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Purpose: To characterize an unusual presentation of infectious posterior uveitis using multimodal imaging, and discuss the clinical decision-making involved in diagnosis and treatment., Methods: Wide-field fundus photography, swept-source optical coherence tomography (OCT), swept-source OCT angiography, fluorescein angiography, and indocyanine green angiography., Results: This patient presented with cyclical fevers and blurry vision. Fundus examination revealed bilateral optic disc edema, macular intraretinal white spots and many scattered yellow-white chorioretinal lesions. Multimodal imaging characteristics suggested that many of these lesions represent choroidal granulomas. Extensive systemic workup was only notable for borderline elevated Bartonella henselae IgG titers (1:128), however convalescent IgG titers were elevated at 38 days (1:512) supporting the diagnosis of Bartonella chorioretinitis., Conclusion: Ocular manifestations of Bartonella henselae infection are varied and may include choroidal granulomas. Multimodal imaging characteristics may help identify etiologies of infectious uveitis. Convalescent titers are important when evaluating patients with suspected Bartonellosis, especially patients with atypical presentations.

Purpose: To report a case of acute unilateral posterior uveitis as a rare manifestation of giant cell arteritis (GCA)., Observation: A 62-year-old male presented to the clinic for evaluation of decreased vision in the right eye (OD). BCVA in OD was 20/60, and fundus examination revealed 3+ vitreous cells along with several inflammatory precipitates located in posterior vitreous and on surface of retina. Although TAB was inconclusive for GCA, the clinical diagnosis of GCA was made according to the GCA diagnostic criteria. This diagnosis was further supported by 18 FDG-PET scan. The patient was started on corticosteroids, and the symptoms improved significantly after first week of treatment. At follow-up visit one month and half later, BCVA improved to 20/40 in the right eye., Conclusion: Although GCA is rarely present with uveitis, in case of unilateral posterior uveitis in elderly patient, it should be considered in the differential diagnosis.

Purpose: To study the safety of extended monthly intravitreal infliximab injections in patients with active posterior uveitis in Behcet's disease., Methods: This is a prospective, interventional, noncomparative, open-label, pilot study of 9 monthly intravitreal infliximab injections (1 mg/0.05 mL) for 22 eyes of 16 patients with active posterior uveitis in Behcet's disease. Control of inflammation and visual outcomes were assessed, and ocular complications were monitored during the study period., Results: Successful treatment was achieved in 7 eyes (35%), and failure was encountered in 13 eyes (65%). Only seven eyes of six patients (35%) had completed the study and achieved complete resolution of inflammation with improved best-corrected visual acuity and no complications. Failure was either because of inability to control the inflammation in nine eyes (45%) or development of exacerbation of inflammation in four eyes (20%). Four eyes developed severe immunological reaction from the drug after first (n = 1), second (n = 2), and third (n = 1) injections and had to discontinue the injections. Kaplan-Meier survival analysis showed that the mean estimated time to failure was 3.3 ± 0.2 months, and all failed eyes required revision of their systemic immunotherapy to control the ocular inflammation., Conclusion: Intravitreal infliximab for active posterior uveitis in Behcet's disease was associated with a high complication rate and failure to control inflammation in most eyes. It should not be considered a substitute to systemic therapy.

Significance: Vaccine-associated uveitis has appeared in recent years because of various vaccines, whereas cases for human papillomavirus (HPV) vaccination were rarely reported. With more and more females becoming aware of its importance and choosing HPV vaccination, much more attention should be paid on the adverse effects of it., Purpose: The purpose of this study was to report a rare case of posterior uveitis after divalent HPV vaccination in an Asian female., Case Report: A 29-year-old woman presented with acute vision loss accompanied by symptoms of headache, tinnitus, and myalgia after the third injection of HPV vaccination. The best-corrected visual acuity dropped to 20/500 for both eyes, and optical coherence tomography revealed bilateral multifocal submacular fluid. A short course of oral prednisone as well as Ozurdex intravitreal injection resulted in the reversal of all signs and symptoms., Conclusions: Although this case resembled Harada disease, we diagnosed it as vaccine-induced uveitis rather than coincidental autoimmune disease based on the rapid response to a short course of systemic corticosteroids. Because vaccine-induced uveitis is rare and difficult to distinguish from coincidental autoimmune disease, our case reminds eye care providers to be aware of the possible association between vaccination and a Harada-like reaction and to ask appropriately directed questions when obtaining history from young patients with uveitis. Based on this case, we also suggest Ozurdex intravitreal injection as a potential therapeutic choice, especially for patients with contraindication or personal concern to systemic corticosteroid.

