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To describe a case of T-cell prolymphocytic leukemia (T-PLL) masquerading as viral retinal necrosis (VRN). A 75-year-old-man with a history of T-PLL in complete remission complained of an acute vision loss in his right and only eye. Ophthalmic examination demonstrated the presence of anterior chamber cells, mild vitritis, and peripheral retinal whitening with intraretinal hemorrhages evocative of VRN. While the anterior chamber tap came back negative for HSV, VZV, and CMV, cytology performed on the aqueous humor described the presence of leukemic cells. T-PLL can rarely masquerade as a viral retinal necrosis. Diagnostic work-up should therefore always rule out the infectious causes of retinitis. Anterior chamber tap can sometimes prove useful in the diagnosis of T-PLL even in the absence of a hypopyon, avoiding the need for vitrectomy. [ABSTRACT FROM AUTHOR]

Purpose: To define a clinically tailored therapeutic strategy for the treatment of viral anterior uveitis (VAU).Methods: A PubMed search spanning the past 5 years was conducted using the MesH-terms "viral anterior uveitis" and "therapy."Results: The herpes simplex virus (HSV), the varicella zoster virus (VZV), and the cytomegalovirus (CMV) are the predominant pathogens in VAU. Other viruses, including rubella, chikungunya, and zika, have been linked with distinct forms of the disease. Depending on the causative agent and the host immunocompetence, the mainstay treatment for suspected VAU is a combination of topical or systemic antivirals and topical corticosteroids, supplemented with cycloplegics and intraocular-pressure-lowering medication.Conclusions: Oral acyclovir, valacyclovir, and famciclovir are the mainstay of treatment for HSV- and VZV-induced infections. Brivudin serves as an alternative in insufficiently responsive cases. CMV-induced infections respond well to valganciclovir. A 3- to 12-month course of prophylactic treatment against recurrences is worth considering. [ABSTRACT FROM AUTHOR]

Purpose: To determine the patterns and causes of uveitis in a referral eye clinic in Qatar.Methods: A retrospective study was conducted on all cases of uveitis that presented to the Uveitis Clinic at Hamad Medical Corporation from March 2007 to February 2011. All patients had detailed ocular, medical examination, and uveitis work up.Results: The study included 310 patients. Anterior uveitis was noted in 53.2% of cases (165/310), followed by panuveitis 16.8% (52/310), intermediate uveitis 16.5% (51/310), and posterior uveitis 13.6% (42/310). Causes of uveitis were determined in 69.7% (216/310) of cases. The most commonly identified causes of uveitis in our study were presumed ocular tuberculosis (14.5%), Fuchs uveitis (11.0%), Behçet disease (7.4%), and VKH disease (6.8%).Conclusions: Anterior uveitis is the most common anatomic/clinical form of uveitis in Qatar and the etiologic diagnosis of uveitis should focus in particular on presumed ocular tuberculosis, Fuchs uveitis, Behçet disease, and VKH disease. [ABSTRACT FROM AUTHOR]

Purpose: To compare the evidence base and systemic treatment strategies for sarcoidosis. Methods: Medline and EMBASE literature search on ''sarcoidosis AND treatment'', ''sarcoidosis AND uveitis AND treatment'', and ''sarcoidosis AND eye AND treatment''. The search was limited to randomized controlled trials (RCTs) and meta-analyses. Results: A total of 19 RCTs for the systemic treatment of extraocular sarcoidosis were identified. The majority were on corticosteroid--oral and inhaled. There were two meta-analyses on corticosteroid, including a Cochrane review. Only two RCTs were indentified for the treatment of intraocular sarcoidosis, one on etanercept, and the other from 1967 on prednisolone or oxyphenbutazone vs. placebo. There were no meta-analyses. Due to the paucity of RCTs other treatment studies were included but these were limited to only a few immunosuppressive agents and on small numbers of patients. Conclusion: Limited high-quality evidence exists for the systemic treatment of sarcoidosis, in particular intraocular disease. [ABSTRACT FROM AUTHOR]

Diagnosis of PIOL can be challenging. It requires a high degree of clinical suspicion and differential diagnosis includes infectious and non-infectious etiologies particularly the common masquaraders sarcoidosis, tuberculosis, viral retinitis and syphilis. The definitive diagnosis depends on demonstration of malignant lymphoma cells in ocular specimens or CSF. Ocular specimen could include vitreous, aqueous or chorioretinal biopsy. Ocular pathologist should be consulted prior to the diagnostic procedure to help handle and process the specimen appropriately. In addition to cytology, flow cytometry, immunohistochemistry, molecular analysis and cytokines may be used as adjuncts in facilitating the diagnosis. [ABSTRACT FROM AUTHOR]

