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Purpose: To compare clinical profile and visual outcomes of occlusive versus non-occlusive retinal vasculitis (RV)., Methods: A retrospective comparative study., Results: 284 patients were enrolled, including 124 patients with occlusive RV (ORV) and 160 patients with non-occlusive RV (NORV). Patients with ORV were older (p ≤ 10-3), predominantly male (p ≤10-3), with less bilateral involvement (31.5% vs 53,4%; p ≤ 10 -3 ). Infectious RV was more frequently diagnosed in the ORV group than in the NORV group (48.8% vs 32.9%, p = .006). Behçet disease and ocular tuberculosis were the leading causes of ORV. Idiopathic RV, Behçet disease, and sarcoidosis were the most common causes of NORV. Independent predictive factors of poor visual outcome were worse baseline visual acuity in both groups ( p = .006 and p ≤ 10-3, respectively), and retinal hemorrhages ( p = .048) and optic atrophy ( p = .040) in the ORV group., Conclusion: Occlusive and non-occlusive RV have distinctive clinical and etiological profile.

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

Purpose: To investigate the epidemiological and clinical profiles in retinal vasculitis in an Asian cohort., Methods: A 5-year retrospective study of 487 uveitis patients in a tertiary referral center at northern Taiwan., Results: Overall, 18.5% of the cases were associated with retinal vasculitis (RV). Sarcoidosis and cytomegalovirus retinitis were two leading diagnoses. Cases with RV were younger (mean: 39.7 years), less unilateral (50%), and had more chorioretinal involvement (95.5%). Definite diagnosis was reached in 86.7% of RV cases, of which 38.5% had infectious uveitis. While sole arteritis was presented in 8.9% of cases, 63.3% showed phlebitis, and 27.8% involved both. Three distinctive patterns were associated with a higher likelihood of infection: nodular sheathing, continuous perivascular sheathing (OR 4.79), and continuous fluorescein leakage (OR 4.11)., Conclusion: The presence of RV, especially arteritis, is highly suggestive of identifiable etiology. Distinctive clinical patterns help differentiation of infectious versus noninfectious causes.

Aim: To look at markers to differentiate ocular sarcoidosis from ocular tuberculosis in a high TB endemic population., Materials & Methods: Retrospective multicenter study involving seven tertiary eyecare centers in India between July 2016 to December 2016. Demographic, clinical and laboratory data were retrieved from respective hospital databases. Group A included biopsy-proven ocular sarcoidosis while group B included ocular tuberculosis., Results: Data from 2726 consecutive patients with uveitis were retrieved from the databases. Group A had 61 cases while group B included 307 cases. The mean age in group A was 43 ± 16.55 years while in group B was 38 ± 13.13 years. Females were more common in group A. Uveitis with low Schirmer test (OR-30, CI-95%), candlewax retinal vasculitis (OR-8.69, CI-95%), hilar lymphadenopathy (OR-15.23, CI-95%), fissural nodules (OR-39.11, CI-95%) had higher odds of having ocular sarcoidosis., Conclusion: Presence of dry eye, candlewax retinal vasculitis, hilar lymphadenopathy, and fissural nodules if present in a patient with uveitis, could help differentiate ocular sarcoidosis from ocular tuberculosis in a high TB endemic population.

Purpose: To characterize the clinical features of occlusive retinal vasculitis (ORV)., Design: Retrospective case series., Participants: Forty-two patients with ORV., Methods: A retrospective chart review identified all patients with ORV seen at the University of Colorado uveitis service between January 2013 and April 2020. All included patients demonstrated noninfectious uveitis and evidence of vascular occlusion in the presence of retinal vascular inflammation on widefield fluorescein angiography., Main Outcome Measures: Demographic data, visual acuity, clinical findings, and fluorescein angiography findings., Results: We identified 73 eyes from 42 patients (15 men, 27 women) with ORV. Thirty-one of 42 patients had bilateral disease. Most eyes (54/73) showed mixed arteriolar and venous vasculitis compared with primarily arteriolar (6/73) or venous (15/73) vasculitis. Thirteen of 42 patients had an underlying systemic condition, most commonly granulomatosis with polyangiitis; however, bilaterality was not associated with a systemic condition. Retinal nonperfusion was present equally in zone 2 (28/73) and zone 3 (28/73) compared with zone 1 (16/73). Retinal or iris neovascularization was present in 25 of 73 eyes. Eighteen of 42 patients required more than 1 immunosuppressive medication (average, 1.33) to prevent progressive vascular occlusive disease., Conclusions: Occlusive retinal vasculitis is a heterogeneous entity with significant risk of visual impairment. Systemic disease was more prevalent in this specific cohort compared with cohorts from prior studies of retinal vasculitis., (Copyright © 2021 American Academy of Ophthalmology. Published by Elsevier Inc. All rights reserved.)