Purpose: To identify risk factors for the development of persistent anterior uveitis (PAU) following uncomplicated phacoemulsification cataract extraction in patients without histories of uveitis or autoimmune diseases., Design: Retrospective cohort study., Methods: Medical records were reviewed of patients who underwent phacoemulsification cataract extraction with intraocular lens implantation between January 1, 2014, and December 31, 2016, at the University of Colorado Hospital. Exclusion criteria included patient history of autoimmune disease and/or uveitis, cataract surgery combined with another intraocular surgery, and complicated cataract surgery. Patients with PAU were identified according to Standardization of Uveitis Nomenclature Working Group criteria. Data including sex, race/ethnicity, surgery length and cumulative dissipated energy (CDE), and postoperative visual acuity (VA) and intraocular pressure (IOP) were obtained. Main outcome measurements were risk factors for the development of PAU., Results: The charts of 3,013 eyes from 2,019 patients were reviewed. A total of 61 eyes (2.0%) from 48 patients developed PAU. African Americans were more likely than whites to develop PAU (relative risk = 11.3; P < 0.0001). Age, sex, surgery length, and CDE were not risk factors. Patients with PAU did not have worse VA than those without PAU, and African Americans with PAU did not have worse VA or IOP than the other races with PAU. Eighteen of the 61 eyes (29.5%) also developed cystoid macular edema., Conclusions: African Americans have a higher risk of developing PAU after uncomplicated phacoemulsification cataract extraction. The mechanism leading to this is unclear. Although PAU requires prolonged treatment, it does not appear to lead to worse visual outcomes., (Copyright © 2019 Elsevier Inc. All rights reserved.)

Purpose: To evaluate the possible association of UBASH3B gene rs4936742 (T > C) polymorphism with Behcet's disease (BD) and posterior uveitis in BD., Materials and Methods: One hundred and thirty-one patients with BD (51 Behcet's posterior uveitis and 80 non-ocular Behcet's patients) and 61 unrelated age-matched healthy individuals as a control group without any inflammatory disease were selected. All BD cases were under follow-up and treatment in uveitis or rheumatology clinics for at least 5 years. All research subjects, including control individuals, received a comprehensive rheumatologic evaluation. All patients and controls were genotyped for UBASH3B rs4936742 (T > C) polymorphism by PCR-RFLP technique., Results: The observed frequencies of genotypes were significantly different among patients and controls (19.7% versus 30.5% for TT, OR = 2.9, P = 0.011 and 36.1% versus 45.8% for CT, OR = 2.38, P = 0.017). Frequencies of T allele showed significantly higher values in Behcet's patients (OR = 1.9, P = 0.004). Subgroup genotypic and allelic analyses disclosed no significant difference between Behcet's posterior uveitis and control groups, neither between Behcet's posterior uveitis and non-ocular BD groups. However, genotypic and allelic analyses between non-ocular BD and control groups revealed statistically significant difference (36.3% versus 19.7% for TT, OR = 4.08, P = 0.003 and 43.8% versus 36.1% for CT, OR = 2.68, P = 0.018, 58.1% versus 37.7% for T allele, OR = 2.29, P = 0.001). Individuals carrying the TT genotype for UBASH3B were four times more likely to develop non-ocular BD than unaffected, control individuals., Conclusion: Our results showed that the UBASH3B gene rs4936742 (T > C) polymorphism is associated with an increased risk of Behcet's disease, especially non-ocular BD, in Iranian population. We could not find any susceptibility role of this genetic locus for posterior uveitis in Behcet's disease.

Purpose: To report the pattern of posterior and panuveitis at a tertiary referral center in Singapore., Methods: Subgroup retrospective analysis of 334 new posterior and panuveitis cases, from the Ocular Autoimmune Systemic Inflammatory Infectious Study (OASIS) database. Descriptive analysis was performed and visual outcome and complications were reported., Results: The etiology for posterior uveitis and panuveitis was infectious in 162 patients (48.5%), non-infectious in 144 patients (43.1%), and idiopathic in 28 patients (8.4%). More patients with bilateral disease had a non-infectious etiology (n = 82, 50.9%) (p = 0.012). The most common complication was epiretinal membrane (n = 20, 12.3%) for the infectious group and cystoid macular edema (n = 12, 8.3%) for the non-infectious group., Conclusions: The proportion of etiologies in our cohort varies from other studies. Understanding the variations and demographic associations allows the diagnosis and management of posterior and panuveitis to be further improved.

Purpose: To report two cases of primary vitreoretinal lymphoma (PVRL), which presented as intermediate and posterior uveitis., Methods: Combined clinical assessment, multimodal imaging with spectral-domain optical coherence tomography, fundus autofluorescence, fluorescein angiography, indocyanine green angiography, brain magnetic resonance imaging and vitreous and retinal biopsy. Case 1 was a 48-year-old woman who complained of visual loss in her right eye secondary to a diffuse vitreous opacification and multiple chorioretinal lesions. Case 2, a 74-year-old man, presented with low vision in his right eye due to a wide chorioretinal lesion at the posterior pole, vitreous opacification and posterior uveitis in both eyes., Results: Diffuse large B cell lymphoma was histologically diagnosed in the cerebellum in the first case and in chorioretinal tissue in the second patient. Atypical lymphoid cells were detected and allowed to make a diagnosis of primary central nervous system lymphoma in case 1 and PVRL in case 2., Conclusion: PVRL often masquerades ad intermediate or posterior uveitis. The management of the patients needed a team of pathologists, haematologists and ophthalmologists to achieve the correct diagnosis and choose the more appropriate therapy. Some peculiar characteristics on multimodal imaging, even in atypical cases of PVRL, should raise suspicious for PVRL and lead to a diagnostic vitrectomy and/or retinal biopsy.