Purpose: To evaluate the efficacy of interferon alfa-2a (IFN alfa) in severe uveitis associated with Behçet's disease, that is refractory to steroids and conventional immunosuppressive agents.Patients and Methods: Patients with Behçet's disease (according to the International Study Group criteria), who relapsed despite steroids and immunosuppressive agents, were included in this retrospective study. Ophthalmological examination, laser flare photometry, and fluorescein angiography associated with laboratory tests were performed at regular intervals. IFN alfa (3 millions units thrice a week) was injected subcutaneously.Results: Eight patients (sex ratio: 1) were included between May 1995 and January 1999. The mean age was 29.1 years (14-54 years) and the disease was present between 11 and 167 months before the administration of IFN alfa. IFN alfa was efficient in all cases with a mean follow-up of 22 months (10-55 months). Steroids were tapered from a mean dosage of 47 mg/d to a mean dosage of 8.5 mg/d. Ocular inflammation was controlled and visual acuity improved in all cases. Treatment was generally well tolerated despite a constant but transient flu-like syndrome. IFN alfa was tapered in three patients and stopped in one case without any relapse after five months.Conclusions: Within the limitations of this retrospective study, low-dose IFN alfa seems to be well tolerated, promising in the management of refractory forms of uveitis due to Behçet's disease, and effective in allowing a reduction of steroid dosage. A prospective controlled study is necessary to confirm these preliminary results. [ABSTRACT FROM AUTHOR]

Graph Collaborative Ocular Tuberculosis Study (COTS) group through COTS-1, COTS Nomenclature and COTS CON have published a significant number of articles on ocular tuberculosis. COTS group has also inspired researchers in other subspecialties of Ophthalmology and the model adopted by COTS is now universally adopted for large-scale collaborative studies and also for generating consensus guidelines. COTS group through its numerous publications has also consolidated the definition of treatment failure in patients with ocular tuberculosis. [Extracted from the article]

Case Report Case description: This report describes the case of a female patient diagnosed with oculo-cerebral toxocariasis manifesting initially in the form of isolated bilateral cystoid macular edema. Diagnosis was made by means of positive anterior chamber and lumbar puncture western blots. The unusual presentation, ancillary findings and treatment are discussed. The control of intraocular inflammation that was only partially responsive to steroids was eventually achieved with pegylated interferon alfa-2a. Isolated macular edema is a rare presentation of ocular toxocariasis. Interferon alfa-2a may prove useful in case of insufficient control of inflammation. [ABSTRACT FROM AUTHOR]

PurposeMethodsResultsConclusionTo report the clinical features and treatment outcomes in adult Caucasians with ocular toxocariasis (OT) and investigate their prognosis depending on their serological status.Retrospective observational cohort study (2016–2021) including consecutive adults with uveitis and positive western blot (WB) in the aqueous humor or vitreous. The presence of serum antibodies was not necessary for inclusion, allowing to compare the outcomes depending on the serological status.Seventeen eyes of 15 patients were included. Mean age at diagnosis was 51.9 years. Vitreous inflammation was the most frequent sign (100%). Vitreoretinal tractions (41.2%) and chorioretinal granulomas (58.8%) were less prevalent. Atypical features were: spontaneous intravitreal hemorrhage (23.5%), exudative retinal detachment (11.8%), isolated macular edema (17.6%), papillitis (29.4%) and vasculitis (47.1%). Twenty percent of patients had a positive serum serology. Baseline clinical features did not differ statistically depending on the serological status; however, the degree of inflammation was numerically higher in patients with negative serology. Overall, macular thickness, anterior and posterior segment inflammation improved significantly after treatment with oral albendazole, systemic ± local corticosteroids. Vitrectomy (47.1%) was performed in case of persistent vitritis (62.5%), retinal detachment (12.5%) and intravitreous hemorrhage (25%).OT has no pathognomonic sign and atypical presentations were not infrequent in this adult Caucasian cohort. Serum antibodies were rarely positive, stressing on the importance of ocular sample analysis, especially in case of atypical features. Serum antibodies may prove useful in forecasting the rapidity of inflammation clearance. Antiparasitic and anti-inflammatory treatment was safe and efficient in most cases. [ABSTRACT FROM AUTHOR]

Introduction: Uveitic macular edema (UME) is a significant cause of visual impairment in all uveitis types. Methods: Reports that were cited in the MEDLINE database, that analyzed the effectiveness of biologics for UME in at least five patients, with a minimum follow-up of 3 months, published prior to April 1, 2019 were included. Reports that did not compare UME findings before and after the therapy, using either OCT or fluorescein angiography, were excluded. Results: Case series that analyzed the efficacy of intravitreal anti-VEGF agents showed modest, short-term benefit. Studies that investigated systemic anti-TNF agents in patients with noninfectious uveitis reported a therapeutic effect on UME. Anti-IL-6 antibodies have shown promising results for most severe cases of noninfectious UME. Interferon represents an option for patients with persistent UME in infectious and noninfectious uveitis. Conclusion: Multicenter, randomized controlled trials are needed to assess the effectiveness of each group of biologic agents in sufficient number of patients. [ABSTRACT FROM AUTHOR]

To report and illustrate the main clinical presentations of posterior herpetic uveitis. Narrative review. The ocular manifestations of posterior herpetic uveitis include different clinical presentations. Herpes simplex and varicella zoster can cause acute retinal necrosis, progressive outer retinal necrosis, and non-necrotizing herpetic retinopathies. Cytomegalovirus has been associated with fulminant retinitis with confluent areas of retinal necrosis and retinal hemorrhages, indolent/granular retinitis, and frosted branch angiitis. These diverse clinical presentations are often associated with specific risk factors and different immunological profiles of the host. Herpetic viruses can cause posterior uveitis, presenting various clinical findings. Specific ocular manifestations and the immunological status of the host can help to differentiate the various herpetic entities before laboratory tests confirm the diagnosis. [ABSTRACT FROM AUTHOR]

Editorial. Discusses the outcome of the 2000 annual meeting of the Society of Ocular Immunoinfectiology in Europe, held in March at the Pasteur Institute in Paris, France. Attendees; Guest speakers; Classical and new aspects of ocular inflammation.

