory findings. Fundus fluorescein angiography revealed optic nerve and retinal venous leakage as well as multiple hyperfluorescent foci in both eyes. The patient’s symptoms and ocular findings significant improved after treatment with a combination of systemic antibiotics and steroids. Ophthalmologists should bear in mind that conditions presenting with uveitis and multifocal white dots may be related to Lyme disease.

Bilateral intermediate uveitis with appearance of frosted branch angiitis and association with Mycoplasma pneumoniae infection: case report and review of the literature

A 12-year-old girl presented with acute bilateral visual loss following a mild upper respiratory tract infection. Clinical examination revealed bilateral severe peripheral retinal vasculitis with an appearance of frosted branch angiitis and cystoid macular edema. All tests for etiological diagnosis were negative, apart from positive IgM and IgG antibodies for Mycoplasma pneumoniae. She was treated with broad-spectrum antibiotics and intravenous corticosteroids. She responded to treatment, with dramatic improvement of her visual acuity, remission of angiitis, and a residual macular star formation. This is the first reported case of bilateral intermediate uveitis possibly associated with Mycoplasma pneumoniae infection.

Efeito terapêutico de Ranibizumabe em edema macular e perfusão retiniana associado a raro caso de Eales Central

Eales disease is an idiopathic peripheral vasculopathy involving the retina that primarily affects healthy young men. The clinical manifestations consist of three characteristic findings: peripheral retinal vasculitis, capillary nonperfusion, and neovascularization of the retina and the optic disc. Few case studies describe the rare form of central involvement in the disease, known as central Eales disease. In it, the typical findings of Eales disease occur in the posterior pole. Eales disease treatment depends on the clinical stage of the disease. Anti-VEGF therapy has recently been used. This report describes the case of a healthy young man with this rare form of Eales disease and the favorable outcome of intravitreal therapy with ranibizumab used in combination with panretinal photocoagulation. Satisfactory clinical improvement was observed in the eye that underwent intravitreal anti-VEGF treatment with ranibizumab, particularly in terms of the reduction in retinal thickness in the macular region and improvement in visual acuity.

Long-term follow-up of herpetic non-necrotizing retinopathy with occlusive retinal vasculitis and neovascularization.

BackgroundHerpetic necrotizing retinitis is a well-recognized entity. A few cases of herpetic non-necrotizing retinitis were previously reported.FindingsWe retrospectively report two cases of herpetic non-necrotizing retinopathy with a long follow-up. A 19-year-old woman presented with a bilateral diffuse occlusive retinal vasculitis, peripheral neovascularization, and no signs of retinal necrosis. Long-lasting immunosuppressive treatment failed to control the vasculitis until herpes simplex virus type 1 (HSV1) was demonstrated by polymerase chain reaction (PCR) in the aqueous. Acyclovir was then added and immunosuppressive tapered and eventually stopped resulting in a resolution of vasculitis. Only two relapses occurred during the next 6 years and responded rapidly to oral acyclovir.An 11-year-old boy presented with unilateral scar of stromal keratitis, severe vitritis, and optic disc neovascularization, followed 6 weeks later by peripheral occlusive retinal vasculitis. Therapeutic and diagnostic vitrectomy was performed, and PCR was found to be positive for varicella zoster virus (VZV) in a vitreous specimen. The inflammation responded to oral acyclovir therapy. Recurrence of anterior uveitis with iris depigmentation occurred 4 months after treatment was arrested. After 4 years, he presented again with a recurrence of anterior inflammation and cystoid macular edema (CME). No sign of inflammation was seen for the next 10 years.ConclusionsThese rare cases support the possible role of herpes viruses (HSV or VZV) in occlusive vasculitis without retinal necrosis. PCR was useful to raise the diagnosis and to adapt the treatment. A good response was obtained on oral antiviral therapy.

Pars Planitis: Epidemiology, Clinical Characteristics, Management and Visual Prognosis.