Purpose: To assess the utility of ultra-wide field (UWF) versus conventional fundus imaging in the management of tubercular (TB) posterior uveitis., Methods: Twenty-two consecutive patients (33 eyes) diagnosed with TB posterior uveitis in a tertiary care center who underwent UWF fundus photography and fluorescein angiography (FA) between July 2014 and March 2015 were included. Complete clinical and imaging records of the patients were retrospectively reviewed. A circle simulating the central 75-degree field was drawn on UWF pseudocolor and fluorescein angiography images. Findings within the circle were compared with the information yielded by the complete image and its impact on patient management was noted., Results: The clinical manifestations of posterior tubercular uveitis included retinal vasculitis (17 eyes), multifocal serpiginoid choroiditis (13 eyes), choroidal granulomas (2 eyes) and intermediate uveitis (1 eye). UWF imaging revealed additional capillary non-perfusion areas, neovascularization, active vasculitis, and peripheral choroiditis lesions in 30/33 eyes (90.9%), which influenced treatment decision in 15 eyes (45.5%)., Conclusions: UWF imaging is useful in the detection of peripheral pathologies in tubercular posterior uveitis that may influence management decisions, such as addition of immunosuppressive therapy or scatter laser photocoagulation.

Objectives: Behçet's syndrome (BS) is a systemic vasculitis, which may involve multiple organ systems simultaneously. Clinical findings in BS often fit into well-recognized patterns, such as the association between papulo-pustular skin lesions and arthritis. We have recently observed a distinct pattern, in which a subtype of neuro-Behçet's syndrome (NBS) is often preceded by specific ophthalmic manifestations of the disease process. The purpose of this study is to evaluate the association between the parenchymal subtype of NBS and posterior uveitis (PU)., Methods: We have retrospectively reviewed the clinical records of 295 patients with BS, who met the international classification criteria for BS, diagnosed at two major rheumatology clinics from 2010 to 2014. Patient demographics, ophthalmic examinations, clinical and radiologic patterns of neurological involvement were recorded. Manifestations of BS were classified as PU, NBS, vascular involvement, and arthritis. The association between clinical findings was analysed for statistical significance., Results: Of the 295 patients, 100 had PU and 44 had NBS. 30 patients had parenchymal NBS and 14 had vascular NBS. Patients with PU were significantly more likely to have neurological involvement compared to those without PU (p<0.001; Odds Ratio: 3.924; 95% CI: 1.786-8.621). Rate of posterior uveitis was higher in patients with parenchymal NBS when compared to patients with vascular NBS, vascular BS or arthritis (63.3%, 21.4%, 22% and 4.2% respectively, p<0.001)., Conclusions: Our findings suggest a clinically and statistically significant association between posterior uveitis and parenchymal type of neurologic involvement in BS. The development of posterior uveitis in a patient with previously diagnosed BS should be recognized as a "warning sign" for predisposition to neurologic involvement. These patients should be informed about the possible signs and symptoms of neurological involvement, which can cause very rapid and irreversible damage unless recognized and treated immediately.

Background: Syphilis is often known as the "Great Imitator". The differential diagnosis of posterior uveitis is broad with ocular syphilis being particularly challenging to diagnose as it presents similarly to other ocular conditions such as acute retinal necrosis., Case Report: A 34-year-old woman with multiple sexual partners over the past few years presented with painful and progressively worsening unilateral vision loss for 2 weeks. Several months prior, she had reported non-specific symptoms of headache and diffuse skin rash. Despite treatment with oral acyclovir for 3 weeks, her vision progressively declined, and she was referred to the university ophthalmology clinic for further evaluation. On examination, there was concern for acute retinal necrosis and she was empirically treated with parenteral acyclovir while awaiting further infectious disease study results. Workup ultimately revealed ocular syphilis, and neurosyphilis was additionally confirmed with cerebrospinal fluid studies. Treatment with intravenous penicillin was promptly initiated with complete visual recovery., Conclusions: Ocular syphilis varies widely in presentation and should be considered in all patients with posterior uveitis, especially with a history of headache and skin rashes. However, given that acute retinal necrosis is a more common cause of posterior uveitis and can rapidly result in permanent vision loss, it should be empirically treated whenever it is suspected while simultaneous workup is conducted to evaluate for alternative diagnoses.

Purpose: To investigate the results of dexamethasone (DEX) implant in the treatment of eyes with refractory Behçet posterior uveitis., Methods: A total of 17 eyes of 12 patients with active Behçet posterior uveitis were enrolled in the study. A single intravitreal injection of DEX implant was applied to each eye. Best corrected visual acuity (BCVA), central macular thickness (CMT), vitreous haze score, and intraocular pressure (IOP) were determined, at baseline and control visits of months 1, 3, 6, and 12., Results: The mean BCVA was significantly increased from baseline at each control visit (all p < 0.05). The mean CMT and vitreous haze score were significantly decreased from baseline at each control visit (all p < 0.05). Three eyes showed IOP spikes requiring topical anti-glaucomatous treatment., Conclusions: A single injection of DEX implant was safe and effective, as an additional treatment to systemic immunomodulatory drugs, in the treatment of refractory Behçet posterior uveitis, for a 6-month period.

