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Purpose: To report the clinical and fluorescein angiographic (FA) features of demyelinating plaque-associated uveitis (DPU), a subset of uveitis in which patients have demyelinating plaques on the brain/cervical magnetic resonance image (MRI) but do not meet the criteria for multiple sclerosis (MS)., Methods: In this retrospective observational study, Persian Patients were diagnosed with DPU and included if (1) they never satisfied the MS criteria, (2) all other possible etiologies were excluded, and (3) they were followed for at least 2 years., Results: After a median follow-up of 3 years (interquartile range, 2.0-5.3), 8 out of 40 (20%) patients diagnosed with DPU were excluded as they subsequently met the MS criteria. Of remaining 32 patients studied, the mean age was 36.3±9.9 (range 20-56 years), and 30 (93.8%) were female. Twenty-four (75.0%) showed bilateral involvement and 27 (84.4%) had insidious-chronic course. Uveitis was classified as intermediate (with or without anterior uveitis) in 29 (90.6%) and isolated anterior in 3 (9.4%) patients. Nine (28.1%) patients had at least one systemic neurological complaint. Ocular findings were: granulomatous keratic precipitates in 43/44 (97.7%) eyes; snowballs in 25/52 (48.1%) eyes; snowbanks in 4/52 (7.7%) eyes; cystoid macular edema in 20/56 (35.7%) eyes; and optic neuritis in 5/56 (8.9%) eyes. Visual acuity was ≥ 20/40 in 39 eyes (69.6%) at presentation which improved to 46 eyes (81.2%) at 2-year follow up. The two most frequent findings in FA were optic disc leakage/staining in 44/52 (81.5%) eyes, and peripheral retinal perivascular leakage in 39/52 (76.9%) eyes, which in 14/52 (26.9%) eyes extended beyond the equator., Conclusion: DPU usually presents as a bilateral chronic granulomatous intermediate and, less often, isolated anterior uveitis, especially in females. Most are neurologically asymptomatic. Visual outcome is generally favorable. In FA, peripheral retinal perivascular leakage is common. DPU patients have an increased tendency to develop MS and should be prohibited from anti-TNF treatment., (© 2023. The Author(s), under exclusive licence to Springer-Verlag GmbH Germany, part of Springer Nature.)

Aims: To present current expert practice patterns and to formulate a consensus for the management of HSV and VZV AU by uveitis specialists worldwide., Methods: A two-round online modified Delphi survey with masking of the study team was conducted. Responses were collected from 76 international uveitis experts from 21 countries. Current practices in the diagnosis and treatment of HSV and VZV AU were identified. A working group (The Infectious Uveitis Treatment Algorithm Network [TITAN]) developed data into consensus guidelines. Consensus is defined as a particular response towards a specific question meeting ≥75% of agreement or IQR ≤ 1 when a Likert scale is used., Results: Unilaterality, increased intraocular pressure (IOP), decreased corneal sensation and diffuse or sectoral iris atrophy are quite specific for HSV or VZV AU from consensus opinion. Sectoral iris atrophy is characteristic of HSV AU. Treatment initiation is highly variable, but most experts preferred valacyclovir owing to simpler dosing. Topical corticosteroids and beta-blockers should be used if necessary. Resolution of inflammation and normalisation of IOP are clinical endpoints., Conclusions: Consensus was reached on several aspects of diagnosis, choice of initial treatment, and treatment endpoints for HSV and VZV AU. Treatment duration and management of recurrences varied between experts., (© 2023. The Author(s), under exclusive licence to The Royal College of Ophthalmologists.)

Aims: To present current practice patterns in the diagnosis and management of Cytomegalovirus anterior uveitis (CMV AU) by uveitis experts worldwide., Methods: A two-round modified Delphi survey with masking of the study team was performed. Based on experience and expertise, 100 international uveitis specialists from 21 countries were invited to participate in the survey. Variation in the diagnostic approaches and preferred management of CMV AU was captured using an online survey platform., Results: Seventy-five experts completed both surveys. Fifty-five of the 75 experts (73.3%) would always perform diagnostic aqueous tap in suspected CMV AU cases. Consensus was achieved for starting topical antiviral treatment (85% of experts). About half of the experts (48%) would only commence systemic antiviral treatment for severe, prolonged, or atypical presentation. The preferred specific route was ganciclovir gel 0.15% for topical treatment (selected by 70% of experts) and oral valganciclovir for systemic treatment (78% of experts). The majority of experts (77%) would commence treatment with topical corticosteroid four times daily for one to two weeks along with antiviral coverage, with subsequent adjustment depending on the clinical response. Prednisolone acetate 1% was the drug of choice (opted by 70% of experts). Long-term maintenance treatment (up to 12 months) can be considered for chronic course of inflammation (88% of experts) and those with at least 2 episodes of CMV AU within a year (75-88% of experts)., Conclusions: Preferred management practices for CMV AU vary widely. Further research is necessary to refine diagnosis and management and provide higher-level evidence., (© 2023. The Author(s), under exclusive licence to The Royal College of Ophthalmologists.)