Neuro-ophthalmologic manifestations are uncommon in sarcoidosis. We aim to assess the prognostic factors and outcome of neuro-ophthalmic sarcoidosis. We conducted a multicenter retrospective study on patients with neuro-ophthalmic sarcoidosis. Response to therapy was based on visual acuity, visual field, and orbital MRI exam. Factors associated with remission and relapse were analyzed. Thirty-five patients [median (IQR) age of 37 years (26.5–53), 63% of women] were included. The diagnosis of sarcoidosis was concomitant of neuro-ophthalmologic symptoms in 63% of cases. Optic neuritis was the most common manifestation. All patients received corticosteroids and 34% had immunosuppressants. At 6 months, 61% improved, 30% were stable, and 9% worsened. Twenty percent of patients had severe visual deficiency at the end of follow-up. Nonresponders patients had significantly worse visual acuity at baseline (p = 0.01). Relapses were less frequent in patients with retro-bulbar optic neuropathy (p = 0.03). Prognosis of neuro-ophthalmic sarcoidosis is poor. [ABSTRACT FROM AUTHOR]

To describe the application of OCT-A in various posterior uveitis disorders in our experience and to compare it with the available literature. Eighteen eyes with the diagnoses of multifocal choroiditis (MFC), multifocal placoid pigment epitheliopathy (APMPPE), multiple evanescent white dot syndrome (MEWDS), tuberculous serpiginous-like choroiditis (SLC), serpiginous choroiditis (SC), and birdshot chorioretinopathy (BSCR) were studied. We found flow void of the choriocapillaris in patients with APMPPE, SC, MFC, BSCR, and in SLC. In contrast, perfusion of the choriocapillaris seemed normal in patients with MEWDS. We confirmed that OCT-A contributes new information on the physiopathology of white dot syndromes and inflammatory chorioretinopathies, notably on whether or not the choriocapillaris is involved. Comparing the OCT-A features allowed us to suggest that both APMPPE and SLC might be part of the same spectrum of inflammatory disease with primary involvement at the level of the choriocapillaris and secondary RPE damage. [ABSTRACT FROM AUTHOR]

Ocular involvement in Behçet disease (BD) is characterized by recurrent inflammatory attacks and spontaneous resolution of acute inflammatory signs. Both frequency and severity of uveitis attacks determine the magnitude of irreversible damage to intraocular structures and long-term visual prognosis. Recurrent attacks of occlusive retinal vasculitis lead to vision-threatening complications such as cystoid macular edema, retinal neovascularization, optic atrophy and retinal atrophy. This manuscript updates about the role of various drugs in the management of BD, discussing corticosteroids, disease modifying immunosuppressive drugs, and finally biologicals (anti-TNF-blocking agents and alpha interferon) which seem to be superior compared to all other available drugs in preventing loss of vision. Also recent findings from new biologicals will be summarized, and especially the role of these drugs in children will be discussed in detail. The authors suggest that at least moderate to severe retinal involvement should become treated with biologicals whenever available. [ABSTRACT FROM AUTHOR]

Juvenile idiopathic arthritis (JIA) is the most common chronic rheumatic disease and the most common systemic association of uveitis in children. JIA is subdivided into seven clinically more homogeneous subtypes, using the International League of Associations for Rheumatology (ILAR) classification system: systemic arthritis, oligoarthritis, rheumatoid factor (RF)-negative polyarthritis, RF-positive polyarthritis, psoriatic arthritis, enthesitis-related arthritis, and undifferentiated arthritis. Uveitis is very rare in patients with systemic onset JIA and in patients with juvenile-onset rheumatoid arthritis. Patients with early-onset antinuclear antibody (ANA)-positive oligoarticular JIA have the highest risk of developing chronic iridocyclitis. Ocular involvement in enthesitis-related arthritis is characterized by recurrent acute symptomatic iridocyclitis. The diagnosis and differential diagnosis of JIA are based on history, physical examination, and laboratory investigations, including complete blood count, acute-phase reactants, ANA, RF, HLA-B27, and musculoskeletal imaging studies. Recent studies have revealed distinct gene expression profiles that may be useful in the diagnosis, classification, and follow-up of JIA patients. [ABSTRACT FROM AUTHOR]