Purpose: To describe a patient with Hodgkin disease with posterior uveitis who also had a thinning of the retina and an antiretinal autoantibody in his serum., Methods: Our patient was a 58-year-old man who had been diagnosed with Hodgkin disease. He had a complete ophthalmologic examination including fluorescein angiography, electroretinography, perimetry, and spectral-domain optical coherence tomography. A search for antiretinal antibodies in the serum was made by Western blot analysis, and the retinal sites reactive to the antibodies were determined by immunohistochemistry., Results: The ocular signs were mild cellular infiltration in the anterior chamber and vitreous, and small, round chorioretinal lesions in the peripheral retina. The electroretinograms were slightly reduced. Small ring-like scotomas were detected in the Goldmann visual fields. An antiretina-specific 116-kDa antibody was detected in the serum by Western blot analysis, and the antibody reacted with the ganglion cell and inner nuclear layers of mice retinas. Although the visual acuities were maintained for over eight years, the macular thickness measured in the spectral-domain optical coherence tomography images was reduced., Conclusion: The presence of an antiretinal autoantibody, granulomatous uveitis, and retinal thinning in a patient with Hodgkin disease suggests that the patient had a granulomatous uveitis associated with Hodgkin disease or lymphoma-associated uveitis with retinal involvement.

Aim: Ocular lesions of Behcet's disease (BD) need aggressive treatment to prevent severe loss of vision or blindness. Cytotoxic drugs are the main therapeutic agents and the first line treatment. Retinal vasculitis is the most aggressive lesion of ocular manifestations and predicts a worse systemic outcome. We present here the outcome with a combination of pulse cyclophosphamide, azathioprine and prednisolone, on long-term usage, up to 10 years, on 295 patients (18 493 eye-months of follow-up)., Methods: Cyclophosphamide was used as a 1-g monthly pulse for 6 months and then every 2-3 months as necessary. Azathioprine was used at 2-3 mg/kg daily. Prednisolone was initiated at 0.5 mg/kg daily. Upon the suppression of the inflammatory reaction, prednisolone was tapered gradually. Patients fulfilled the International Criteria Behcet's Disease (ICBD) and had active posterior uveitis (PU) and/or retinal vasculitis (RV). Visual acuity (VA), PU, RV and TADAI (Total Adjusted Disease Activity Index) were calculated., Results: Overall results: mean VA improved from 3.5 to 4.3 (P < 0.0001), 44% of eyes improved (95% CI = 40-50). Mean PU improved from 2.1 to 0.8 (P < 0.0001), 73% of eyes improved (95% CI = 69-78). Mean RV improved from 3.0 to 1.4 P < 0.0001), 70% of eyes improved (95% CI = 65-74). Mean TADAI improved from 29 to 18 (P < 0.0001), 72% of patients improved (95% CI = 66-77). The details of the longitudinal studies are given in the main article., Conclusion: All parameters significantly improved. VA improvement was the least, mainly due to cataracts. This combination is the best treatment choice for retinal vasculitis before opting for biologic agents., (© 2013 Asia Pacific League of Associations for Rheumatology and Wiley Publishing Asia Pty Ltd.)

We report on a patient with chronic herpes simplex virus-2 encephalitis who was characteristic for concomitantly having chronic or recurrent posterior uveitis. A 66-year-old immunocompetent man suffering from a 6-month refractory posterior uveitis developed a 1-month history of impaired short-term memory and orientation. Brain MRI demonstrated hyperintense lesions in the right parietal lobe in diffusion and fluid attenuated inversion recovery (FLAIR) sequences. Cerebrospinal fluid (CSF) examination showed mild pleocytosis and increased protein concentration. Quantitative PCR for HSV-2 DNA was positive in CSF. Treated with acyclovir, his cognitive functions gradually improved and the posterior uveitis was cured. Clinicians must be aware that HSV-2 should be considered in the aetiological investigation of chronic encephalitis in an immunocompetent patient. HSV-2 is well known for its ability to cause unilateral chronic or recurrent posterior uveitis. Therefore, posterior uveitis should be considered as an associated feature of HSV-2 encephalitis.

Purpose: To evaluate the risk of adverse effects and to demonstrate short-term results after selective laser trabeculoplasty (SLT) in glaucoma patients., Methods: Sixty-four eyes of 64 patients with primary open-angle glaucoma, not sufficiently treated with local antiglaucomatous therapy, were included in this prospective study. Intraocular pressure (IOP), anterior chamber cells, anterior chamber flare, and vitreous haze (according to the Standardization of Uveitis Nomenclature Working Group) were examined before SLT, for 24 hours, 14 days, 6 weeks, and 3 months after laser. Furthermore, macular thickness measurements in 9 Early Treatment Diabetic Retinopathy Study subfields, including central subfield, measured by Spectralis OCT were performed. The differences between prelaser and postlaser values were obtained., Results: The average of mean preoperative IOP measurement was 19.1±3.972 mm Hg compared with 12.9±2.513 (P<0.001), 13.2±3.331 (P<0.001), 14.1±2.731 (P<0.001), and 13.9±2.922 mm Hg (P<0.001) 24 hours, 14 days, 6 weeks, and 3 months post-SLT, respectively. The central subfield preoperatively was 278.14±74.355 µm compared with 277.14±71.461 (P=0.177), 277.14±71.461 (P=0.354), 287.34±74.363 (P=0.414), and 257.45±68.431 µm (P=0.214) 24 hours, 14 days, 6 weeks, and 3 months after treatment. Anterior chamber cells, anterior chamber flare, and vitreous haze were not denoted at any time of examination., Conclusions: In this study, no significant increase in macular thickness and no other adverse effects were present. Furthermore, SLT was found to significantly lower IOP in glaucoma patients in addition to local therapy. In conclusion, SLT has a good ability to reduce IOP with a minor risk of adverse effects.