Purpose: An independent Safety Review Committee (SRC), supported by Novartis Pharma AG, analyzed investigator-reported cases of intraocular inflammation (IOI), endophthalmitis, and retinal arterial occlusion in the phase 3 HAWK and HARRIER trials of brolucizumab versus aflibercept in neovascular age-related macular degeneration (nAMD)., Design: A post hoc analysis of a subset of data from two 2-year, double-masked, multicenter, active-controlled randomized phase 3 trials (NCT02307682, NCT02434328)., Participants: Patients (N = 1817) with untreated, active choroidal neovascularization due to age-related macular degeneration in the study eye were randomized and treated in HAWK/HARRIER. The SRC reviewed data from cases of investigator-reported IOI (60/1088 brolucizumab-treated eyes; 8/729 aflibercept-treated eyes)., Methods: The SRC received details and images (color fundus photography, fluorescein angiography, and OCT) for all investigator-determined cases of IOI, retinal arterial occlusion, and endophthalmitis. Cases were reviewed in detail by ≥2 readers, then adjudicated by the SRC as a group., Main Outcome Measures: Within this patient subset: incidence of IOI, signs and incidence of retinal vasculitis and/or retinal vascular occlusion, and visual acuity loss; time since first brolucizumab injection to IOI event onset; and frequency of visual acuity loss after brolucizumab injection by time of first IOI event onset., Results: Fifty brolucizumab-treated eyes were considered to have definite/probable drug-related events within the spectrum of IOI, retinal vasculitis, and/or vascular occlusion. On the basis of these cases, incidence of definite/probable IOI was 4.6% (IOI + vasculitis, 3.3%; IOI + vasculitis + occlusion, 2.1%). There were 8 cases (incidence 0.74%) of at least moderate visual acuity loss (≥15 ETDRS letters) in eyes with IOI (7 in eyes with IOI + vasculitis + occlusion). Of the 8 cases, 5 experienced their first IOI-related event within 3 months of the first brolucizumab injection (increasing to 7/8 within 6 months). Incidence of IOI in aflibercept-treated eyes was 1.1%, with at least moderate visual acuity loss in 0.14%., Conclusions: This analysis of IOI cases after brolucizumab injection identified signs of retinal vasculitis with or without retinal vascular occlusion and an associated risk of visual acuity loss. The findings will help physicians to evaluate the risks and benefits of brolucizumab treatment for nAMD., (Copyright © 2020 American Academy of Ophthalmology. Published by Elsevier Inc. All rights reserved.)

Purpose: This study presents clinical features and prognosis after long-term (12-18 months) antitubercular therapy (ATT) in patients with ocular tuberculosis (OTB) in East China, an endemic area of tuberculosis. Methods: This retrospective study reviewed data from OTB patients treated at the Eye and ENT Hospital of Fudan University from 2008 to 2018. All the patients completed a minimum follow-up of 6 months after the cessation of ATT. Results: Sixty-six patients with OTB were studied. The ocular manifestations included retinal vasculitis (51.6%), choroiditis (24.2%), panuveitis (23.2%), intermediate uveitis (7.4%), scleritis (5.3%), anterior uveitis (2.1%), and optic neuropathy (1%). Except for two patients (ATT for 6 months), all other patients (64/66, 96.97%) received ATT for at least 12 months (6 patients for 12 months, 30 patients for 15 months, and 28 patients for 18 months). Treatment in conjunction with oral corticosteroids was used in 48 patients (72.7%). The average initial best-corrected visual acuity (BCVA) was 0.8 ± 0.64 (LogMAR), which improved to 0.31 ± 0.35 (LogMAR) at the last follow-up ( P  < 0.05). The final BCVA was significantly associated with the initial BCVA and the duration of clinical symptoms. A complete remission of uveitis was achieved in 97% of the patients. Conclusions: This study observed a favorable prognosis with long-term ATT regimens. Patients with better baseline visual acuity and a shorter duration of clinical symptoms before diagnosis had a better prognosis.

Purpose: To determine clinical signs suggestive of tubercular etiology in retinal vasculitis., Methods: A retrospective comparative study of patients who presented with retinal vasculitis at three tertiary care centers in India. All patients underwent detailed clinical evaluation and tailored laboratory investigations for etiological diagnosis. Tubercular etiology was diagnosed on basis on the presence of retinal periphlebitis in association with ancillary evidence of systemic tuberculosis and exclusion of nontuberculosis entities. Patients with tubercular (Group A) and nontubercular (Group B) etiology were compared for demographic characteristics, supportive diagnostic evidence, and specific ocular signs. Statistical analysis was performed at 5% confidence levels., Results: Of the 114 patients diagnosed with retinal vasculitis, Group A had 69 patients (100 eyes) and Group B had 45 patients (75 eyes). Active or healed subvascular lesions (P ≤ 0.0001), focal vascular tortuosity (P ≤ 0.0001), and occlusive vasculitis (P = 0.002) were significantly more common in Group A patients than in Group B patients. All three were independent predictors of tubercular etiology on multivariate regression analysis., Conclusion: The presence of healed or active subvascular lesions, focal vascular tortuosity, and occlusive vasculitis could be predictive of tubercular etiology in retinal vasculitis.

Objective: To characterize the trends and explore the demographics, ophthalmic manifestations, and outcomes of ocular syphilis cases in British Columbia., Design: Retrospective chart review., Participants: Ocular syphilis cases reported to the British Columbia Centre for Disease Control (BCCDC) between January 2013 and December 2016., Methods: The demographic and clinical data were extracted from the BCCDC's centralized sexually transmitted infection database and the ophthalmologists' clinical charts., Results: There was a steady increase in the rate of syphilis infection per 100 000 population, from 3.4 in 2010 to 18.4 in 2018. There were 39 ocular syphilis cases identified from January 2013 to December 2016. The median age was 50 years (interquartile range: 40-59.5 years); 82.1% were male and 51.3% were HIV positive. The clinical charts belonging to 32 patients were available for review, 14 of which (43.8%) presented with bilateral ocular complaints (46 affected eyes). The most commonly noted ocular presentations were uveitis (93.5%), including retinal vasculitis in 54.3%, and optic nerve involvement in 65.2% (which included papillitis, optic nerve swelling, or pallor). Panuveitis was the most frequent type of uveitis (52.2% of all eyes); 77.8% of affected eyes with best-corrected visual acuity (BCVA) ≤20/50 on presentation had an improvement of 2 or more Snellen lines of visual acuity at their final assessment. At presentation, 37.0% of eyes had BCVA ≤20/200, which decreased to 17.1% at final assessment., Conclusion: Ocular syphilis, although rare, is on the rise globally and can result in serious ocular sequelae. A high index of suspicion is required for proper diagnosis and treatment., (Copyright © 2019 Canadian Ophthalmological Society. Published by Elsevier Inc. All rights reserved.)