Cytomegalovirus (CMV) anterior uveitis is the most common form of ocular manifestation of CMV in immunocompetent individuals. The difficulty in diagnosing CMV anterior uveitis may delay adequate treatment and affect outcomes. We sought to review systemically the overall clinical characteristics and compare treatment outcomes in CMV anterior uveitis and endotheliitis. A literature search was performed, and studies describing clinical characteristics, treatment regimens, and outcomes that included more than 5 treated eyes were included. In these 23 studies, acute CMV anterior uveitis commonly presented with high intraocular pressure (95.31%, 95% CI 90.45-98.60) and mild anterior chamber inflammation (cells >2+ = 3.18%, 95% CI 0.21-0.54). About two-thirds of CMV endotheliitis cases presented with high intraocular pressure and coin-shaped corneal lesions. Acute CMV anterior uveitis showed good clinical response to topical 0.15% ganciclovir (GCV) gel or oral valganciclovir (VGCV) (90%, 95% CI 74-100% and 95%, 95% CI 88-100%, respectively). For chronic CMV anterior uveitis, both topical GCV and oral VGCV yielded comparable results. Topical 0.5-2% GCV or a combination of topical and oral VGCV for CMV endotheliitis both resulted in good clinical response. Recurrence of inflammation was common after cessation of maintenance therapy. Overall, topical GCV resulted in an optimal outcome for CMV anterior uveitis. Escalated concentration and frequency of usage are needed for chronic CMV anterior uveitis and endotheliitis. Adequate induction and maintenance phases of anti-CMV treatment seem necessary to prevent recurrences., (Copyright © 2021. Published by Elsevier Inc.)

Purpose: Viral agents are the most common cause of infectious anterior uveitis worldwide. The purpose of this review is to analyze the frequency, gender and racial differences of viral anterior uveitis (VAU) in various populations., Methods: Systematized literature review of epidemiological reports of VAU cited in PubMed, EMBASE and the Cochrane Library database published until June 30th, 2020., Results: A total of 12 clinical studies on epidemiology of definite VAU and 36 clinical studies of presumed VAU were identified. Members of Herpesviridae family represent the most common causes of VAU. Other less frequently reported causes, such as rubella and endemic viruses (HTLV-1, Chikungunya, Dengue, Ebola, Zika virus) were also analyzed., Conclusion: HSV, VZV are prevalent worldwide. CMV is more frequent in Asia, and rubella in the West. However, due to globalization and air travel, HTLV-1, Chikungunya, Dengue and Ebola may become important causes of VAU across the world.

The morphology of keratic precipitates (KPs) may yield important diagnostic clues. However, KPs have not been described in a standardized manner and the traditional classification of granulomatous or non-granulomatous KPs is not helpful in differentiating infectious from noninfectious uveitis. A granulomatous uveitis may initially appear non-granulomatous. We suggest three ways to examine KPs that may aid in differentiating the infectious from noninfectious etiologies. The first method is the in vivo confocal microscopy (IVCM) description and classification of KPs, in which the "non-granulomatous" subset of dendritiform and infiltrative KPs should be differentiated from smooth-rounded KPs and globular KPs which are "granulomatous." The second and third clues are the distribution and color of the KPs. KPs that extend beyond the midline may suggest an infective cause, and fresh pigmented KPs suggest a viral cause. Careful examination of the KPs may immediately reveal the clues to the diagnosis, minimizing unnecessary tests and costs.