Purpose: To assess long-term efficacy and safety of infliximab for refractory Behçet disease (BD) uveitis and to evaluate the effect of withdrawal of infliximab after achieving long-term remission., Methods: Retrospective study of 19 patients., Results: Mean follow-up was 44.1 ± 36.5 months and mean number of infliximab infusions was 21.6 ± 14.6. At end of follow-up, there was significant improvement of visual acuity and reduction of central macular thickness. All patients achieved remission, 14 of whom were able to discontinue corticosteroids. Ten patients developed autoantibodies and 1 patient developed infusion reactions. Eight eyes underwent intraocular surgery without exacerbation of quiescent uveitis. After achieving complete remission, 5 patients discontinued infliximab and maintained remission during a mean of 24.6 ± 5.5 months., Conclusions: Infliximab is effective and safe for long-term treatment for refractory BD uveitis. Repeated infusions are required to maintain long-term remission, which may be sustained despite withdrawal of infliximab. Induction of autoantibodies is common.

Purpose: Investigation of subfoveal choroidal thickness in Behçet disease (BD) with enhanced depth imaging optical coherence tomography (EDI-OCT)., Methods: The study included 35 patients with posterior uveitis (PU) associated with BD, 35 patients with BD without ocular involvement, and 30 healthy controls., Results: There were no significant differences between the groups in terms of age, gender, axial length, or spherical equivalent refractive error. The mean subfoveal choroidal thickness was thinner in eyes with PU than in eyes without PU and healthy controls (p = 0.026)., Conclusion: EDI-OCT is a beneficial test for evaluating choroid morphology in BD. Thinning of the subfoveal choroidal tissue has been observed in patients with BD-associated PU.

Background: Ocular manifestations of Behcet's disease (BD) need aggressive treatment to prevent severe loss of vision or blindness. Cytotoxic drugs are the main therapeutic agents and the first line treatment. Methotrexate is the least toxic, used mainly for posterior uveitis. We present here the outcome of eye lesions with methotrexate and prednisolone, in a longitudinal study of up to 15 years, on 682 patients (5447 eye-years of follow-up)., Patients and Methods: Methotrexate was started at 7.5-15 mg/week. Prednisolone was added at 0.5 mg/kg/daily, then adjusted as needed., Inclusion Criteria: (i) fulfilling the International Criteria for Behcet's Disease; and (ii) having active posterior uveitis (PU). Visual acuity (VA) was calculated on a scale of 10. Activity indexes were calculated for PU and retinal vasculitis (RV) for each eye. Total Inflammatory Activity Index (TIAI) demonstrating the inflammatory index of both eyes of the patient, and Total Adjusted Disease Activity Index (TADAI) showing both TIAI + VA were also calculated., Results: Overall results: the mean VA improvement was 0.4 (P < 001), PU 1.2 (P < 0.001) and RV 0.6 (P < 0.001). VA improved in 46.5%, PU in 75.4%, and RV in 53.7% of eyes. TIAI improved in 74% of patients and TADAI in 69.4%. VA was aggravated in 37.2%, PU in 11.1%, and RV in 30.3% of eyes. TIAI was aggravated in 17.4% and TADAI in 21.6% of the patients. The remaining kept their baseline values., Conclusion: All parameters improved, PU better than RV. Improvement of VA was the least, mainly due to secondary cataracts., (© 2013 Asia Pacific League of Associations for Rheumatology and Wiley Publishing Asia Pty Ltd.)

Objective: Retinal vasculitis (RV) is the most aggressive lesion of ocular manifestations of Behcet's disease, seen in 32.1% of patients. Although visual acuity (VA) improves with early and aggressive treatment, in the long run it is seen in only 48% of patients. Mesenchymal stem cell (MSC) transplantation (MSCT) can theoretically reverse the RV process., Patients and Methods: Three patients with advanced RV and very low VA were selected. Eyes selected for MSCT were legally blind (no useful vision) with severe retinal damage due to vasculitis, resistant to combinations of monthly pulse-cyclophosphamide (1000 mg) + azathioprine 2-3 mg/kg/day + prednisolone 0.5 mg/kg/day. After patient signed written consent, 30 mL of bone marrow were taken and cultured for MSC growth. After having enough MSCs in culture (4-5 weeks) and taking into consideration all safety measures, cells were injected in one eye of each patient (approximately 1.8 million MSCs). VA was measured. Disease Activity Index (DAI) was calculated for anterior uveitis (AU), posterior uveitis (PU) and RV., Results: Visual acuity was light perception (LP) for two patients and finger count (FC) for the third. Follow-up at 1, 6 and 12 months were respectively LP/LP/FC at 0.5 m, no-light perception (NLP)/LP/LP, NLP/LP/NLP., Discussion: Results showed a total failure of the procedure, essentially due to the late and advanced state of vasculitis. However, the autoimmune/inflammatory reaction was greatly controlled by the procedure., Conclusion: Earlier cases have to be selected for further trials., (© 2013 The Authors International Journal of Rheumatic Diseases © 2013 Asia Pacific League of Associations for Rheumatology and Wiley Publishing Asia Pty Ltd.)