We evaluated laser flare photometry (LFP) values in patients with juvenile idiopathic arthritis (JIA)-associated uveitis. Retrospective study. A decrease of the LFP value between baseline visit and 1 month after anti-inflammatory treatment intensification allowed us to define two groups of patients: group 1 (decreased LFP value ≥50%) and group 2 (<50%). We evaluated the prevalence of vision-threatening complications in both groups. Fifty-four patients (87 eyes) were followed for 9.9 ± 5 years. Group 1 eyes (n = 54) had significantly fewer ocular complications than group 2 eyes (n = 33) at both 5 years visit (p =.03) and final visit (p =.047). At the final visit, group 2 eyes had significantly more band keratopathy, trabeculectomy, cataract surgery, glaucoma and papille edema. Group 1 eyes kept a better visual acuity (p <.0001). The decrease of LFP values ≥50% of the initial value 1 month after treatment intensification is a good early prognostic factor. [ABSTRACT FROM AUTHOR]

Basic science and clinical investigations in cancer research have contributed to our understanding of the genetic causes of various neoplasms and discovery of novel therapeutic interventions to fight malignancies such as lymphoma. During this exciting time, we have witnessed the advent of new technologies to further characterize primary intraocular lymphoma (PIOL), or retinal lymphoma, which is selected as the first “Disease of the Year” by Ocular Immunology and Inflammation. Different comprehensive aspects of PIOL, including epidemiology, clinical manifestations, diagnosis, pathophysiology, therapy, and animal models are discussed. The future of PIOL holds an opportunity to really understand the unique cytologic, histopathologic, physiological and immunologic features, as well as the genotypic traits (gene expression, interaction, polymorphism, epigenetics, etc.) and epidemiology. This information will empower us to truly make a difference in patients’ managements with this devastating disease. While most of this technology already exists, much work still needs to be done to make translational therapy a reality for PIOL patients in the future. [ABSTRACT FROM AUTHOR]

Purpose: To report the use of intravenous (IV) antiviral agents and intravitreal ganciclovir injections in three immunocompetent patients with severe acute retinal necrosis (ARN). Method: Case series. Results: Three immunocompetent patients, who had lost vision in the first eye due to ARN, received intensive treatment with IV foscarnet or acyclovir or ganciclovier and intravitreal ganciclovir injections for the treatment of severe ARN involving the fellow eye. The retinitis resolved and final visual acuity of the fellow eye improved to 20/20 in all three cases after a mean follow-up of 17 months. Conclusions: Intensive treatment with a combination of two intravenous antiviral drugs and intravitreal ganciclovir injections was successful in the management of patients with acyclovier-resistant ARN and monocular status. [ABSTRACT FROM AUTHOR]

The term "vitritis" refers to the presence of a cellular infiltration of the vitreous body, usually in the context of an intraocular inflammation, but not exclusively. Intermediate uveitis is the most prominent cause of vitritis, including infectious and auto-immune/auto-inflammatory etiologies. Corticosteroids and immunosuppressive therapies should not be started before ruling out the infectious causes of vitritis, especially in immunosuppressed individuals. Other situations can mimic intermediate uveitis such as amyloidosis and ocular tumors. Primary intraocular lymphoma should always be suspected in case of vitreous infiltrations in individuals aged over 50 years. [ABSTRACT FROM AUTHOR]

Objective: To report the clinical features and treatment outcomes in immunocompetent patients with anterior segment inflammation (ASI) related to human cytomegalovirus (HCMV) depending on their ethnic origin.Material and Methods: Multicenter retrospective study of 38 patients with at least one test, either HCMV-positive PCR or GWc.Results: Features of Posner-Schlossman syndrome were observed in 50% of the eyes, Fuchs heterochromic iridocyclitis in 13% of the eyes, chronic nonspecific anterior uveitis in 21% of the eyes, and corneal endotheliitis in 18% of the eyes. PCR and GWc were positive for HCMV in 50% and 96.2% of the eyes, respectively. Glaucoma was diagnosed in 50% of eyes. Treatment was oral valganciclovir in about half of the patients. Other treatments were intravenous ganciclovir and/or ganciclovir topical ointment and/or intravitreal ganciclovir.Conclusions: No obvious association of specific clinical features with individual ethnicity could be identified. We found a high rate of glaucoma in all ethnic groups. There was a delay in diagnosis and specific treatment of HCMV in most patients. [ABSTRACT FROM AUTHOR]

Purpose: To monitor perivascular sheathing during the course of retinal vasculitis by flood illumination adaptive optics ophthalmoscopy (AOO). Methods: Perivenous sheathing and venous diameters were quantitatively analyzed by semi-automatic segmentation of AOO images in 12 eyes of treatment-naive patients with retinal vasculitis. Results: The width of venous sheathing ranged from 45 to 225 µm (mean 101.0 µm ± 54.3). In 10 cases, the underlying vein showed focal narrowing (mean ± SD 14% ± 10). Focal narrowing of arteries was also present in one eye. At presentation, width of sheathing and vessel diameters were not correlated with fluorescein leakage. During follow-up, 5 eyes showed an increase in vein diameter or resolution of narrowing and in 10 eyes a thinning of vascular sheathing was observed (p= .003). Conclusions: Perivenous sheathing may be quantitatively analyzed and monitored by AOO. AOO may therefore contribute to monitor vascular sheathing during posterior uveitis. [ABSTRACT FROM AUTHOR]