Cytomegalovirus (CMV) causes clinical issues primarily in immune-suppressed conditions. CMV-associated anterior uveitis (CMV-AU) is a notable new disease entity manifesting recurrent ocular inflammation in immunocompetent individuals. As patient demographics indicated contributions from genetic background and immunosenescence as possible underlying pathological mechanisms, we analyzed the immunogenetics of the cohort in conjunction with cell phenotypes to identify molecular signatures of CMV-AU. Among the immune cell types, natural killer (NK) cells are main responders against CMV. Therefore, we first characterized variants of polymorphic genes that encode differences in CMV-related human NK cell responses (Killer cell Immunoglobulin-like Receptors ( KIR ) and HLA class I ) in 122 CMV-AU patients. The cases were then stratified according to their genetic features and NK cells were analyzed for human CMV-related markers (CD57, KLRG1, NKG2C) by flow cytometry. KIR3DL1 and HLA class I combinations encoding strong receptor-ligand interactions were present at substantially higher frequencies in CMV-AU. In these cases, NK cell profiling revealed expansion of the subset co-expressing CD57 and KLRG1, and together with KIR3DL1 and the CMV-recognizing NKG2C receptor. The findings imply that a mechanism of CMV-AU pathogenesis likely involves CMV-responding NK cells co-expressing CD57/KLRG1/NKG2C that develop on a genetic background of KIR3DL1/HLA-B allotypes encoding strong receptor-ligand interactions.

Viral anterior uveitis (VAU) needs to be suspected in anterior uveitis (AU) associated with elevated intraocular pressure, corneal involvement, and iris atrophic changes. Common etiologies of VAU include herpes simplex, varicella-zoster, cytomegalovirus, and rubella virus. Clinical presentations can vary from granulomatous AU with corneal involvement, Posner-Schlossman syndrome, Fuchs uveitis syndrome, and endothelitis. Due to overlapping clinical manifestations between the different viruses, diagnostic tests like polymerase chain reaction and Goldmann-Witmer coefficient analysis on the aqueous humor may help in identifying etiology to plan and monitor treatment., Competing Interests: None

A viral aetiology should be suspected when anterior uveitis is accompanied by ocular hypertension, diffuse stellate keratic precipitates or the presence of iris atrophy. The most common viruses associated with anterior uveitis include herpes simplex virus, varicella-zoster virus, cytomegalovirus and rubella virus. They may present as the following: Firstly, granulomatous cluster of small and medium-sized keratic precipitates in Arlt's triangle, with or without corneal scars, suggestive of herpes simplex or varicella-zoster virus infection. Secondly, Posner-Schlossman syndrome with few medium-sized keratic precipitates, minimal anterior chamber cells and extremely high intraocular pressure; this is mainly associated with cytomegalovirus. Thirdly, Fuchs uveitis syndrome, with fine stellate keratic precipitates diffusely distributed over the corneal endothelium, with diffuse iris stromal atrophy but without posterior synechiae, is associated mainly with rubella or cytomegalovirus infection. In rubella, the onset is in the second to third decade. It presents with posterior subcapsular cataract, may have iris heterochromia and often develops vitritis without macular oedema. Cytomegalovirus affects predominantly Asian males in the fifth to seventh decade, the keratic precipitates may be pigmented or appear in coin-like pattern or develop nodular endothelial lesions, but rarely vitritis. Eyes with cytomegalovirus tend to have lower endothelial cell counts than the fellow eye. As their ocular manifestations are variable and may overlap considerably, viral AU can pose a diagnostic dilemma. Thus, quantitative polymerase chain reaction or Goldmann-Witmer coefficient assay from aqueous humour samples are preferred to confirm the aetiology and determine the disease severity as this impacts the treatment., (© 2018 Royal Australian and New Zealand College of Ophthalmologists.)

Herpes simplex virus, varicella zoster virus, human cytomegalovirus, and rubella virus are the most common causes of virus-induced anterior uveitis. They can present in a variety of entities not only with typical but also overlapping clinical characteristics. These viral infections are commonly associated with ocular infiltration of T cells and B/plasma cells, and expression of cytokines and chemokines typical of a proinflammatory immune response. The infections differ in that the herpes viruses cause an acute lytic infection and inflammation, whereas rubella virus is a chronic low-grade infection with slowly progressing immunopathological responses. The outcome of an intraocular viral infection may largely be guided by the characteristics of the virus, which subsequently dictates the severity and type of the immune response, and the host immune status.

Diagnosis of uveitis is often challenging, but can be easy in typical viral-induced anterior uveitis (VIAU). Associated symptoms and signs are an important source of information. Certain classical clinical features such as keratic precipitates (KPs) distribution, iris atrophy, elevated intraocular pressure (IOP), and unilaterality are commonly used to support the diagnosis of VIAU. However, many etiologies of anterior uveitis may to a certain extent mimic VIAU, especially the ones with unilateral granulomatous KPs and elevated IOP. This review begins with how the clinician can differentiate viral from nonviral anterior uveitis, and subsequently focuses on the key features which may aid in differentiating among the different viruses that cause VIAU.