Purpose: To study the effectiveness of mycophenolate mofetil (MMF) as first-line therapy combined with systemic corticosteroids in acute uveitis associated with Vogt-Koyanagi-Harada (VKH) disease. The outcomes in this group were compared with those of another group of patients with VKH disease who were treated with corticosteroid monotherapy or with delayed addition of immunomodulatory therapy., Methods: This prospective study included 19 patients (38 eyes) diagnosed with acute uveitis associated with VKH disease., Results: The mean follow-up period was 27.0 ± 11.1 months (range 16-54 months). Corticosteroid-sparing effect was achieved in all patients. The mean interval between starting treatment and tapering prednisone to 10 mg or less daily was 5.1 ± 1.2 months (range 3-7 months). Ten (53%) patients discontinued treatment without relapse of inflammation. The mean time observed of treatment was 17.3 ± 11.9 months (range 3-41.5 months). Visual acuity of 20/20 was achieved by 38% of the eyes in the corticosteroid group and by 74% in the corticosteroid + MMF group (p < 0.001). Recurrent inflammation of ≥3 times was reduced significantly (p = 0.0383) in the corticosteroid + MMF group (3%) as compared to corticosteroid group (18%). Development of all complications was significantly higher in the corticosteroid group (43%) compared with the corticosteroid + MMF group (8%) (p < 0.001). None of the eyes in the corticosteroid + MMF group developed 'sunset glow fundus'., Conclusions: Addition of MMF as first-line therapy to corticosteroids in patients with acute uveitis associated with VKH disease leads to significant reduction in recurrences of uveitis and development of late complications and significantly improves visual outcome., (© 2012 The Authors. Acta Ophthalmologica © 2012 Acta Ophthalmologica Scandinavica Foundation.)

Purpose: To assess the safety and to conduct a preliminary assessment of efficacy of intravitreal infliximab, an anti-tumor necrosis factor antibody, for sight-threatening relapsing uveitis in Behçet disease., Design: Prospective, noncomparative, interventional pilot study., Methods: A single intravitreal injection of infliximab (1 mg/0.05 mL) was given to 15 patients with relapsing posterior uveitis at the onset of a unilateral attack. Best-corrected visual acuity, anterior chamber cells, vitreous haze, and posterior eye segment inflammation were assessed at baseline and at 1, 7, 14, and 30 days after treatment., Results: Ocular or extra-ocular side effects were not observed. Baseline best-corrected visual acuity (mean logarithm of minimal angle of resolution, 0.74; range, 0.15 to 1.7) improved significantly by day 7 and continued to improve through day 30 after infliximab (mean, 0.30; P < .0001). Profound decreases in anterior chamber cells and vitreous haze (both P < .0001), as well as beneficial effects in retinal vasculitis (P = .0001) and retinitis (P = .001) were evident through day 30. Cystoid macular edema persisted in 9 of 11 eyes affected, but central macular thickness decreased from a baseline mean of 434 to 309 mm at the end of follow-up (P < .0001). Lack of systemic treatment at baseline in 4 patients or background immunosuppressive medications, which remained unchanged during follow-up, did not influence significantly these responses; additional treatment was not required., Conclusions: These findings suggest that intraocularly produced or acting tumor necrosis factor, or both, is crucial in Behçet disease-associated relapsing uveitis and that intravitreal infliximab should be considered when systemic administration is not feasible or contraindicated. Further studies may identify patients for whom intravitreal infliximab is preferable to systemic treatment., (Copyright © 2012 Elsevier Inc. All rights reserved.)

Two clinical forms of the "white spot" syndrome in patients with posterior uveitis in definitive and presumable ocular sarcoidosis were analyzed. Group 1 was characterized by periphlebitis and discrete white spots around the vein of the retina, so-called "candle-wax", whereas group 2 showed yellow-orange solitary nodules located at the choroid, i.e. multifocal choroiditis. Visual acuity and the severity of clinical presentation were assessed in both groups. Visual acuity, Snellen equivalent was 0.52 +/- 0.36 in group 1 and 0.82 +/- 0.39 in group 2 with lesions at the level of choroid. One-way analysis of variance ANOVA showed a statistically significant between-group difference in visual acuity (p = 0.03). The mean severity of clinical presentation was 11.80 +/- 2.04 points in group 1 and 5.80 +/- 4.18 points in group 2. T-test for independent samples yielded a statistically significant difference between the groups (p = 0.02). A statistically significant difference in visual acuity was the result of vasculitis in the group with the "candle-wax" phenomenon, which is associated with retinal vasculitis and causes cystoid macular edema and reduction of visual acuity. Complications such as cataract, glaucoma and neovascularization, which also decrease visual acuity, were more frequent in group 1.