An international, expert led consensus initiative was set up by the Collaborative Ocular Tuberculosis Study (COTS) group to develop systematic, evidence, and experience-based recommendations for the treatment of ocular TB using a modified Delphi technique process. In the first round of Delphi, the group identified clinical scenarios pertinent to ocular TB based on five clinical phenotypes (anterior uveitis, intermediate uveitis, choroiditis, retinal vasculitis, and panuveitis). Using an interactive online questionnaires, guided by background knowledge from published literature, 486 consensus statements for initiating ATT were generated and deliberated amongst 81 global uveitis experts. The median score of five was considered reaching consensus for initiating ATT. The median score of four was tabled for deliberation through Delphi round 2 in a face-to-face meeting. This report describes the methodology adopted and followed through the consensus process, which help elucidate the guidelines for initiating ATT in patients with choroidal TB. [ABSTRACT FROM AUTHOR]

Purpose: Evaluate OCT (optical coherence tomography) angiography (OCTA) features in patients with Takayasau disease. Methods: The OCTA was analyzed to evaluate perifoveal anastomatic capillary arcade disruption, microaneurysms. The foveal avascular zone (FAZ) was measured for superficial (SCP) and deep capillary plexus. Results: There were 26 eyes included. In OCTA, 11 eyes presented ruptures of the perifoveal anastomotic capillary arcade in SCP. A total of 5 had microaneurysms. The average SCP FAZ was increased to 0.34 mm2 in Takayasu compared to 0.27 mm2 in control patients. Conclusion: Fluorescein angiography is the gold standard to describe Takayasu retinopathy ischemic signs. Macular abnormalities are uncommon in Takayasu patients as retinopathy signs are mostly located in peripheral retina. This study reveals that most of our patients present an enlargement of the FAZ then highlights the relevance of OCTA to evaluate macular ischemia as a complement to usual retina global study in fluorescein angiography. [ABSTRACT FROM AUTHOR]

Purpose: To evaluate the efficacy and safety of dexamethasone (DEX) implant compared with inferior fornix-based sub-Tenon triamcinolone injection (PSTA) for treatment of uveitis.Methods: A total of 48 eyes received DEX and 49 eyes received PSTA as the first treatment.Results: A total of 31 eyes were implanted with DEX relapsed (64.5%) after the first injection, while 32 eyes were injected with PSTA as the first treatment relapsed (65.3%). Kaplan-Meier estimated survival to overall relapse after the first injection was a mean 20 months± 3.6 months for DEX (median,7) and 14 months± 1.9 months (median,9) for the PSTA (P = 0.505). Of 49 eyes receiving the PSTA implant as the first treatment, inflammation persisted in 14.3% after the first injection but persisted in none after the DEX injection (P = 0.005).Conclusions: DEX implantation achieved a higher rate of disease control in the initial 12 weeks postinjection with a relative equivalence in the duration of effect and relapse rates when compared with PSTA. [ABSTRACT FROM AUTHOR]

Purpose: To evaluate the evolution of chronic uveitis in children undergoing cataract surgery with primary intraocular lens (IOL) implantation.Methods: Twelve children with chronic uveitis underwent cataract surgery with primary posterior chamber intraocular lens (IOL) implantation.Results: Fourteen eyes were implanted with a foldable hydrophobic acrylic IOL. The mean follow-up was 35.39 months (8.72-69.57). The mean BCDVA before surgery and at the end of follow-up was 1.11 (0.40-2.30; SD: 0.57) and 0.48 (0-3; SD: 0.77; p=0.007) respectively. The mean oral corticosteroids dosage after surgery and at the end of follow-up was 0.80 mg/kg/day (SD: 0.37) and 0.17 mg/kg/day (SD: 0.24; p=0.001) respectively. All patients except one were treated with methotrexate. Four patients (5 eyes) were additionally treated with anti-tumor necrosis factor agent.Conclusions: Cataract surgery with primary posterior chamber hydrophobic IOL implantation is possible and leads to a good visual recovery in cases of pediatric chronic uveitis. This surgery requires aggressive anti-inflammatory management with immunosuppressive drugs to control inflammation and reduce the corticosteroids dosage. [ABSTRACT FROM AUTHOR]

Cytomegalovirus (CMV) anterior uveitis is the most common ocular manifestation of CMV disease in immunocompetent individuals. It is thought to be due to a local reactivation of latent CMV and is usually unilateral. The acute form presents as Posner-Schlossman Syndrome, a recurrent hypertensive anterior uveitis with few granulomatous keratic precipitates. There are geographic differences in the chronic form of CMV anterior uveitis. Asian patients commonly present as Fuchs Uveitis Syndrome with diffuse stellate keratic precipitates, while the European patients present with a chronic hypertensive anterior uveitis with fewer keratic precipitates that are brown in color and located inferiorly. Characteristic features of CMV anterior uveitis include mild anterior chamber inflammation, elevated intraocular pressure, stromal iris atrophy. Synechiae, macular edema and retinitis are typically absent. CMV disease may also be associated with the development of corneal endotheliitis with a reduced endothelial cell count. Long-term complications include glaucomatous optic neuropathy and cataract formation. [ABSTRACT FROM AUTHOR]