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.

Cytomegalovirus (CMV) anterior uveitis and endotheliitis occurs among immunocompetent individuals and may manifest as Posner-Schlossman syndrome or Fuchs uveitis syndrome. The condition may first present following ophthalmic surgery, the use of a fluocinolone-sustained steroid drug delivery implant, or the use of topical prostaglandin analogues for the treatment of glaucoma. We report the first case of a non-human immunodeficiency virus-infected individual who presented with CMV anterior uveitis after the use of topical cyclosporine A 0.05% ophthalmic emulsion for the treatment of symptomatic dry eyes.

Anterior uveitis is the most common group of uveitis worldwide, with a diverse spectrum ranging from autoimmune and infectious to masquerade etiologies. Elucidation of the underlying etiology may be challenging, but it remains important, especially for recurrent uveitis. Multimodal imaging has improved our understanding and management of many posterior uveitis and panuveitis. Similarly, a wide variety of anterior segment ophthalmic imaging techniques are available to allow the monitoring of the structural changes that may develop during anterior uveitis. These include anterior segment photography, specular microscopy, in vivo confocal microscopy, anterior segment optical coherence tomography (AS-OCT), ultrasound biomicroscopy (UBM), laser flare photometry, and less commonly used techniques such as anterior segment fluorescein and indocyanine green angiography, iris autofluorescence, and infrared imaging. This review recapitulates the past and recent application of these ophthalmic imaging modalities to the diagnosis and management of anterior uveitis.

Purpose: We sought to investigate and describe the clinical spectrum of posterior segment abnormalities in immunocompetent patients presenting with CMV-associated anterior uveitis., Methods: This was a prospective study conducted at the Singapore National Eye Centre, a tertiary referral centre, from August 2010 to June 2011. Eleven eyes of eleven patients with CMV anterior uveitis confirmed by polymerase chain reaction on aqueous humor sampling were recruited based on the study criteria. Patients were recruited from a single uveitis specialist clinic and underwent aqueous humor sampling and fluorescein and indocyanine green angiography as well as optical coherence tomography. They were further evaluated by the Infectious Disease physician for immunocompetence., Results: Mean presenting visual acuity was logMAR 0.35 ± 0.29. The main presenting complaints were blurring of vision, eye redness, and pain. Anterior chamber cellular activity was present in all cases. Fine diffuse keratic precipitates (KPs) were present in 10 eyes, and the remaining one eye had mutton fat KPs. Iris changes were present in three eyes. Intraocular pressure (IOP) was elevated in nine eyes (mean presenting IOP was 40.2 ± 16.8 mmHg). In the posterior segment, none of the eyes had evidence of retinitis or hemorrhage. Posterior segment abnormalities were present in six eyes (macular edema, disc leakage, epiretinal membrane, phlebitis). Eight eyes also had prolonged arm to retina time (mean 24.8 ± 10.6 s) on fluorescein angiography. Indocyanine green angiography was unremarkable., Conclusion: Posterior segment manifestations can be seen in a proportion of immunocompetent patients with CMV anterior uveitis. The underlying mechanism remains to be determined.

To describe clinical features, laboratory findings, course of disease and response to treatment in four patients diagnosed with Posner-Schlossman syndrome (PSS) who had evidence of cytomegalovirus (CMV) infection in their aqueous humour. Review of four consecutive CMV positive PSS patients seen at the Noor Eye Hospital. CMV was diagnosed by polymerase chain reaction analysis of their aqueous. All four patients were immunocompetent males. Mean age of onset was 44.5 years (range, 32-63 years). Maximum intraocular pressure ranged from 36 to 48 mmHg. Mild to moderate anterior chamber reaction, lack of posterior synechiae and posterior segment involvement were consistent features in all the patients. Three patients had diffuse iris atrophy. One patient with a stromal immune ring at presentation developed features of Fuch's heterochromic iridocyclitis (FHI) later in his course of disease. Heterochromia was also observed in another patient. All patients had a positive titre of IgG but not IgM against CMV, and none had concomitant CMV antigenemia. One patient who had recalcitrant uveitis and glaucoma and another patient who was suffering from frequent attacks and showed evidence of significant endothelial cell loss received anti-CMV therapy. In our population, PSS may be associated with CMV infection and may develop clinical features similar to FHI. Anti-CMV therapy should be reserved for cases with recalcitrant course or secondary complications.