Case Report: A diabetic patient who developed a unilateral uveitis with a chorioretinitis patch in his right eye associated with decreased visual acuity and fever. Endogenous endophthalmitis was suspected and complementary tests were performed, finding hepatic abscesses with Klebsiella isolation in the biopsy. The ocular disorder slowly improved with intravenous therapy and guided percutaneous liver drainage., Conclusion: Endogenous Klebsiella endophthalmitis is an uncommon condition with severe complications. An early diagnosis and aggressive antibiotic therapy can ameliorate the final course but the visual outcome still remains poor., (2010 Sociedad Española de Oftalmología. Published by Elsevier Espana. All rights reserved.)

This prospective study was performed to find out the profile of newly diagnosed uveitis patients in a tertiary eye hospital in Eastern India over one year from August 2006 to July 2007. One hundred and sixty-one patients were included; 58 patients (36.02%) had anterior uveitis, 21 (13.04%) had intermediate uveitis, 56 (34.78%) had posterior uveitis and 26 (16.15%) had panuveitis. A specific diagnosis could be made in 50 patients (31.06%). The profile of uveitis patients in this study from Eastern India correlated with those published in studies from North and South India.

Purpose: To describe uveitis clinical characteristics in the elderly., Methods: Retrospective review of 91 patients at the age of 60 or more years at the authors' uveitis tertiary center over a 7-year period., Results: Uveitis in the elderly accounted for 30.1% of this population. Uveitis localization was anterior in 22.0% of patients, intermediate in 8.8%, posterior in 20.9%, while 41.7% patients presented with panuveitis. Sarcoidosis (37.4%) and idiopathic uveitis (36.3%) accounted for the majority of cases, whereas other diagnostic entities accounted for 26.3%. Panuveitis (41.7%) and sarcoidosis (37.4%) were detected at a significantly higher frequency than in the younger population. Contrarily, ankylosing spondylitis and established ophthalmological entities (pars planitis, Birdshot chorioretinopathy, Fuchs heterochromic cyclitis) were more common in patients younger than 60 years old., Conclusion: In the authors' experience, sarcoidosis is the leading cause of uveitis in the elderly. Idiopathic uveitis and other specific entities account for less than two-thirds of cases.

Objectives: To investigate the long-term effect of infliximab on ocular and extraocular manifestations in patients with Behçet's disease., Methods: Seven patients with active Behçet's disease and treated with infliximab at Aichi Medical University Hospital for more than 18 months were included in the study. We evaluated visual acuity, the average number of uveitis attacks involving the posterior segment, and general disease activity every 2 months. The Behçet's Disease Current Activity Form (BDCAF) was used for an overall index of disease activity. Anti-infliximab antibody levels were examined in the patients' sera., Results: The follow-up period after initial introduction of infliximab ranged from 19 to 40 months (mean ± SD, 32 ± 8.7 months). The number of infliximab infusions ranged from 12 to 24 (19 ± 4.4). By the 2-month follow-up, the frequency of uveitis attacks involving the posterior segment and the BDCAF scores were significantly improved compared to the 2 months before introducing infliximab. Anti-infliximab antibodies were detected in the sera of all examined patients., Conclusions: Significant long-term improvement in both the frequency of uveitis attacks involving the posterior segment and overall disease activity was provided by the administration of infliximab to patients suffering from Behçet's disease, despite the presence of anti-infliximab antibodies.

It is of general belief that males are prone to more frequent, more severe manifestations, and less favorable outcome. We evaluated this hypothesis in ophthalmological manifestations (OM) of Behcet's Disease (BD). Visual acuity (VA), anterior uveitis, posterior uveitis (PU), and retinal vasculitis (RV) were checked, according to Ben Ezra, in 1,515 patients with eye lesions. The data at baseline and last visit were compared. Male/female ratio was 1.2 in the BD registry (6,500 patients) and 1.51 for OM patients (Chi(2) = 98.962, p < 0.0001). The patients-year-follow-up was 4,987. All parameters improved significantly from the baseline. Mean VA improved from 4.87 to 5.35 for males (p < 0.0001) and from 5.20 to 5.74 for females (p < 0.0001). Difference between males/females at baseline was not significant (p = 0.60). The mean improvement for males/females was statistically non-significant (p = 0.58). Percent improvement of eyes for males/females was 47.1%/48.8% (p = 0.41). Mean PU improved from 1.83 to 0.71 for males (p < 0.0001) and from 1.66 to 0.49 for females (p < 0.0001). Difference between males/females at baseline was significant (p = 0.01). The mean improvement for males/females was statistically non-significant (p = 0.45). Percent improvement of eyes for males/females was 75.4%/81.0% (p = 0.004). Mean RV improved from 2.05 to 1.16 for males (p < 0.0001) and from 1.97 to 0.99 for females (p < 0.0001). Difference between males/females at baseline was not significant (p = 0.42). The mean improvement for males/females was statistically non-significant (p = 0.47). Percent improvement of eyes for males/females was 62%/64.4% (p = 0.33). Male gender is more prone to ocular manifestations. The severity of lesions at baseline was the same for VA and RV. For PU, the difference was statistically significant, but was not clinically relevant. The therapeutic outcome (mean improvement and percent of improved eyes) was the same for all parameters.