Pars planitis is an idiopathic chronic intermediate uveitis which predominantly affects children and adolescents, and accounts for 5-26.7% of pediatric uveitis. Although an autoimmune process with a genetic predisposition has been suggested, its etiology still remains unknown. The most common presenting symptoms are floaters and blurred vision. Diffuse vitreous cells, haze, snowballs and snowbanks are typical findings of pars planitis. Peripheral retinal vasculitis, optic disc edema and anterior segment inflammation are other well-known findings. Although pars planitis is known to be a benign form of uveitis in most cases, it may become a potentially blinding disease due to complications including cataract, cystoid macular edema, vitreous opacities and optic disc edema. Cystoid macular edema is the most common cause of visual morbidity. Band keratopathy, epiretinal membrane formation, vitreous condensation, neovascularizations, vitreous hemorrhage, retinal detachment, cyclitic membranes, glaucoma and amblyopia may develop as a consequence of the chronic course of the disease. Exclusion of infectious and non-infectious causes which may present with intermediate uveitis is of utmost importance before starting treatment. Treatment of pars planitis has been a controversial issue. There is no consensus specifically for treatment of cases with minimal inflammation and relatively good visual acuity. However, current experience shows that pars planitis may cause severe inflammation and needs an aggressive treatment. A stepladder approach including corticosteroids, immunosupressive agents, anti-tumor necrosis factor-alpha and pars plana vitrectomy and/or laser photocoagulation is the most commonly used method for treatment of pars planitis. Adequate control of inflammation and prompt detection of associated complications are crucial in order to improve the overall prognosis of the disease.

Unusual presentation of a multiple sclerosis case involving central retinal artery occlusion

The term intermediate uveitis (IU) refers to a subgroup of uveitis in which the vitreous is the site of greatest inflammation. Patients with multiple sclerosis (MS) have a greater frequency of IU compared with the general population.The IU associated with MS is characterized by the presence of pars planitis (occasionally accompanied by anterior uveitis) and the presence of peripheral retinal vasculitis in the form of periphlebitis (venous sheathing) in 6–26% of patients.We present a patient with an unusual initial presentation of MS involving central retinal artery occlusion (CRAO) in the right eye (RE).Although retinal vascular changes are asymptomatic in the majority of MS patients, the spectrum of impairment ranges from simple peripheral retina periphlebitis to the presence of peripheral occlusive retinal vasculitis in 6.5% of patients.This atypical case may represent an extreme of the spectrum of retinal vasculitis associated with demyelinating disease.

Role of ultra-widefield fluorescein angiography in the management of uveitis

ObjectiveTo discuss and illustrate the role of ultra-widefield fluorescein angiography (UWFFA) in the diagnosis and management of peripheral retinal vasculitis. DesignRetrospective case series. ParticipantsFour consecutive patients in whom UWFFA showed far peripheral vasculitis were included. All patients were seen between May 2011 and May 2012 at the Doheny Eye Institute. MethodsConventional fluorescein angiogram (FA) images or areas determined by the Early Treatment of Diabetic Retinopathy Study Group protocol for imaging the posterior pole and peripheral retina were superimposed on the UWFFA images. The ability to detect the extent and severity of vasculitis and vascular occlusion using both conventional FA and UWFFA was compared by 2 investigators, and any discrepancies were adjudicated by a third investigator. ResultsIn none of the cases was the full extent of vasculitis and capillary occlusion visible in the fields normally portrayed by conventional FA. In contrast, capillary nonperfusion and peripheral vasculitis were detectable by UWFFA in all cases. In 2 cases, the posterior extent of vasculitis could have been detected by conventional FA. ConclusionsDetection and depiction of the extent and severity of peripheral vascular changes are enhanced with UWFFA, aiding in the management of vasculitis in the retinal periphery.

