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Aim: This study aimed to report a case of vitreous and optic nerve infiltration of a primary adrenal B lymphoma. Case Report: An 81-year-old patient consulted for decrease in vision in the left eye and pain with ocular movements during the last week. The patient was being treated with chemotherapy for an adrenal gland diffuse large B-cell lymphoma. On examination, vitritis was seen in both eyes and optic disc edema in his left eye. After an extensive study based on orbital and brain magnetic resonance imaging and vitreous cytology, an ocular infiltration by systemic B lymphoma was confirmed. A treatment based on intravitreal methotrexate was carried out, achieving the regression of the vitreous infiltration and resolution of the optic disc edema. Systemic B lymphoma metastasizing ocular structures is extremely infrequent. The manifestations may mimic an inflammatory disease. Ophthalmologists should be aware of these manifestations and consider among the masquerade syndromes.

Idiopathic retinal vasculitis, aneurysms, and neuroretinitis (IRVAN) syndrome is a rare and progressive disorder that predominantly affects both the eyes of young female individuals and can threaten visual function. Peripheral ischemia and macular exudation are common findings in patients. The treatment options include panretinal photocoagulation (PRP), systemic immunosuppression, and intravitreal antiangiogenic and corticosteroid therapy. Fluocinolone acetonide intravitreal implant is approved for the treatment of nonanterior noninfectious uveitis and diabetic macular edema (ME), with an estimated therapeutic duration of 3 years. We describe a case of IRVAN syndrome in a child with ME who had been previously treated with PRP, antiangiogenic therapy, and several dexamethasone intravitreal implants and received a fluocinolone acetonide intravitreal implant in her right eye. The patient showed stabilization of the visual acuity and a marked reduction of the macular thickness 1 month after the treatment. At 12-month follow-up, the patient required perifoveal focal photocoagulation due to a rebound of the ME. After 2 years of follow-up, visual acuity remains stable and macular retinal thickening under control. Local long-standing steroid therapy has proved to be quite efficient in controlling the progression of the disease in our patient.

PURPOSE To report multimodal imaging findings in a case of presumed Diffuse Unilateral Subacute Neuroretinitis (DUSN) associated with Strongyloides stercoralis infection. METHODS Retrospective case report. RESULTS A thirty-eight-year-old woman presented with visual loss of one week’s duration in her left eye. She had a medical history of chronic dermatitis and epigastralgia. Fundoscopic examination revealed diffuse subretinal inflammatory lesions. Optical coherence tomography showed a disruption of the ellipsoid layer and focal thickenings of the retinal pigment epithelium in the macular region, as well as cells in the vitreous cavity. Fundus autofluorescence showed granular hyperautofluorescence in the posterior pole. Fluorescein angiography revealed leakage of the optic disk and pinpoint disperse hyperfluorescent lesions. Serology tests detected Strongyloides stercoralis antibodies and blood count showed eosinophilia. Treatment with Ivermectine resolved both the ophthalmologic abnormalities and the eosinophilia. CONCLUSION DUSN may be associated with Strongyloides stercoralis infection. Inflammatory lesions, if treated early, may resolve without sequelae.

The aim of the UVHER project is to evaluate the risk of development of optic nerve damage in patients with herpetic anterior uveitis (AU) prospectively followed over 2 years. Herein, we described the baseline characteristics.This is a multicentre, prospective study. An aqueous humour tap was performed. Only patients with a positive PCR were included. Clinical characteristics, optical coherence tomography (OCT) parameters and visual field (VF) abnormalities were registered.27 patients were included: 18 Herpes Simplex (HSV), one Varicella Zoster (VVZ), and 8Cytomegalovirus (CMV). Patients with HSV-AU had severe inflammation, iris atrophy and corneal involvement. In patients with CMV-AU, less inflammation and medium-to-large keratic precipitates were observed. OCT showed a thinner RNFL and GCL in CMV-AU patients in comparison to HSV patients. VF showed abnormalities in six cases.Patients in the UVHER cohort showed the typical clinical manifestations of herpetic AU. In CMV patients, optic nerve damage was observed at baseline, and in HSV patients, inflammation was more severe.