Introduction: TB has seen resurgence associated with HIV. Tuberculosis can affect any ocular tissue. The association of HIV with TB is supposed to increase the incidence and plethora of ocular manifestations in tuberculosis., Objectives: To study the various ocular manifestations seen in tuberculosis patients with associated HIV infection., Material and Methods: This hospital based, cross sectional descriptive study was conducted in Tribhuvan University, Teaching Hospital, Maharajgunj, Nepal and Geta Eye Hospital, Kailali from 2010 to 2015. Diagnosed cases of pulmonary and extra pulmonary tuberculosis with HIV co infection were evaluated for ocular manifestations after excluding other opportunistic infections., Results: Of 70 cases eligible for the study, extra pulmonary tuberculosis was seen in60% of the cases. 5 patients (7.1 %) had ocular manifestations. CD4 counts were <50/mm3 in 3 cases. Ocular involvement was seen in the form of choroidal granulomas, papillitis, cranial nerve palsy, retinal vasculitis and central serous chorioretinopathy., Conclusion: This study demonstrated that ocular involvement is a frequent finding in cases with tuberculosis and HIV. Ocular findings are more common in cases with lesser CD4 counts. As ocular tuberculosis can be visually devastating, we recommend regular ocular evaluation of all patients with HIV and systemic tuberculosis., (© NEPjOPH.)

Behçet's disease is a chronic multisystem disorder characterized by relapsing inflammation for which the underlying histopathology is an occlusive vasculitis. The disease has a high prevalence in the Mediterranean region, Far and Middle Eastern countries. It is clinically characterized by oral and genital ulcerations, ocular inflammatory involvement, skin lesions, vascular involvement and numerous other systemic manifestations. Uveitis is by far the most common ocular manifestation of Behçet's disease. It typically presents in the form of relapsing-remitting panuveitis with retinal vasculitis that may result in severe visual loss. Management of Behçet's uveitis relies on corticosteroid therapy, conventional immunosuppressive drugs and biological agents and requires a close collaboration between ophthalmologist and internist. A better understanding of auto-inflammatory process and of the role of cytokines implicated in the pathogenesis of Behçet's disease will enable researchers to develop more specific and effective therapy., (Copyright © 2019 Elsevier Masson SAS. All rights reserved.)

Sarcoidosis is a systemic granulomatosis characterized by the formation of epithelioid and giant cell granulomas without caseous necrosis. To make the diagnosis, it is necessary to prove systemic granulomatosis involving at least two organs; but in practice, a combination of clinical, paraclinical and histologic findings is used. It affects predominantly women with a bimodal age distribution: 25-29years and 65-69years. The most commonly affected organs are the mediastinal lymphatic system, lungs, skin and eyes. Ophthalmological involvement is present in 20 to 50% of cases. The typical ocular presentation is that of granulomatous uveitis associated with venous retinal vasculitis and lesions of peripheral multifocal choroiditis. This ophthalmological presentation, although very evocative, is not always associated with systemic disease. The diagnosis of ocular sarcoidosis is then presumed in the absence of histological evidence. Algorithms combining ophthalmological and systemic signs have been proposed in cases of isolated uveitis. They make it possible to establish the diagnosis of ocular sarcoidosis with various levels of probability. The absence of significant granulomas on a systemic level during primary ocular involvement remains the main hypothesis to explain these diagnostic difficulties. Treatment is well described, as the uveitis of sarcoidosis is most often steroid responsive. In the case of corticosteroid-dependent uveitis, the first-line immunosuppressant remains methotrexate. The use of anti-tumor necrosis factor-alpha is an interesting alternative in patients whose ocular sarcoidosis is refractory to conventional immunosuppressants., (Copyright © 2019 Elsevier Masson SAS. All rights reserved.)

Purpose: To describe the clinical experience with retinal vasculitis at 4 Egyptian tertiary eye centers. Methods : A multicenter retrospective chart review of all patients with retinal vasculitis encountered at 4 ocular inflammation referral clinics in Egypt between February 2013 and February 2018. Results : The study included 618 patients (327 males and 291 females). Of these, 284 patients had isolated retinal vasculitis, whereas 233 patients had an associated systemic inflammatory disease, the most frequent being Behçet's disease, followed by sarcoidosis. In 101 patients, retinal vasculitis could be attributed to an infectious etiology, and among this category, the most common was tuberculosis, followed by toxoplasmosis. Conclusion : In our Egyptian cohort, more than half the patients had their retinal vasculitis as part of a serious systemic disease, or as part of an infectious process, as tuberculosis. And in view of the significant potential morbidity of these conditions, the local ophthalmologists need to be aware of these entities when faced with patients presenting with retinal vasculitis.

Importance: In a subset of patients with retinal vasculitis, there is occlusion of blood flow through the retinal vessels. These eyes are at high risk of sight-threatening complications., Background: To characterize epidemiology, clinical course, treatment and outcomes of occlusive retinal vasculitis (ORV)., Design: Retrospective study PARTICIPANTS: Seventy-seven uveitis patients with ORV at two large tertiary-care institutions (the USA and India)., Methods: Out of 2438 patients screened, 346 patients were diagnosed with retinal vasculitis of which 77 patients (96 eyes) were diagnosed with ORV. Patients with ORV (capillary, arteriolar and/or venular) were further analysed. Diagnostic criteria for occlusive vasculitis included (i) absence of blood flow in vessels (arterioles, venules and/or capillaries), (ii) capillary non-perfusion areas and/or arteriolar-venous anastomosis and (iii) intraretinal haemorrhages, cotton-wool spots or vitreous haemorrhage., Main Outcome Measures: Best-corrected visual acuity, treatment and complications., Results: The mean age was 32.09 ± 13.51 years. Most common aetiologies were tuberculosis and Adamantiades-Behçet's disease in India and systemic lupus erythematosus in the USA. Best-corrected visual acuity improved from 0.38 ± 0.30 logMAR (20/48 Snellen equivalent) (baseline) to 0.25 ± 0.30 (20/35 Snellen equivalent) at final visit (P < 0.0001). Vitreous haemorrhage was seen in 31.08% eyes. Pars plana vitrectomy was performed in 12.16% eyes. Therapy with systemic steroids was required in 78.48% patients. In addition, 46.75% patients required immunomodulators and/or biologics., Conclusions: Occlusive retinal vasculitis is caused by heterogeneous group of uveitides depending upon the geographic location. It is imperative to identify eyes with ORV as they are predisposed to complications requiring aggressive therapy., (© 2017 Royal Australian and New Zealand College of Ophthalmologists.)