Retinal Vasoproliferative Tumors

To compare the clinical features of primary vs secondary retinal vasoproliferative tumors (VPTs). Retrospective case series of 334 tumors in 295 eyes of 275 patients. Of 275 patients with VPT, 41% (n = 113) were male and 59% (n = 162) were female, with a mean age of 44 years at presentation. Primary VPT occurred in 80% (n = 219) and secondary VPT, in 20% (n = 56) of patients. Secondary VPT (n = 67) occurred in eyes with retinitis pigmentosa (n = 15, 22%), pars planitis (n = 14, 21%), Coats disease (n = 11, 16%), previous retinal detachment surgery (n = 8, 12%), idiopathic peripheral retinal vasculitis (n = 4, 6%), familial exudative vitreoretinopathy (n = 3, 4%), and others (n = 12, 18%). The mean interval between diagnosis of underlying ocular condition and secondary VPT was 160 months. Statistically significant differences (P < .05) in clinical features (primary vs secondary VPTs) included mean age at presentation (46 vs 38 years), visual symptoms (74% vs 87%), poor visual acuity worse than 20/200 (15% vs 28%), bilaterality (4% vs 20%), multifocality (5% vs 15%), postequatorial tumor location (20% vs 33%), tumor basal dimension (6 vs 7 mm), anterior chamber cells (16% vs 30%), and vitreous cells (19% vs 48%). Retinal vasoproliferative tumor can be primary (80%) or secondary (20%). Compared with primary VPT, secondary VPT is more often bilateral, multiple, and larger and occurs at an earlier age associated with poorer visual acuity.

Boston Keratoprosthesis (Type I): visual prognosis and complications

AbstractPurpose to describe the functional results and sight threatening post‐operative complications of Type I Boston Keratoprosthesis.Methods retrospective descriptive study of Boston Keratoprosthesis patients from two European eye centers.Results A total of 66 eyes were included for analysis. Mean follow‐up time was 28 months. Majority of patients had more than 2 penetrating keratoplasties, primary clinical diagnosis are presented. Mean preoperative best corrected visual acuity was 2.05, mean best ever achieved post‐operative best corrected visual acuity was 1.16 and final mean postoperative best corrected visual acuity was 1.47 . The most common sight‐threatening complications were as follows: retroprosthetic membrane, retinal/choroidal detachment, infectious keratitis, endophthalmitis, peripheral retinal occlusive vasculitis and prosthesis extrusion. Management of complications are discussed.Conclusion 95 % retention rate with overall improvement in visual acuity demonstrates that Type I Boston Keratoprosthesis is a good alternative to cases of multiple graft failure due to various primary clinical diagnoses. Complications must be detected early on and managed accordingly to ensure success of the procedure.

Acute retinal necrosis. 3 case reports

AbstractPurpose To present three case reports of acute retinal necrosis (ARN) syndrome and discuss the aspects of diagnosis, treatment and prognosis.Methods Retrospective analysis of clinical, laboratory, photographic and angiographic records of three immunocompetent patients with acute retinal necrosis syndrome.Results Two male 22 and 36 years old and one female 25 years old patients had typical clinical symptoms of ARN syndrome: sudden decrease of vision acuity, eye pain, multiple areas of peripheral retinal necrosis, occlusive vasculitis, opticopathia, anterior uveitis and vitritis. Serum Ig G antiviral (VZV, HSV, CMV) titers were the basis for the etiological diagnosis. After applying systemic antiviral and steroid therapy, a rapid improvement of symptoms with the development of pigmentation in the areas of retinal whitening was observed in all patients. Retinal detachment occurred in 2 male patients and was successfully treated.Conclusion Acute retinal necrosis syndrome is one of the vision threatening diseases for immunocompetent patients with poor prognosis. Early diagnosis and urgent antiviral therapy is therefore of vital importance for visual outcomes. Long term follow‐up is necessary due to high risk of retinal detachment.