Introduction: Chronic uveitis treated with immunosuppressive agents may have unfavorable outcomes due to delays in diagnosis. The aim of this study was to review initial data from clinical and ocular assessments and patient outcomes following specific treatments for atypical herpes virus ocular infections.Methods: The records of four consecutive patients with recurrent uveitis for at least five years treated in our department between 2001 and 2016 were retrospectively reviewed. They had atrophic retinal lesions resulting in an unfavorable outcome with lesion progression and vision loss under immunosuppressive treatment. Varicella zoster virus (1 case) and herpes simplex virus type 2 (3 cases) were detected in ocular samples.Results: Uveitis was granulomatous and bilateral (3 cases) or unilateral (1 case). Immunosuppressive treatments worsened the clinical situation whereas antiherpetic treatments improved the control of intraocular inflammation.Conclusion: This description should be added to the broad repertoire of clinical polymorphisms associated with herpes virus infections. Recognizing these cases should be useful due to their sensitivity to antiherpetic treatments. [ABSTRACT FROM AUTHOR]

Purpose: To report on the clinical data of seven patients with T-cell intraocular lymphoma (IOL).Methods: Retrospective case series.Results: Seven immunocompetent patients, 12 eyes, 6 women, with T-cell-IOL were included from five countries. Mean age was 53.5 years (range: 25-82). Four patients had systemic-ocular lymphoma, two had CNS-ocular lymphoma, and one had systemic-CNS- ocular lymphoma. Vitritis was the most frequent clinical sign, followed by anterior uveitis and serous retinal detachment. Cytopathologic examination was performed on all ocular specimens (vitreous in six and iris mass biopsy in one patient). Adjunctive diagnostic procedures included immunohistochemistry, molecular tests, and cytokine profiling of vitreous samples. Treatment modalities included systemic chemotherapy (five patients), intravitreal methotrexate (three patients), globe radiotherapy, and intrathecal chemotherapy. Mean survival from diagnosis was 21.7 months (range: 2-69). Two patients are still alive.Conclusions: T-cell IOL has variable clinical manifestations and prognosis. Systemic involvement, SRD, and vitreoretinal involvement were frequently observed. [ABSTRACT FROM AUTHOR]

Purpose: To describe the clinical outcome of phakic eyes with macular edema (ME) due to non-infectious uveitis treated with a dexamethasone intravitreal implant.Methods: A retrospective analysis of 41 eyes treated with a total of 58 dexamethasone intravitreal implants was conducted. Best corrected visual acuity (BCVA), central retinal thickness (CRT) and complications data were collected.Results: One month after the first implant, even as CRT improved significantly in most eyes (p<0.001), 31.7% showed no improvement in BCVA. At 6 months post-implantation, CRT and BCVA had deteriorated in up to 70% of patients. Thirteen eyes were re-implanted, with a similar effect to that of the first implant. Ocular hypertension developed in 36.2% of eyes, and three eyes had cataract surgery, all in eyes with repeated implants.Conclusions: The dexamethasone intravitreal implant can be safely used to treat ME due to non-infectious uveitis, but with a limited and short effect on BCVA. [ABSTRACT FROM AUTHOR]

Purpose: Few data are available regarding the optimal treatment of multiple sclerosis (MS)-related uveitis. The aim of this study was to describe clinical features of MS-associated uveitis and determine how MS treatment affects the course of uveitis.Methods: Retrospective, multicenter study. Patients were divided into two groups according to the use (group 2) or not (group 1) of immunomodulatory drugs. Characteristics of uveitis and treatment were reviewed.Results: A total of 68 eyes from 36 patients (17 in group 1 and 19 in group 2) were included. All patients were treated with topical and/or systemic steroids for uveitis. Uveitis occurred 1-17 years prior to neurologic symptoms in 78% of patients. Uveitis was more severe in group 2 (p<0.05), with a tendency toward a higher rate of chronic uveitis (p = 0.06).Conclusions: MS-related uveitis has often a favorable evolution. Patients on interferon-beta have more severe and chronic uveitis. As far as we are concerned, interferon-beta given on the sole indication of uveitis is not recommended. If steroid-sparing agent is required for intraocular inflammation, immunosuppressive drugs should be considered. [ABSTRACT FROM AUTHOR]

The treatment of articular and extra-articular manifestations associated with HLA-B27 has undergone dramatic changes over the past two decades, mainly as a consequence of the introduction of biologic agents and in particular anti-tumor necrosis factor α (anti-TNFα) agents. Uveitis is known to be the most frequent extra-articular feature in HLA-B27-associated spondyloarthritides. Topical corticosteroids and cycloplegic agents remain the cornerstones of treatment. However, biologic therapy may be effective in the management of refractory or recurrent forms of uveitis. This review gives an update on the management of HLA-B27-associated ocular disorders with biologics, including anti-TNFα agents and non-anti-TNFα biologic modifier drugs. There is an emerging role for newer biologics targeting interleukin-12/23 and interleukin-17 for the treatment of spondyloarthritides but data on their efficacy on anterior uveitis are sparse. [ABSTRACT FROM PUBLISHER]