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

Introduction: Rickettsial infections are re-emerging in the Indian subcontinent, especially among children. Understanding geographical and clinical epidemiology will facilitate early diagnosis and management., Methodology: Children aged <18yrs hospitalized with clinically-diagnosed rickettsial fever were reviewed retrospectively. Frequency distributions and odds ratios were calculated from tabulated data., Results: Among 262 children hospitalized between January 2008-December 2012, median age was five years, and 61% were male children. Hospitalized cases increased steadily every year, with the highest burden (74%) occurring between September and January each year. Mean duration of fever was 11.5 days. Rash was present in 54.2% (142/262) of children, with 37.0% involving palms and soles. Prevalence of malnutrition was high (45% of children were underweight and 28% had stunting). Retinal vasculitis was seen in 13.7% (36/262), and the risk appeared higher in females. Severe complications were seen in 29% (purpura fulminans, 7.6%; meningitis and meningoencephalitis, 28%; septic shock, 1.9%; acute respiratory distress syndrome, 1.1%). Complications were more likely to occur in anemic children. Positive Weil-Felix test results (titers ≥1:160) were seen in 70% of cases. Elevated OX-K titers suggestive of scrub typhus were seen in 80% (147/184). Patients were treated with chloramphenicol (32%) or doxycycline (68%). Overall mortality among hospitalised children was 1.9%., Conclusions: This five-year analysis from southern India shows a high burden and increasing trend of rickettsial infections among children. The occurrence of retinal vasculitis and a high rate of severe complications draw attention to the need for early diagnosis and management of these infections.

The syndrome of recurrent vitreous hemorrhages in young men was described for the first time by Henry Eales in 1880. The association with a clinical manifestation of ocular inflammation was reported 5years later. Eales disease affects young adults who present with ischemic retinal vasculitis, with the peripheral retina most commonly affected. Most cases have been reported in South Asia. Although the etiology of this abnormality is unknown, it may be related to an immune sensitivity to Mycobacterium tuberculosis antigens. Its pathogenesis is related to extensive ischemia that affects the retina, secondary to an obliterative retinal vasculopathy with release of angiogenic factors of the VEGF type. Involvement of the retina is the hallmark of the disease, which manifests as follows: periphlebitis, retinal capillary ischemia most often affecting the periphery with secondary proliferative retinopathy and retinal and/or papillary neovascularization, recurrent vitreous hemorrhages and tractional retinal detachment. These complications are potentially blinding. The natural history of Eales disease varies, with temporary or permanent remission in some cases and continuous progression in others. Progression is often bilateral, which necessitates regular follow-up. The treatment of Eales disease depends on the stage of the disease and is not well defined. Observation only, pars plana vitrectomy surgery and/or intravitreal injections of anti-VEGF are recommended in cases of vitreous hemorrhage, associated with corticosteroids when retinal vasculitis is present. Laser pan-retinal photocoagulation is necessary when neovascularization is present., (Copyright © 2016 Elsevier Masson SAS. All rights reserved.)

The primary aim of the study was to evaluate the incidence of de novo major involvement during follow-up in a cohort of patients with Behçet's syndrome (BS); the secondary aim was to analyse the epidemiological profile and the long-term outcome of those patients who developed new major involvement. Among our cohort of 120 BS patients, we evaluated all subjects who had no major organ involvement during the early years of their disease; specifically, at disease onset, the 52% of the cohort presented a prevalent mucocutaneous involvement. The primary outcomes were represented by the following: Hatemi et al. (Rheum Dis Clin North Am 39(2):245-61, 2013) the incidence of de novo major involvement during the follow-up and Hatemi et al. (Clin Exp Rheumatol 32(4 Suppl 84):S112-22, 2014) the use of immunosuppressive drugs during the follow-up. We have defined the development of de novo major involvement during the follow-up as the occurrence of severe ocular, vascular or CNS involvement after a latency period from the diagnosis of at least 3 years. Among 62 patients characterized by a mild onset of disease, we observed that after at least 3 years from the diagnosis, 21 BS patients (34%) still developed serious morbidities. Specifically, three patients developed ocular involvement, nine patients developed neurological involvement and nine patients presented vascular involvement. Comparing main epidemiological and clinical findings of the two groups, we observed that patients who developed de novo major involvement were more frequently males and younger; furthermore, 95% of these patients were characterized by a young onset of disease (p < 0.001). Being free of major organ complication in the first years of BS is not necessary a sign of a favourable outcome. Globally, the development of de novo major involvement during the coursfce of BS suggests that a tight control is strongly recommended during the course of the disease.

Background/aims: Estimated glomerular filtration rate (eGFR) is used extensively in epidemiological research. Validations of eGFR have demonstrated acceptable performance, but the dependence of creatinine and cystatin C on non-GFR factors could confound associations with disease. Few studies have investigated this issue in direct comparison with measured GFR (mGFR). We compared the associations between eGFR and mGFR and retinal vasculopathy, a marker of systemic microvasculopathy., Methods: Iohexol clearance and retinal photography were examined in the Renal Iohexol Clearance Survey in Tromsø 6, which consists of a representative sample of middle-aged persons from the general population. A total of 1,553 persons without self-reported kidney disease, cardiovascular disease or diabetes were investigated. Three eGFR equations based on creatinine and/or cystatin C from the Chronic Kidney Disease Epidemiology Collaboration were studied. Differences between eGFR and mGFR were analyzed with seemingly unrelated regression methods., Results: mGFR in the lowest quartile was associated with an increased multivariable-adjusted odds ratio of retinopathy (OR 1.86, 95% CI 1.16-2.97), but not with retinal artery or vein diameters. eGFR based on cystatin C (eGFRcys) was consistently biased relative to mGFR in its associations with retinal vessel diameters across different models. eGFR based on creatinine (eGFRcrea) and eGFR based on both creatinine and cystatin C were also biased in several of these models (p < 0.05). For retinopathy, the differences between the 3 eGFR and mGFR measurements were not statistically significant., Conclusions: Low mGFR is associated with retinopathy in the general population. eGFR based on creatinine and/or cystatin C are not valid substitutes for mGFR in studies of the relationship between the retina and kidney function in healthy persons., (© 2015 S. Karger AG, Basel.)