Peripheral Retinal Vasculitis Associated with Lyme Disease

Peripheral Retinal Vasculitis Associated with Lyme Disease

Intravitreal bevacizumab (Avastin®) in idiopathic retinitis, vasculitis, aneurysms and neuroretinitis

Editor, First characterized by Chang et al. (1995), idiopathic retinitis, vasculitis, aneurysms and neuroretinitis (IRVAN) syndrome is defined by the association of peripheral retinal vascular occlusion, retinal vasculitis and multiple posterior retinal arterial aneurysms. The severity of the disease results from retinal ischaemia as much as neovascular complications; the consensual treatment is panretinal photocoagulation (PRP) started as quickly as possible (McDonald 2003; Samuel et al. 2007). We report a patient treated with bevacizumab (Avastin®) for a neovascular form of IRVAN syndrome. A 60-year-old woman was referred as an emergency to the XV–XX National Hospital Centre in Paris for sudden vision loss of her right eye. Her best-corrected visual acuity (BCVA) was hand movements in the right eye and 20/20 in the left eye. Ocular examination revealed profuse bilateral rubeosis iridis with an open angle and normal intraocular pressure, total vitreous haemorrhage in the right eye and epipapillary neovascularization in the left eye (Fig. 1). According to the new classification proposed by Samuel, she was classified as stage 4 (Samuel et al. 2007). (A) Gonioscopic view of rubeosis, (B) rubeosis iridis and (C, D) marked regression of rubeosis iridis. On fluorescein angiography, we noted multiple aneurysms, arterial vascular occlusion, late diffuse staining of the optic nerve head and diffuse retinal ischaemic areas in the right eye (Fig. 2). (A) Epipapillary neovascularization, (B) inferotemporal and superonasal aneurysm, ischaemia and temporal arterial occlusion, and (C) regression of neovascularization, panretinal photocoagulation. Systemic examination and laboratory findings ruled out any systemic abnormality. The patient was treated by bilateral intravitreal injection (IVT) of bevacizumab (1.25 mg, 0.05 ml) with 24 hr delay. Detailed informed consent was obtained from the patient. PRP was begun in the left eye; she underwent a pars plana vitrectomy with endolaser photocoagulation in the right eye three days later. Five days after IVT, we noticed the total regression of the rubeosis iridis (Fig. 1) and regression of the epipapillary neovascular vessel. The injection was performed following the standard IVT protocol. Fluorescein angiography carried out 2 months later revealed total regression of retinal neovascularization, complete PRP (Fig. 2). No side-effects were noted. At 6-month follow-up, final visual acuity was 20/25 in the left eye and 20/80 in the right eye. This was explained by a corticonuclear cataract and persistent macular oedema with exudation from an aneurism. Intravitreal injection of bevacizumab has been effective in treating retinal neovascularization and bilateral rubeosis iridis in our patient and others described in the literature (Avery 2006; Avery et al. 2006; Jiang et al. 2008). It has also been used to treat choroidal neovascularization in posterior uveitis such as toxoplasmosis, lupus or sarcoidosis (Guthoff & Goebel 2008; Kurup et al. 2009). As a result of ischaemic sequelae, and despite usual treatment, the poor visual outcome reported in the literature at this level of severity has motivated the use of anti-vascular endothelial growth factor (VEGF) to stop the predictable evolution to neovascular glaucoma or tractional retinal detachment and definitive visual loss. Theoretically, the focal nature of the pathology in this condition is similar to that found in retinal neovascularization associated with diabetes. Anti-VEGF IVT seems to be a new approach in the treatment of all kinds of pathology where a high level of VEGF is produced secondary to large retinal ischaemic areas (Caldwell et al. 2003). Therefore, the favourable results of IVT of anti-VEGF for age-related macular degeneration or diabetes (Avery et al. 2006) may be helpful in deciding whether treatment is indicated for a particular patient with neovascularization associated with IRVAN (stages 3, 4 and 5). As in combined treatment for high-risk proliferative diabetic retinopathy (Tonello et al. 2008), we treated this patient using PRP and bevacizumab successfully. Although PRP remains the gold-standard treatment of IRVAN, intravitreal injection of anti-VEGF could be an option to consider when an IRVAN is discovered at an advanced stage as rubeosis iridis or neovascular glaucoma. Further study with a larger group of patients is required.