Purpose: To retrospectively assess the frequency of ocular relapse and the possibility of long-term remission in patients treated with interferon (IFN) for severe uveitis associated with Behçet disease.Methods: All patients were treated with an initial dosage of 3 million IU IFN three times a week. The main outcome measure was the number of relapses per person per year before, during, and after IFN treatment.Results: Of 36 patients (67 eyes), 31 (86.1%) responded to IFN. The mean follow-up was 8.19 years. Twenty-one out of 36 patients discontinued IFN and 76% of these have not relapsed within 5.05 years after discontinuation. The mean relapse per person per year decreased significantly from 1.39 to 0.0496 (p = 1.82×10-10) during the treatment period and remained at 0.057 relapses per person per year after IFN discontinuation.Conclusion: IFN efficiently decreases the relapse rate and seems to permit long-term remission even after discontinuation. [ABSTRACT FROM AUTHOR]

The author reflects on Behcet Uveitis (BU). Topics discussed include Behcet disease (BD) as a systemic disorder; and ocular complications of BU that are edema, non-perfusion, neovascularization, and atrophy of the retina and optic disc. Also being discussed are tumor necrosis factor (TNF) inhibitor or interferon, conventional immunosuppressive therapy, and indocyanine green angiography (ICGA) and fundus autofluroescence (FAF).

Purpose: In juvenile idiopathic arthritis (JIA)-associated uveitis complicated by foveal serous retinal detachment (FSRD), we documented the relationship between best corrected visual acuity (BCVA), level of anterior chamber flare and OCT features.Methods: Nine children (15 eyes) with FSRD were identified among 38 children with JIA-associated maculopathy. Outcome measures included BCVA, ocular inflammatory activity quantified by laser flare photometry and the macular profile analyzed by OCT.Results: The diagnosis of FSRD led to intensification of the treatment using subtenon's injection of triamcinolone or systemic immunomodulatory therapy. The improvement of BCVA at presentation (0.46 logMAR) was significant at 36 months follow-up (0.15 logMAR). The resolution of FSRD along with visual improvement (p = 0.0032) correlated with improvement in anterior chamber flare (p = 0.01).Conclusion: FSRD is a complication of chronic JIA-associated uveitis that responds well to intensification of immunomodulation. Visual improvement is correlated with FSRD resolution and with flare photometry values. [ABSTRACT FROM AUTHOR]

Purpose: Two atypical cases of ocular localizations of chronic granulomatous disease are reported.Methods: The first case is about a 22-year-old woman carrier of the disease who developed active intraocular inflammation and choroidal granulomas successfully treated by steroids. The second is about a 2-year-old boy consulting for unilateral anterior uveitis and subsequent anterior chamber granuloma development as first signs of the disease.Results: X-linked chronic granulomatous disease is a rare inherited primary immunodeficiency syndrome characterized by disorders of phagocytic cells resulting in recurrent infections and development of granulomas. Ophthalmological manifestations are not rare and are mainly represented by surface and intraocular inflammation with possible choroidal granulomas. The two cases reported here are atypical, one of active inflammation in a carrier and the other revealing the disease.Conclusion: Ophthalmologists must be aware of chronic granulomatous disease and the possible ocular involvement of this disorder. [ABSTRACT FROM AUTHOR]

Purpose: To report atypical ophthalmologic manifestations and complications of IgG4-related disease (IgG4-RD). Methods: Patients with isolated ophthalmologic involvement of IgG4-RD other than lacrimal or orbital infiltration seen between 2009 and 2011 in a single tertiary center were retrospectively reviewed and their clinical and histological features, treatment, and prognosis were studied. Case reports: Two patients (mean age 56.5 years) were included. One patient presented with recurrent anterior and posterior scleritis, and one patient had chronic conjunctival infiltration. Histopathology demonstrated lymphoplasmacytic proliferation with overexpression of IgG4+ plasma cells. Both patients initially responded to a high dose of oral corticosteroids (1 mg/kg/d). However, one patient required the adjunction of methotrexate and one patient developed an intra-epithelial conjunctival carcinoma on the site of the initial lesion. Conclusion: Patients with atypical presentation of IgG4-RD, such as chronic conjunctival infiltration or scleritis, can suffer from considerable diagnostic delay leading to fibrosis or malignancy development. We report the first case of conjunctival carcinoma in a patient with IgG4-RD. [ABSTRACT FROM AUTHOR]

Purpose: To present a population of patients sharing atypical manifestations of vitreoretinal lymphoma (VRL). Methods: Institutional case series in a single tertiary center. Patients with cytologically proven VRL, referred between November 2009 and May 2010, were retrospectively reviewed. Diagnosis of VRL was based on cytology of vitreous samples, immunohistochemistry, and molecular biology. Patients with confirmed VRL and clinical features different from the typical manifestations were included. Demographical and clinical characteristics of these patients were studied. Results: Twelve cases of VRL were diagnosed. Four cases (2M/2F) were considered atypical (A-VRL) in their presentations. Mean age for typical lymphoma (T-VRL) was 71.9 years (range: 62-87 years); mean age for A-VRL was 54 years (range: 50-59 years). Conclusions: Diagnosis of VRL is challenging and diagnostic delay is frequent. The authors describe a series of patients sharing common characteristics, such as a younger age ( p = 0.05), severe anterior chamber reaction, mild or no vitritis, and possible fulminant evolution. [ABSTRACT FROM AUTHOR]