Purpose: To assess tuberculous uveitis in Chinese patients., Methods: The hospital-based observational case series study included patients who attended a third-referral hospital and presented with chronic and recurrent uveitis without primarily detected aetiology. The patients underwent the tuberculin skin test (TST) and/or interferon gamma release test (IGRA). Patients with positive test results received standard antituberculous therapy. Patients who responded to the therapy and did not show recurrence of uveitis in the follow-up period were diagnosed as tuberculous uveitis and formed the study group. The remaining patients were diagnosed as non-tuberculous uveitis and formed the control group. The clinical characteristics were compared between both groups., Results: The study group with tuberculous uveitis included 46 patients and the non-tuberculous group 38 patients. Multifocal choroiditis [n = 9 (20%) versus n = 1(3%); p = 0.04] and retinal vasculitis [n = 25(54%) versus 8 = (21.1%); p = 0.002] were significantly more common in the study group. Of 25 patients with retinal vasculitis in the study group, 11 patients (44%) additionally showed choroiditis lesions, compared with only one (13%) of eight patients in the control group (p = 0.01). In multivariate regression analysis, multifocal choroiditis [odds ratio (OR): 32.1], choroidal granuloma (OR: 21.4) and retinal vasculitis (OR: 11.2) were independent predictors of tubercular uveitis., Conclusions: About 50% of a group of 84 patients with primarily unexplained chronic posterior uveitis had tuberculosis and showed multifocal choroiditis, choroidal granuloma and retinal vasculitis. These features had a high predictive value for the diagnosis of tuberculous uveitis. Tuberculosis is an important part in the differential diagnosis of unexplained uveitis., (© 2014 Acta Ophthalmologica Scandinavica Foundation. Published by John Wiley & Sons Ltd.)

Purpose: To report on the clinical features and etiology of patients with retinal vasculitis (RV)., Materials and Methods: We reviewed medical records of 47 patients (75 affected eyes) diagnosed with RV. Clinical presentations, ocular complications, associated systemic diseases, and treatment regimens were registered., Results: Etiology of RV included infectious causes in 10/47, (21%) while an association with systemic and/or ocular non-infectious disorders was noted in 22/47 (47%). Eales' disease and Behcet's disease represented the most common clinical entities in non-infectious group while tuberculosis-associated RV was diagnosed in 6/10 (60%) among those with infectious disorders. RV was bilateral in 28/47 (60%) patients. Retinal veins were most commonly affected (72%, 34/47). Involvement of arteries was present in 12/47 (25%) and was associated with viral infections and Behcet's disease. Ocular complications developed in 60/75 (80%) eyes. The most common complications were elevated intraocular pressure and/or glaucoma (33/75, 44%). Retinal detachment, vitreous hemorrhage, and cystoid macular edema developed in similar percentages (15%)., Conclusions: RV in Thailand manifested mostly in male patients, was typically bilateral and involved mostly veins. Involvement of arteries was observed in patients with viral infections and Behcet's disease. Tuberculosis was the most common infectious cause.

Purpose: To analyze the incidence and clinical course of patients developing progressive ocular inflammation following anti-tubercular therapy (ATT) for presumed ocular tuberculosis (TB)., Methods: Retrospective analysis of medical records of patients who received ATT for presumed ocular TB and completed at least 12 months follow-up after initiation of ATT. The diagnosis of presumed ocular TB was based on presence of ocular signs suggestive of TB, evidence of past tubercular infection, and exclusion of mimicking clinical entities. All patients received a combination of ATT and corticosteroid therapy. Primary outcome measure was progression (worsening) of ocular inflammation, defined as a two-step increase in level of inflammation (anterior chamber/ vitreous) or the appearance of new lesions following initiation of ATT., Results: A total of 106 patients (64 male, 42 female) received ATT for presumed ocular TB. Twenty-six (24.5%) patients developed progressive intraocular inflammation following ATT. Primary diagnoses in these patients were: anterior uveitis (n=1), intermediate uveitis (n=9), retinal vasculitis (n=3), serpiginous-like choroiditis (n=7), multifocal choroiditis (n=2), and pan-uveitis (n=4). Following progressive inflammation, diagnosis was revised in two patients (7.7%)-both responded to alternative therapy. Of the rest, majority (n=16; 61.5%) resolved with escalation of corticosteroid therapy. Five patients (19.2%)-all having intermediate uveitis-required therapeutic vitrectomy for resolution. Three patients (11.5%) had persistent inflammation at end of follow-up period., Conclusion: Progressive inflammation following ATT for presumed ocular TB is common. It generally resolves on escalation of corticosteroid therapy. Cases not responding to increased immunosuppression need to be re-investigated to rule out a nontubercular cause.