Purpose: The aim of this study was to compare the antiinflammatory effect and ocular surface toxicity of topical nonpreserved methylprednisolone sodium succinate 1% and preserved prednisolone acetate suspension 1% for the management of acute anterior uveitis (AAU)., Methods: In this prospective, randomized, investigator-masked, comparative clinical trial, patients with mild-to-moderate noninfectious AAU were assigned randomly to receive either hourly nonpreserved methylprednisolone 1% (group A) or preserved prednisolone 1% (group B) eye drops followed by a 2-week tapering regimen. Anterior chamber cells and flare were clinically evaluated for the objective comparison of the antiinflammatory effect. The main outcome measure was the percentage of patients with a resolution of inflammation (anterior chamber cells <1+) on day 14. Ocular surface toxicity was assessed by means of the corneal fluorescein staining score, tear breakup time, Schirmer I test, and questionnaire-based grading of ocular discomfort parameters., Results: Seventy-two eyes of 68 patients were studied, of which 38 eyes were enrolled in group A and 34 eyes were enrolled in group B. On day 14, 76.3% of the patients in group A had resolution of inflammation compared with 70.6% of the patients in group B, proving noninferiority (χ = 0.303, P = 0.582). The mean anterior chamber cell grade reduction for patients in group A was similar to that in group B (2.52 vs. 2.86, respectively; P = 0.92). Group A patients showed significantly lower corneal fluorescein staining scores (P < 0.001) and reported milder subjective ocular discomfort (0.55 vs. 1.43, P = 0.01) as compared with group B., Conclusions: Both preparations demonstrated equal antiinflammatory effects for the treatment of AAU. Nonpreserved methylprednisolone eye drops exhibited a significantly lower ocular surface toxicity profile and milder subjective discomfort when compared with that exhibited by preserved prednisolone.

Purpose of Review: With improvements in molecular diagnostics tests, viruses are increasingly being found to be associated with what was previously described as idiopathic anterior uveitis. This review presents the clinical features of viral anterior uveitis., Recent Findings: Herpes simplex virus/varicella zoster virus (HSV/VZV) are important causes of anterior uveitis, but other viruses including cytomegalovirus (CMV) and rubella are also found in a significant number of cases. The various viral anterior uveitides have similar features and should be suspected in eyes with diffuse, fine, stellate keratic precipitates, iris atrophy or ocular hypertension. Acyclovir remains the mainstay of therapy in HSV/VZV infections. CMV responds to ganciclovir, but the relapse rate is high and prolonged therapy may be required. Cataract and glaucoma are the main complications of viral anterior uveitis requiring appropriate management., Summary: There is considerable overlap between clinical manifestations of the different viral anterior uveitides as well noninfectious hypertensive anterior uveitis syndromes. Hence, corticosteroids should be used with caution in these eyes if virus diagnostic tests are not available.

Purpose: To describe immune ring (IR) formation as a manifestation of cytomegalovirus (CMV) endotheliitis., Design: Retrospective observational case series., Methods: Review of all consecutive CMV anterior uveitis and endotheliitis patients seen at the Singapore National Eye Centre for the occurrence of an IR. CMV infection was diagnosed by polymerase chain reaction analysis of the aqueous., Results: None of the 72 eyes with CMV anterior uveitis had an IR formation. Four episodes of IR formation were seen in 3 eyes (14.3%) of 21 cases of CMV-positive endotheliitis. All were unilateral and all 3 patients were human immunodeficiency virus negative middle-aged Chinese males. The IR developed 2 to 7 months after occurrence of the endotheliitis and the aqueous was positive for CMV during 2 of the episodes of IR formation. In 2 eyes, the IR occurred at the completion of a course of systemic ganciclovir when the aqueous was negative for CMV. The immune rings resolved with combination therapy of topical prednisolone acetate 0.12% and ganciclovir. Patient 1 had a recurrence of the IR 4 months after stopping treatment but again resolved following treatment with ganciclovir and topical corticosteroids., Conclusion: Corneal immune rings can occur as a result of CMV infection. Hence CMV infection may have to be considered in such cases., (Copyright © 2011 Elsevier Inc. All rights reserved.)