Our aim is to describe clinical and optical coherence tomographic features of acute paraneoplastic focal outer retinitis associated with optic neuropathy in a patient diagnosed with small cell lung carcinoma. Bilateral focal outer retinitis, bilateral optic disc oedema and vitritis were identified in a patient with progressive bilateral visual loss and ataxia. Spectral domain optical coherence tomography (SD-OCT) revealed various extents of granular hyperreflectivity and atrophy of the macular outer retinal layers. Serum and cerebrospinal fluid positivity for anti-CRMP5, anti-HU and anti-amphiphysin antibodies intensified the search for an underlying malignancy, and a small cell lung carcinoma (SCLC) was finally diagnosed. Local and oral steroids along with systemic chemotherapy and radiotherapy achieved cancer remission and partial recovery from the ophthalmic manifestations. Focal outer retinitis associated with optic disc oedema and antibody positivity facilitated the diagnosis of SCLC in a localized stage. SD-OCT allowed the characterization of this entity, expanding the spectrum of paraneoplastic ophthalmological syndromes.

Purpose To assess the efficacy and safety of adalimumab in elderly patients with noninfectious uveitis (NIU). Methods An observational, retrospective, multicenter study was done. Changes in best-corrected visual acuity (BCVA), inflammatory activity parameters, central retinal thickness (CRT), and the occurrence of adverse events (AE) developed during follow-up were recorded. Results A total of 82 eyes from 41 patients 60 years of age and older with noninfectious uveitis treated with adalimumab were included. A significant improvement in BCVA (71.5 to 75.4 letters,p= .001) and in CRT (311.1 mu m to 265 mu m,p= .001) was observed. Moreover, a significant decrease from baseline in the rate of patients with anterior chamber cell (ACC) >0+ (34.6% to 5.7%,p= 0+ (21.3% to 4.3%,p= .002) was determined. AEs were observed in 11 patients (26.8%). Conclusion Adalimumab can be safe and efficacious for the treatment of NIU in patients 60 years of age and older.

To compare imaging modalities for the choroid of the eye, and evaluate various choroidal changes in uveitides entities.A comprehensive systematic literature review was conducted looking at current imaging modalities available to assess choroid architecture and commonly used parameters available to qualify and quantify choroidal changes, before looking at specific uveitides entities with choroidal involvement which have been broadly separated into non-infectious and infectious in etiology.We describe the various modalities currently available to evaluate the choroid of the eye such as Ultrasound B Scan, ICGA, and OCT. Choroidal changes in various ocular and systemic diseases such as Behcet's Disease, Sarcoidosis, Syphillis, Tuberculosis, and many more have been reported and published.Multiple choroidal tomographic and angiotomographic findings have been demonstrated for evaluation in uveitis. These findings can manifest in multiple ocular and systemic diseases, and can be illustrated using the various imaging modalities at present. Future advancements in choroidal imaging would help to adapt these findings into parameters for clinical practice to properly evaluate these ocular and systemic diseases.

BackgroundOcular toxoplasmosis is common across all regions of the world. Understanding of the epidemiology and approach to diagnosis and treatment have evolved recently. In November 2020, an international group of uveitis-specialised ophthalmologists formed the International Ocular Toxoplasmosis Study Group to define current practice.Methods192 Study Group members from 48 countries completed a 36-item survey on clinical features, use of investigations, indications for treatment, systemic and intravitreal treatment with antiparasitic drugs and corticosteroids, and approach to follow-up and preventive therapy.ResultsFor 77.1% of members, unilateral retinochoroiditis adjacent to a pigmented scar accounted for over 60% of presentations, but diverse atypical presentations were also reported. Common complications included persistent vitreous opacities, epiretinal membrane, cataract, and ocular hypertension or glaucoma. Most members used clinical examination with (56.8%) or without (35.9%) serology to diagnose typical disease but relied on intraocular fluid testing—usually PCR—in atypical cases (68.8%). 66.1% of members treated all non-pregnant patients, while 33.9% treated selected patients. Oral trimethoprim–sulfamethoxazole was first-line therapy for 66.7% of members, and 60.9% had experience using intravitreal clindamycin. Corticosteroid drugs were administered systemically by 97.4%; 24.7% also injected corticosteroid intravitreally, almost always in combination with an antimicrobial drug (72.3%). The majority of members followed up all (60.4%) or selected (35.9%) patients after resolution of acute disease, and prophylaxis against recurrence with trimethoprim–sulfamethoxazole was prescribed to selected patients by 69.8%.ConclusionOur report presents a current management approach for ocular toxoplasmosis, as practised by a large international group of uveitis-specialised ophthalmologists.