Vitreous amyloidosis is an example of conformational diseases caused by the accumulation of unfolded or misfolded proteins [[1]]. Once suspected, diagnostic vitrectomy should be considered for patients with severe visual impairment, with close postoperative follow-up, as long as patients may develop post-vitrectomy glaucoma [[5]]. [Extracted from the article]

Purpose: To determine the incidence and to discuss pathogenic mechanisms of sympathetic ophthalmia in patients treated for choroïdal melanoma. Design: Retrospective Methods: We analyzed the clinical and pathological data of 4867 patients, treated for choroïdal melanoma at Institute Curie-Orsay, between 1998 and 2011. Were involved patients with uveitis of the adelphe eye filling clinical and angiographic criteria of sympathetic ophthalmia. Extensive work-up was undertaken to rule out another etiology of the inflammation.Results: Three patients developed sympathetic ophthalmia respectively 7, 4 and 3 years after protontherapy.The incidence of this complication was 6.1 per 10000 patients treated by proton beam radiation. Ocular perforating injury was ruled out and radioactive effect of treatment was incriminated in the occurrence of sympathetic ophthalmia. Conclusion: Sympathetic ophthalmia remains a rare complication which must be treated promptly and aggressively to prevent blindness.Proton beam irradiation could induce disruption of uveal tissue which enhances auto immune reactions. [ABSTRACT FROM AUTHOR]

Purpose: To describe four cases of familial Juvenile Idiopathic Arthritis (JIA) associated uveitis. Design: Retrospective observational case series. Methods: Clinical data from patients who present familial JIA-associated uveitis are described and possible genetic influence in disease etiology is reviewed. Results: Two sisters, 6- and 7-year-old, developed bilateral anterior uveitis related to oligoarticular JIA. Arthritis in both began by the age of 18 months; uveitis developed simultaneously in one of them and less than one year after arthritis in the other one. Both received topical and systemic treatment to control the ocular disease. Glaucoma occurred in both cases and bilateral cataract in one case. In the other family, a 2-year-old girl developed unilateral uveitis in the context of oligoarticular JIA which was diagnosed by 1-year-old. The disease became soon bilateral and she received topical and systemic immunosuppressive treatment. Her mother suffered from bilateral anterior uveitis associated to oligoarticular inflammatory disease during her infancy, later diagnosed as JIA-associated uveitis. Conclusions: Familial cases of JIA uveitis, although not very common, support the possible role of genetic influence in the pathogenesis of the disease, with horizontal and vertical inheritance being possible. The evolution of ocular disease is similar to the non-familial cases, except for an earlier age of onset. [ABSTRACT FROM AUTHOR]

Purpose: To report the value of endoretinal biopsy in establishing the diagnosis of severe posterior uveitis. Methods: A clinicopathologic report of three patients with severe posterior uveitis unresponsive to corticosteroids and with a negative systemic workup. These patients subsequently underwent vitrectomy and endoretinal biopsy. Results: Endoretinal biopsy provided an accurate diagnosis in the three cases, leading to appropriate treatment. Histopathologic examination of the three retinal samples confirmed the diagnosis of sarcoidosis, tuberculosis, and lymphoma. Targeted treatment improved the ocular conditions in all three cases. Conclusions: In selected cases of severe bilateral vision-threatening uveitis, retinal biopsy may be indicated as a last resort to confirm a suspected diagnosis of infectious disease or malignancy. [ABSTRACT FROM AUTHOR]

Purpose: To evaluate the clinical, angiographic, and cytopathologic features of ocular and central nervous system (CNS) lymphoma.Patients and Methods: Retrospective study of 44 patients over a 10-year period.Results: A total of 36 women and six men, mean age 54 years (range: 36-90 years), were included. The mean time interval between onset of ocular symptoms and diagnosis was 40 months (range: 1-144 months). Ocular involvement was bilateral in 84% of the cases. Laser flare photometry readings averaged 9.6 photons/ms (2.9-78.3 photons/ms). Vitritis was constant. Funduscopy revealed RPE abnormalities in 60.49% of the cases and punctuate retinal infiltrates in 33.5%. The most common findings with fluorescein angiography were window defects and hypofluorescent round lesions. Patients had CNS involvement in 66% of the cases. Cytologic examination of the vitreous samples showed high-grade B lymphoma in 86% of the cases. Interleukin-10 dosage, when performed, showed elevated levels averaging 2352 pg/ml in all vitreous samples. Molecular biology based on PCR confirmed the diagnosis in 12 patients. Treatment included systemic chemotherapy alone or associated with radiotherapy in various regimens. Fourteen patients died during follow-up. Only 12 patients were in complete remission.Conclusion: The prognosis of the disease remains poor. However, the new diagnostic tools and therapeutic strategies may improve the diagnostic delay and the survival outcome. [ABSTRACT FROM AUTHOR]