A 61-year-old male developed recurrent anterior uveitis over a period of 8 months after an uncomplicated phacoemulsification and posterior chamber intraocular lens implantation surgery. This was subsequently found to be due to a retained lens fragment in the anterior segment, with complete resolution following surgical extraction of the fragment. To our knowledge, this is the first report of recurrent anterior uveitis attributable to a retained lens fragment following uncomplicated cataract surgery, and the diagnosis should be considered in a pseudophakic patient presenting with recurrent episodes of anterior uveitis.

Purpose: To compare the characteristics of cytomegalovirus (CMV)-positive and negative eyes with presumed Posner-Schlossman syndrome (PSS) and Fuchs heterochromic iridocyclitis (FHI)., Design: Retrospective interventional case series., Methods: One hundred and three eyes of 102 patients with presumed PSS or FHI, seen at the Singapore National Eye Centre, underwent aqueous analysis for CMV by polymerase chain reaction (PCR). Their records were reviewed for clinical features and human immunodeficiency virus (HIV) status of the CMV-positive patients. The main outcome measures were age, gender, maximum intraocular pressure, endothelial cell count, endothelial changes, PCR results, and presence of uveitic cataract and/or glaucoma., Results: Sixty-seven eyes with presumed PSS were tapped, of which 35 (52.2%) were CMV-positive. There were 36 eyes of 35 patients with presumed FHI, of which 15 (41.7%) were CMV-positive. All the CMV-positive patients were HIV negative. Nodular endothelial lesions were seen in 18 eyes (36.0%) with CMV infection, and reticulate deposits were seen in all the presumed FHI eyes. CMV-positive and CMV-negative PSS eyes were clinically similar. Older age at diagnosis, male gender, and nodular endothelial lesions were significantly associated with CMV infection in presumed FHI eyes (age: odds ratio [OR], 1.1; 95% confidence interval [CI], 1.0 to 1.2; P = .01; male gender: OR, 9.4; 95% CI, 1.0 to 88.6; P = .049; nodular endothelial lesions: OR, 13.9; 95% CI, 1.5 to 132.7; P = .02)., Conclusions: There are no clinically detectable differences between CMV-positive and negative presumed PSS eyes. CMV-positive presumed FHI patients are more likely to be male, older at diagnosis or have nodular endothelial lesions.

Purpose: To report the clinical and fluorescein angiographic (FA) features of demyelinating plaque-associated uveitis (DPU), a subset of uveitis in which patients have demyelinating plaques on the brain/cervical magnetic resonance image (MRI) but do not meet the criteria for multiple sclerosis (MS). Methods: In this retrospective observational study, Persian Patients were diagnosed with DPU and included if (1) they never satisfied the MS criteria, (2) all other possible etiologies were excluded, and (3) they were followed for at least 2 years. Results: After a median follow-up of 3 years (interquartile range, 2.0–5.3), 8 out of 40 (20%) patients diagnosed with DPU were excluded as they subsequently met the MS criteria. Of remaining 32 patients studied, the mean age was 36.3±9.9 (range 20–56 years), and 30 (93.8%) were female. Twenty-four (75.0%) showed bilateral involvement and 27 (84.4%) had insidious-chronic course. Uveitis was classified as intermediate (with or without anterior uveitis) in 29 (90.6%) and isolated anterior in 3 (9.4%) patients. Nine (28.1%) patients had at least one systemic neurological complaint. Ocular findings were: granulomatous keratic precipitates in 43/44 (97.7%) eyes; snowballs in 25/52 (48.1%) eyes; snowbanks in 4/52 (7.7%) eyes; cystoid macular edema in 20/56 (35.7%) eyes; and optic neuritis in 5/56 (8.9%) eyes. Visual acuity was ≥ 20/40 in 39 eyes (69.6%) at presentation which improved to 46 eyes (81.2%) at 2-year follow up. The two most frequent findings in FA were optic disc leakage/staining in 44/52 (81.5%) eyes, and peripheral retinal perivascular leakage in 39/52 (76.9%) eyes, which in 14/52 (26.9%) eyes extended beyond the equator. Conclusion: DPU usually presents as a bilateral chronic granulomatous intermediate and, less often, isolated anterior uveitis, especially in females. Most are neurologically asymptomatic. Visual outcome is generally favorable. In FA, peripheral retinal perivascular leakage is common. DPU patients have an increased tendency to develop MS and should be prohibited from anti-TNF treatment. [ABSTRACT FROM AUTHOR]