Purpose: To report a case of Acute Lymphoblastic Leukemia masquerading as Vogt-Koyanagi-Harada disease.Case Report: A 61-year-old man presented with decreased visual acuity in both eyes. Optical coherence tomography showed bilateral exudative retinal detachment and fluorescein angiography pinpoint leakage. The patient was diagnosed with Vogt-Koyanagi-Harada disease. Blood analysis revealed increased percentage of blast cells (69%) and bone marrow biopsy confirmed the diagnosis of acute B cell lymphoblastic leukemia. He received chemotherapy treatment with multiple drugs including asparaginase, which caused hepatic disease and patient’s death despite ocular findings improvement.Conclusions: Acute lymphoblastic leukemia may present as a bilateral exudative retinal detachment and can mimic VKH. Therefore, clinicians should take into consideration the possibility of leukemia in such patients. This fact stresses out the importance of multidisciplinary management of uveitis conditions.

Objective The objective of this report is to analyse retinal changes over a five-year period, assessed by spectral domain-optical coherence tomography (SD-OCT), in patients from the Lupus-Cruces cohort treated with hydroxychloroquine (HCQ). Methods SD-OCT screening was performed annually between 2012 and 2017. Average macular thickness (AMT), ganglion cell layer thickness (GCLT) and qualitative data of retinal pigment epithelium (RPE) and external retina (ExtR) were collected prospectively. We compared data from 2012 (first) and 2017 (second) SD-OCT. Results We studied 110 patients and 195 eyes. No cases of HCQ toxicity were detected. At the time of the second SD-OCT, 99% patients had taken a daily dose of HCQ ≤5 mg/kg/day. The median time on HCQ was 133 months. The mean AMT and GCLT were significantly lower in both eyes at the second SD-OCT; however, all the differences were clinically insignificant at less than 1%. Qualitative analysis of RPE and ExtR showed no significant changes. Similar results were found among patients with risk factors for retinopathy. The comparison of patients with and without risk factors showed no differences. Conclusions This study shows clinically irrelevant retinal changes in an SLE cohort on HCQ treatment over a five-year follow-up. Our findings support the safety of long-term HCQ at doses ≤5 mg/kg/day.

A recent clinical image of hydroxychloroquine (HCQ) retinal toxicity has been recently published1. In this case, the patient who presented with symptoms was on long-term treatment with HCQ at more than twice the recommended dose related to her weight, and the tests were consistent with established retinopathy.

To assess the safety and efficacy of intravitreal dexamethasone implants in the treatment of macular edema secondary to infectious uveitis.We retrospectively reviewed clinical records from three uveitis referral units in Spain. The main outcome measures were rate of reactivation of infection, improvements in visual acuity and resolution of macular edema, as measured by optical coherence tomography.We included eight eyes from seven patients with a median age of 64 years (30-75). Etiologies of the infections were Herpes simplex virus-type 1, Varicela-Zoster virus, Treponema pallidum, Brucella mellitensis, Borrelia burgdorferi, Toxoplasma gondii, and cytomegalovirus. Median visual acuity was 20/160 (20/30-20/400) at baseline and 20/70 (20/25-20/200) at the last follow-up visit. Mean macular thickness was 516 μm (115) at baseline and 266.3 μm (40) at the last follow-up visit. Visual acuity improved in 100% of the eyes and none of the eyes showed macular edema at the last follow-up visit. Five patients required reinjections of the implant. Only one patient required antiglaucoma drops for a temporary increase in ocular pressure. There were no cases of reactivation of the infectious ocular disease. Median follow-up time was 18 months.In this small case series of eyes with macular edema secondary to infectious uveitis, treatment with dexamethasone intravitreal implants was not associated with reactivation of the infectious ocular disease. Furthermore, significant improvements in visual acuity and macular thickness were observed in our patients.

Background: Hyperreflective foci have been described in OCT imaging of patients with retinal vascular diseases. It has been suggested that they may play a role as a prognostic factor of visual outcomes in these diseases. The purpose of this study is to describe the presence of hyperreflective foci in patients with non-infectious uveitic macular edema and evaluate their behavior after treatment. Methods: We conducted a multicenter, prospective, observational, 12-month follow-up study. Inclusion criteria were age > 18 years and a diagnosis of non-infectious uveitic macular edema, defined as central macular thickness of > 300 mu m as measured by OCT and fluid in the macula. Collected data included best corrected visual acuity, central macular thickness and the presence, number and distribution (inner or outer retinal layers) of hyperreflective foci. Evaluations were performed at baseline, and at 1, 3, 6, and 12 months after starting treatment. Results: We included 24 eyes of 24 patients. The frequency of patients with >= 11 hyperreflective foci was 58.4% at baseline, falling to 20.8% at 12 months. Further, hyperreflective foci were observed in the outer retinal layers in 50% of patients at baseline and just 28.6% at 12 months. Mean LogMAR visual acuity improved from 0.55 (95% CI 0.4-0. 71) at baseline to 0.22 (95% CI 0.08-0.35) at 12 months (p < 0.001). Mean central macular thickness decreased from 453.83 mu m (95% CI 396.6-511) at baseline to 269.32 mu m (95% CI 227.7-310.9) at 12 months (P < 0.001). Central macular thickness was associated with number (p = 0.017) and distribution (p = 0.004) of hyperreflective foci. Conclusions: We have observed hyperreflective foci in most of our patients with non-infectious uveitic macular edema. During follow-up and after treatment, the number of foci diminished and they tended to be located in the inner layers of the retina. This work was supported by grants from: Spanish Ministry of Economy, Industry and Competitivity, Carlos III Health Institute: PI 13/02148, cofinanced by the European Regional Development Fund.