Purpose: To report the clinical profile, etiologies, treatment modalities, and outcomes for vitreous hemorrhage in patients <18 years of age in India., Methods: The medical records of patients presenting with vitreous hemorrhage between January 2000 and January 2010 were retrospectively reviewed., Results: A total of 261 eyes of 246 patients (201 males) were included. Of these patients, 231 (94%) were unilateral. Trauma was the leading cause of injury in 179 eyes (68.5%); blunt trauma was more common than penetrating trauma in both males (64.7%) and females (57.7%). Wooden sticks were the most common cause of trauma (20.7%). Spontaneous causes accounted for 82 (31.5%) eyes, including 33 eyes (40%) with Eales disease, and 16 eyes (19.7%) with sequelae of retinopathy of prematurity. Trauma was most common cause of vitreous hemorrhage in all age groups, except in children <4 years of age, in whom spontaneous hemorrhage predominated. Overall, the most common management was surgery, performed in 173 (66.2%) eyes; 63 (24.1%) eyes were observed. Male sex, age >8 years, unilateral presentation, surgical treatment (P < 0.001), and blunt trauma (P = 0.047) were associated with severe visual loss. Mean follow-up was 636.5 ± 802.0 days overall, 597.0 ± 749.0 days for eyes observed and 638.8 ± 802.6 days for eyes that underwent surgery., Conclusions: Posttraumatic vitreous hemorrhage comprises almost two-thirds of children and adolescents presenting with vitreous hemorrhage. Blunt trauma is more common than penetrating trauma. Male sex, age >8 years, unilateral presentation, and surgical treatment were all significantly associated with severe visual loss., (Copyright © 2013 American Association for Pediatric Ophthalmology and Strabismus. Published by Mosby, Inc. All rights reserved.)

Kikuchi-Fujimoto Disease (KFD) is a benign, self-limited disease characterized by tender regional lymphadenopathy with fever. KFD remains a poorly defined disease, and no clear diagnostic criteria are available. Here, we assess the clinical, laboratory, and histopathologic findings of KFD cases and report two unusual cases. Forty KFD patients that underwent lymph node (LN) biopsy and diagnosed by immunohistochemical staining, from January 2003 to November 2010, were enrolled in this retrospective study. The patients had a mean age of 29.3 years, and 29 (72.5 %) were women. Affected LNs were mainly located unilaterally in the cervical area. Mean LN size was 15.3 mm. Twenty-eight (70 %) patients had LN tenderness, and 25 (62.5 %) patients had fever. Leukopenia was observed in 18 of 35 evaluable patients. C-reactive protein and erythrocyte sedimentation rate were elevated in most patients. Anti-nuclear antibody was positive in four of 19 evaluable patients, but all had been diagnosed with concurrent systemic lupus erythematosus. Histologically, the 40 cases were classified into three types, that is, as proliferative (37.5 %), necrotizing (55.0 %), or xanthomatous (7.5 %). Interesting cases: Case 1 was a 35-year-old female with KFD and uveitis, retinal vasculitis, and superior sagittal sinus thrombosis. Case 2 was a 47-year-old male with KFD and bone marrow involvement and presented with severe bicytopenia. Although KFD is an uncommon self-limited benign disorder, it must be included in the differential diagnosis of lymphadenopathy with fever and cytopenia. It is important that the clinical features of KFD be understood to reach a correct diagnosis.

Behçet disease (BD) is a multisystem inflammatory disorder that is an important cause of morbidity worldwide. BD is most common along the ancient "Silk Road" route in the Far East and Mediterranean basin. The eye is the most commonly involved organ in BD patients.The prototypical form of involvement is a relapsing remitting panuveitis and retinal vasculitis. Less commonly, BD may present in the form of conjunctivitis, conjunctival ulcers, keratitis, episcleritis, scleritis, and extraocular muscle paralysis. Uveitis in BD carries significant implications for the patient, because it is a chronic recurrent disease characterized by explosive attacks of severe inflammation that may cause significant, cumulative damage to the intraocular structures. This review summarizes the epidemiology of systemic and ocular clinical features of BD with particular focus on risk factors, clinical characteristics, complications, and prognosis of BD-associated uveitis.

Objectives: To study the demographic profile, clinical presentations, management and visual outcome of retinal vasculitis in a tertiary eye care center of Nepal, Materials and Methods: A retrospective, record based study of retinal vasculitis cases in the retina clinic of a tertiary care centre in Nepal from January 2009 to January 2011 was carried out. Results of the study were compared to those from the study conducted in a similar set- up between 1998 and 2000., Results: Sixty-nine eyes of 51 affected patients were evaluated in the study. The male/female ratio was 2.64:1 vs 19:1 a decade ago (p=0.0027). The mean age of the patients was 33.53 ± 12.29 years in the present study. Bilateral ocular involvement was present in 18 cases (35.3%) vs 56.14% in the past decade (p=0044).The common symptoms were dimness of vision (29.4%), floaters (25.5 %) and flashes of light (3.92%). Seventy-one eyes (69.6%) had the best corrected visual acuity of 6/18 or better. Four eyes (3.92 %) had no light perception. Vascular sheathing was the most common finding (32.35%), followed by vitritis (30.39%). Corticosteroids were primarily used to manage retinal vasculitis (39.21%). No association of retinal vasculitis with tuberculosis was found., Conclusion: The demographic pattern and clinical presentation of idiopathic retinal vasculitis has changed over a decade period in Nepal., (© NEPjOPH.)

Purpose: To evaluate the role of interleukin-6 (IL-6) in the inflammatory and proliferative stages of Eales' disease (ED) and to determine the influence of IL-6-174G/C polymorphism in the IL-6 and IL-6-regulated protein expression, as well as the development of ED., Methods: One hundred and twenty-one patients diagnosed with ED, 223 matched healthy controls, and 16 control patients with macular holes were recruited from the eastern Indian population. Serum and vitreous levels of IL-6 and vascular endothelial growth factors (VEGF) were measured by enzyme-linked immunosorbent assay. Serum levels of high-sensitivity C-reactive protein (hsCRP) were measured by enzyme immunoassay. Subjects were genotyped for the IL-6-174G/C polymorphism (rs1800795) by a custom TaqMan single-nucleotide polymorphism (SNP) Genotyping Assays system., Results: Serum IL-6 (p<0.0001), hsCRP (p<0.0001), and VEGF (p=0.0031) levels were significantly higher in the inflammatory stage of ED than in healthy controls. Serum IL-6 also significantly correlated with hsCRP (Spearman's correlation coefficient; r=0.4992, p=0.0009), but not with VEGF in this stage in ED patients. At the proliferative stage of ED, significantly higher levels of vitreous IL-6 (p=<0.0001) and VEGF (p=<0.0001) were found compared with the vitreous of patients with macular holes. A significant correlation was observed between vitreous IL-6 and VEGF in ED patients (Spearman's correlation coefficient; r=0.5834, p=0.0087). A statistically significant association was found between the -174GG genotype (p=0.006) and occurrence of ED. Mean serum and vitreous concentrations of IL-6 were also higher in the subjects with the GG genotype than in those with the GC or CC genotype in this population., Conclusions: IL-6 expression, regulated by the allelic distribution of -174 loci and the enhanced level of IL-6, modulates CRP and VEGF concentration depending respectively on the acute inflammatory stimulation at the initial stage and angiogenic stimulation at the advanced stage of ED.