Background Continuous progression of lesions despite an adequate treatment has been described in tubercular multifocal serpiginoid choroiditis. Reported treatments for this paradoxical response include systemic steroids, immunosuppressive drugs, and intravitreal methotrexate. We describe the use of dexamethasone intravitreal implants in a patient presenting with this condition. Findings A 46-year-old woman sought medical attention for scotomas in her left eye. Tests suggested multifocal serpiginoid choroiditis associated with latent tuberculosis infection, and hence, she was started on anti-tuberculosis drugs in combination with corticosteroids. Given that lesions progressed despite this treatment, we began treatment with dexamethasone intravitreal implants. After injection of the second implant, we succeeded in inactivating the inflammatory process. Conclusions Dexamethasone intravitreal implants may be a suitable alternative to systemic steroids or immunosuppressive therapy in the management of continuous progression of lesions in tubercular multifocal serpiginoid choroiditis.

A 32 year-old woman was referred from the Ophthalmology Department to rule out a possible systemic disease. Her only past medical history of relevance was a tuberculosis contact during childhood. She complained of floaters and progressive blurring of vision in both eyes for some months, as well as arthralgia and cough. Her visual acuity was 0.3 in the right eye and 0.4 in the left eye. Biomicroscopy showed bilateral anterior granulomatous uveitis (1+ cells). Funduscopy showed bilateral vitritis 3+, snow banking and peripheral phlebitis. Fluorescein angiography did not show central vasculitis, and optical coherence tomography showed bilateral cystoid macular oedema. Fundus autofluorescence was normal. How would you initially assess this patient in order to decide which systemic examination should be performed, bearing in mind the ophthalmological manifestations?

Purpose: To describe a case of sarcoid uveitis subsequent to anti-TNFα therapy and review previously reported cases.Methods: Review of the clinical records of the authors’ patient and of the literature using the PubMed database.Results: A 30-year-old woman presented with intermediate uveitis with snowballs in both eyes. She was on treatment with etanercept due to psoriatic arthritis. An ultrasound-guided biopsy of a mediastinal adenopathy showed non-necrotizing granulomas compatible with sarcoidosis. The clinical picture resolved after etanercept was withdrawn and treatment with immunosuppressants. In a literature search the authors identified six other cases of sarcoid uveitis induced by anti-TNFα therapy. The types of uveitis were anterior uveitis, posterior uveitis, or panuveitis. Management consisted of withdrawal of anti-TNFα therapy and administration of immunosuppressive agents in 5 cases.Conclusions: Sarcoid uveitis induced by TNFα antagonists is a rare complication. Appropriate management consist...

Purpose: To describe the clinical characteristics and to assess visual prognosis of patients with syphilis-associated uveitis in northern Spain.Methods: Retrospective review of clinical records in eight general uveitis referral centers.Results: Since the year 2000, 50 patients have been diagnosed: 31 men and 19 women; median age was 41 (19–76) years. A total of 34% were co-infected with HIV and 24% presented systemic manifestations of syphilis. Median initial visual acuity and vision at last visit in 93 affected eyes was 20/50 (20/20–20/2000) and 20/22 (20/20–20/2000), respectively (p

Purpose: To report a case of neuroretinitis associated with ischemic nasal branch retinal vein occlusion, periphlebitis, and neovascularization of the optic disk.Methods: Case report.Results: A 32-year-old man presented with a typical image of neuroretinitis, retinal hemorrhages and sheathing of the retinal veins in the nasal retina. His left hand had been bitten by a kitten 8 weeks before. Serology for Bartonella henselae was negative. On the 6th week of follow-up, optic disk neovascularization developed, which required retinal photocoagulation. Photocoagulation was performed again at the 12th and 18th week revision since further new vessels had developed. At the 32nd week of follow-up neovascularization had regressed.Conclusions: Neuroretinitis may be associated with severe complications such as retinal vascular occlusions and optic disk neovascularization.

Objective:Interferon (IFN) signaling plays a crucial role in autoimmunity. Genetic variation in interferon regulatory factor 5 (IRF5), a major regulator of the type I interferon induction, has been associated with risk of developing several autoimmune diseases. In the current study we aimed to evaluate whether three sets of correlated IRF5 genetic variants, independently associated with SLE and with different functional roles, are involved in uveitis susceptibility and its clinical subphenotypes.Methods:Three IRF5 polymorphisms, rs2004640, rs2070197 and rs10954213, representative of each group, were genotyped using TaqMan® allelic discrimination assays in a total of 263 non-anterior uveitis patients and 724 healthy controls of Spanish origin.Results:A clear association between two of the three analyzed genetic variants, rs2004640 and rs10954213, and the absence of macular edema was observed in the case/control analysis (PFDR=5.07E-03, OR=1.48, CI 95%=1.14-1.92 and PFDR=3.37E-03, OR=1.54, CI 95%=1.19-2.01, respectively). Consistently, the subphenotype analysis accordingly with the presence/absence of this clinical condition also reached statistical significance (rs2004640: P=0.037, OR=0.69, CI 95%=0.48-0.98; rs10954213: P=0.030, OR=0.67, CI 95%=0.47-0.96), thus suggesting that both IRF5 genetic variants are specifically associated with the lack of macular edema in uveitis patients.Conclusion:Our results clearly showed for the first time that two functional genetic variants of IRF5 may play a role in the development of macular edema in non-anterior uveitis patients. Identifying genetic markers for macular edema could lead to the possibility of developing novel treatments or preventive therapies. © 2013 Márquez et al.

Ocular toxoplasmosis causes abnormalities in the vitreous that are responsible for several types of well-known complications including retinal detachment and epiretinal membranes. We report on a patient who developed toxoplasmic panuveitis with a full-thickness macular hole (MH) and was successfully treated with vitreoretinal surgery. A 35-year-old Hispanic female presented with a 2-week history of loss of visual acuity and metamorphopsia in her right eye. Funduscopy revealed a typical toxoplasmosis lesion and a MH, which was confirmed by optical coherence tomography. After 8 weeks of medical treatment with sulfamethoxazole (800 mg)/trimethoprim (160 mg) and steroids, the intraocular inflammation was considered inactive. Pars plana vitrectomy with inner limiting membrane peeling and injection of 24 % sulphur hexafluoride gas were performed to treat the MH, without success. Repeat pars plana vitrectomy was then performed with injection of 14 % perfluoropropane (C3F8). Closure of the MH was achieved after this second procedure. Vitreoretinal surgery may be safe and effective for treating MHs secondary to toxoplasmosis lesions, a very uncommon complication of this disease.

Purpose To identify clinical outcomes and treatment patterns of intravitreal dexamethasone implant (Ozurdex; Allergan, Inc) in noninfectious uveitis in the clinical setting. Design Multicenter retrospective cohort study. Methods Eighty-two eyes (63 patients) receiving 142 implant injections over 35 months were included. Treatment indication, uveitis diagnosis, visual acuity, intraocular pressure, vitreous haze score, central retinal thickness by optical coherence tomography, phakic status, number of injections, time to reinjection, systemic treatments, and complications data were collected. Time to visual acuity and vitreous haze score improvement as per the Standardization of Uveitis Nomenclature guidelines were also determined. Results The probability of visual acuity improvement (≥0.3 logarithm of the minimal angle of resolution units improvement) was 39% at 1 month, 49% at 3 months, 52% at 6 months, and 58% at 12 months. Eyes with baseline vitritis (vitreous haze score ≥+0.5, n = 45) had a probability of vitreous haze score improvement (2-step decrease or change from +0.5 to 0) at 2 weeks of 41%, at 1 month 63%, at 3 months 73%, at 6 months 79%, and at 12 months 88%. In eyes that completed 12-month follow-up (n = 54), 40.7% underwent 2 injections (mean time to second injection of 6.6 ± 1.9 months) and 11.2% required ≥3 injections (mean time to third injection of 11 ± 1.5 months). Conclusions Dexamethasone implant use in uveitis provides favorable visual acuity and vitreous haze score outcomes but requires repeated injections, an important consideration when choosing intraocular treatment as a route to controlling uveitis.