Purpose: To study the epidemiological and clinical features of noninfectious retinal vasculitis (NIRV)., Methods: We analyzed 128 consecutive patients with NIRV, collected over 15 years (1993-2007) in an ophthalmological reference university hospital in Tunis, Tunisia. Data were analyzed regarding associated systemic disease, ocular syndromes, anatomic features (type and topography of vessel and type of capillaropathy), age and sex. The results of the etiologic work-up were based on the Levy-Clarke and Perez classification., Results: A total of 240 cases of NIRV (128 patients) were collected (mean age: 32; sex ratio: 2.6). It was bilateral in 93.7% of cases. The mean visual acuity (VA) was 20/50 (range: 20/800-20/20). NIRV was mainly venous (84.1%), diffuse (57%), with a mixed capillaropathy (40.2%). There were complications in 56.25% of the cases, mainly macular edema (48.1%), vascular occlusion (25.9%), optic atrophy (22.2%) and cataract (19.2%). NIRV was idiopathic in 15.6% of the cases, characterized by a predominance of young subjects (mean: 38 years old), males (sex ratio: 4), VA at 20/25, and edematous periphlebitis in 100% of cases. There were ocular disorders in 12.5% of the cases and systemic disease in 72% of the cases, with a predominance of Behçet disease (BD): 53.9% of all patients and 81% of systemic disease with predominant venous features. In 48.3% of cases, VA was less than 20/200, due to BD in 48% of the cases., Conclusion: In NIRV, the etiologic work-up is oriented on anatomic presentation, based on fluorescein retinal angiography, and requires an interdisciplinary approach. In young adults with retinal phlebitis, BD is suggested first., (Copyright © 2010 Elsevier Masson SAS. All rights reserved.)

Objectives: To analyse the clinical manifestations and severity of Behçet's disease (BD) in Korea according to geographic region of residence, and to identify risk factors associated with clinical severity., Methods: We enrolled 246 BD patients (88 males) who fulfilled the criteria of the International Study Group for BD. These patients were assigned into two groups: a Western group comprising 127 residents in west regions and an Eastern group comprising 119 residents of the SoBaek Mountains situated in the center of Korea. Clinical severity was assessed using a severity scoring index of BD., Results: BD patients from the Western group had a greater prevalence of typical skin lesions, deep vein thrombosis, gastrointestinal bleeding, posterior/ panuveitis, and retinal vasculitis than did the Eastern group (p<0.001, p=0.009, p=0.032, and p=0.007, respectively). The Western group also had higher severity scores than did the Eastern group (5.88±2.5 for the Western group vs. 4.94±2.6 for the Eastern group, p=0.004). Male BD patients had higher severity scores than did female patients (6.14±2.8 vs. 5.03±2.4, p=0.001). The disease duration of BD in the Western group showed a significant association with total severity scores (r=0231, p=0.009), but this was not observed for the Eastern group., Conclusions: We found distinct differences in the clinical manifestations as well as severity of BD according to geographic region in Korean BD patients. Males, particularly those from the Western region of Korea, had significantly higher severity scores than did females.

Purpose: To compare clinical findings in patients with ocular tuberculosis experienced during two different decades., Methods: Thirty-four patients with ocular tuberculosis were divided into two groups: a 1990s group (n = 18) and a 2000s group (n = 16), according to the dates of their first outpatient visit. The clinical profiles of the two groups were then compared., Results: More cases of the 1990s group had complications involving extraocular tuberculosis than those of the 2000s group. While various ophthalmic manifestations were observed clinically in the 1990s group, all retinal periphlebitis cases presented in the 2000s group. The proportion of patients who received antituberculous treatment was higher in the 1990s group, but the proportion who received oral corticosteroid therapy did not differ between the two periods. However, more patients underwent laser photocoagulation in the 2000s group. The percentage of eyes with final visual acuity better than 20/20 increased in the 2000s group., Conclusions: The clinical outcome of patients with ocular tuberculosis was improved in the 2000s group, which may be attributable to the increase in active use of laser photocoagulation therapy.

Background: Epidemiological studies demonstrate a prevalence of Adamantiades-Behçet's disease (ABD) in the range of 0.12-420 per 100,000 inhabitants, with the highest rates in Istanbul, Turkey, and the lowest rates in the USA. Ophthalmological data on the prevalence of ocular involvement are limited for ABD in Germany, because most epidemiological studies are based on rheumatological or dermatological data. Berlin is the city with the highest number of non-native German inhabitants, and its multi-ethnic character renders it uniquely appropriate for epidemiological studies on ABD., Methods: We retrospectively analyzed all ABD patients seen in our department since 1982. All patients fulfilled the criteria of the International Study Group for Behçet's Disease. We found 140 patients (63 female, 77 male), with a mean follow-up of 6.4 years (0.5-22 years)., Results: The mean age was 23 at the first manifestation and 32 when the full-blown disease was noted. The mean age at onset of ocular involvement was 30. Most of the patients were of Turkish (n = 73) or German (n = 34) origin. Fifty-six percent developed ocular involvement, which was the first manifestation in 8.6% and the second manifestation in 19.3% of cases., Conclusions: More than half the patients developed ocular involvement. The calculated prevalence of ocular involvement in ABD is 1.77/100,000 inhabitants for the municipality of Berlin.

Retinal vasculitis is an idiopathic inflammatory venous occlusion primarily affecting the peripheral retina of otherwise healthy young adults. Eales' disease is recognized as primary vasculitis of unknown etiology occurring in young adults. This article aims at the overall review of the etiopathogenesis, clinical presentations, pathology, management and prognosis of retinal vasculitis.

The possible occurrence of asymptomatic retinal vascular damage was investigated in 87 hyperhomocysteinemic (plasma total homocysteine >13micromol/L) adult epileptic patients (46 M, 41 F; age 34.2+/-7.5 years; mean plasma homocysteine levels 29.8+/-15.4micromol/L; duration of epilepsy 11.5+/-2.4 years) with no other risk factors for atherosclerosis. Plasma total homocysteine (t-Hcy) levels were assayed by high performance liquid chromatography. Retina vascular status was assessed by fundus oculi ophthalmoscopy performed in blind conditions by two skilled ophthalmologists and compared with that obtained from 102 randomly chosen epileptic patients and 94 healthy subjects, matched for age and sex, showing normal t-Hcy levels. No retina abnormality was detected in any of the subjects belonging to the three groups. Based on these results, we conclude that epileptic patients with mild to intermediate hyperhomocysteinemia are not at risk to develop retinal vascular disease.

Introduction: Malaria is a tropical disease causing an estimated 300 million infections and one million deaths per year. In sub-Saharan Africa, most infections are due to Plasmodium falciparum. The hallmark of the clinical syndrome of cerebral malaria is coma, and the associated mortality rate, even in appropriately treated patients, is 15 - 50 %. Funduscopy plays a major role for the ophthalmologist in the differential diagnosis because of the characteristic changes., Methods: To date more than 1000 children who satisfied the standard clinical case definition of cerebral malaria were admitted to the Blantyre Malaria Project (Malawi, Africa) for inpatient treatment and examined using indirect ophthalmoscopy through fully dilated pupils. The gender distribution was homogeneous and the children were between 2 and 14 years old. The optic nerve head, central and peripheral retina and central and peripheral vessels were described and photographed using a hand-held fundus camera (KOWA)., Results: The spectrum and severity of findings of the ocular fundus in children with CM include the following distinct entities: haemorrhages (with and without a white centre), cotton wool spots, papilloedema, retinal whitening and retinal vessel abnormalities that may appear to be orange or white. Most of the retinal haemorrhages (in 40 %) have white centres and resemble Roth spots. Cotton wool spots were seen in only 5 %. Papilloedema was also not commonly seen (8 %) but is a poor prognostic sign. Retinal whitening (in 50 %) is seen more commonly at the posterior pole than in the periphery. 20 % of patients show retinal vessel abnormalities that may be orange or white in colour., Conclusion: Ocular fundus changes in CM can be detected by the ophthalmologist using simple means and are decisive for the prognosis and timely therapy for this potentially lethal disease.

Objective: To report ocular complications of Rift Valley fever (RVF) during its first reported outbreak in southwest Saudi Arabia in autumn 2000., Design: Cross-sectional study of patients in a referral hospital., Participants: One hundred forty-three consecutive patients with confirmed RVF serologic test results and ocular lesions were enrolled in the study., Methods: Hospitalized patients (n = 30) and outpatients (n = 113) with clinical symptoms consistent with RVF, positive RVF serologic test results, and ocular abnormalities were studied. Ophthalmologic examinations, including fundus photography and fluorescein angiography, were performed. Patients were followed up at regular intervals to determine the prognosis and outcome of identified ocular abnormalities., Main Outcome Measures: Visual acuity at initial presentation and course of anterior and posterior segment complications., Results: Among 143 patients (78% males; mean age, 53.2 years), 212 eyes were affected, comprising 47 eyes in 30 inpatients and 165 eyes in 113 outpatients. The mean interval between the onset of RVF and visual symptoms ranged from 4 to 15 days (mean, 8.8 days). Macular or paramacular retinitis was identified in all the affected eyes (n = 212) at the time of initial assessment. Lesions included retinal hemorrhages (40%), vitreous reactions (26%), optic disc edema (15%), and retinal vasculitis (7%). Anterior uveitis was present in 31% of outpatients. Fluorescein angiography of the retinitis showed early hypofluorescence with late staining of retinal lesions and blood vessels. Initial visual acuity was less than 20/200 in 80% of eyes in the outpatient group; their vision improved, deteriorated, or remained the same in 13%, 15%, or 72%, respectively. Evaluation at the last follow-up showed macular (60%) or paramacular (9%) scarring, vascular occlusion (23%), and optic atrophy (20%) in the outpatient group., Conclusions: Rift Valley fever was associated with major ocular morbidity. Ocular manifestations of RVF occurred with a relatively higher frequency than reported up to now and were not limited to severe infections. Rift Valley fever affects the uvea and posterior chorioretinal area and is associated with permanent visual loss resulting from macular and paramacular scarring, vascular occlusion, and optic atrophy. The study demonstrated for the first time that transient nongranulomatous anterior uveitis is associated with RVF.

Purpose: To describe predominant retinal vasculitis in children carrying human T-cell lymphotropic virus type 1 (HTLV-1)., Methods: The authors examined clinical records of patients with HTLV-1-associated uveitis between 1987 and 2001 in Kagoshima University Hospital and reviewed cases of extensive, smoldering retinal vasculitis., Results: Three previously healthy teenagers noted mild visual symptoms and presented with extensive sheathing of retinal vessels, complicated by mild anterior segment inflammation. The retinal vascular disease responded poorly to systemic corticosteroids, had a smoldering course with persistent sheathing of retinal vessels, and eventually resulted in diffuse chorioretinal degeneration. Results of laboratory studies were unremarkable except for the presence of serum antibodies to HTLV-1. One patient developed HTLV-1-associated myelopathy 11 years after the onset of ocular disease., Conclusions: The retinal vasculitis differed from the retinal vascular changes commonly seen in HTLV-1-associated uveitis. The authors suggest a clinical disease HTLV-1-associated retinal vasculitis that affects young HTLV-1 carriers, characterized by smoldering retinal vasculitis with ultimate retinal degeneration.