Purpose: To investigate the incidence and clinical characteristics of Behçet disease in children., Methods: The authors retrospectively reviewed the charts of 3382 patients with Behçet disease from October 1986 to December 2005 at Ankara University Medical School Behçet Unit and/or Atmaca private clinic., Results: 110 children were diagnosed with Behçet disease. The 110 children represented 3.3% of the total number of Behçet patients that were reviewed. 62.7% were girls and 37.3% were boys. The mean age at initial manifestation was 11.63 ± 3.46 years and at diagnosis was 14.15 ± 2.13 years. Ocular involvement was found in 30.9% of the children. Of these, 61.8% were bilateral and 38.2% were unilateral. Anterior uveitis was found in 18 eyes (32.8%), posterior uveitis in 24 eyes (43.6%), and panuveitis in 13 eyes (23.6%)., Conclusion: Behçet disease should be considered in the differential diagnosis of childhood uveitis, particularly in endemic areas. The collaboration of pediatricians, rheumatologists, dermatologists, ophthalmologists, and other specialists when necessary is required in the diagnosis and management of children with Behçet disease.

Purpose: To analyze clinical patterns, causes, and systemic disease associations among patients with uveitis admitted to King Abdulaziz University Hospital., Methods: The authors retrospectively reviewed the medical records of 351 patients (600 eyes)., Results: The study subjects consisted of 183 (52.1%) males and 168 (47.9%) females, with a mean age of 39.9 ± 14.2 years at presentation. The most common anatomic diagnosis was panuveitis (68.6%), followed by posterior uveitis (12.7%), anterior uveitis (12.7%), and intermediate uveitis (6.0%). The most common identifiable specific diagnoses were presumed tuberculous uveitis (PTU) (28.2%), Vogt-Koyanagi-Harada (VKH) disease (19.4%), Behçet disease (BD) (12.5%), and toxoplasmosis (8.2%). After a mean follow-up period of 29.5 ± 22.1 months, 63.5% of the eyes achieved visual acuity of 20/40 or better. Eyes from patients with PTU had the worst final visual outcome., Conclusions: The most common anatomic diagnosis was panuveitis. PTU, VKH disease, BD, and toxoplasmosis were the most frequent specific diagnoses.

Background: Ocular lesions, the main morbidity of Behcet's disease (BD), are the most difficult to treat. The aim of this study was to evaluate the efficacy of rituximab., Methods: Inclusion criteria were retinal vasculitis and edema, resistant to cytotoxic drugs. Twenty patients were randomized to a rituximab group (RG) or cytotoxic combination therapy group (CCTG). Rituximab was given in two 1000-mg courses (15-day interval). Subjects received methotrexate (15 mg/weekly) with prednisolone (0.5 mg/kg per day). The CCTG received pulse cyclophosphamide (1000 mg/monthly), azathioprine (2-3 mg/kg per day) and prednisolone (0.5 mg/kg per day). The primary endpoint was the overall state of patients' eyes and the Total Adjusted Disease Activity Index (TADAI). Secondary endpoints were: visual acuity (VA), posterior uveitis (PU), and retinal vasculitis (RV). The baseline data were compared at 6 months by paired sample t-test and analysis of variance., Results: TADAI improved significantly in the RG (t = 3.340, P = 0.009), but not in the CCTG (t = 2.241, P = 0.052). For secondary endpoints (RG/CCTG), the mean VA improved in two patients versus three (2/3), remained unchanged in 1/1, and worsened in 7/6 patients. The mean PU improved significantly in the RG (t = 3.943, P = 0.001), not in the CCTG (t = 2.371, P = 0.028). RV improved, but not statistically (t = 2.027, P = 0.057 vs. t = 1.045, P = 0.31). Edema of retina, disc and macula improved significantly in both, but much better for the RG (t = 2.781, P = 0.012 vs. t = 2.707, P = 0.014)., Conclusion: Rituximab was efficient in severe ocular manifestations of BD, TADAI improved significantly after 6 months with rituximab, but not with CCT.

Posterior uveitic entities are varied entities that are infective or non-infective in etiology. They can affect the adjacent structures such as the retina, vitreous, optic nerve head and retinal blood vessels. Thorough clinical evaluation gives a clue to the diagnosis while ancillary investigations and laboratory tests assist in confirming the diagnosis. Newer evolving techniques in the investigations and management have increased the diagnostic yield. In case of diagnostic dilemma, intraocular fluid evaluation for polymerase chain testing for the genome and antibody testing against the causative agent provide greater diagnostic ability.

Aim: To assess basic thrombophilic parameters in both active and inactive periods of the ocular Behçet disease (BD) with posterior segment involvement in comparison with those in healthy adults as controls., Method: Thirty patients within the active period of the ocular BD with posterior segment involvement, 30 patients within the inactive period of the ocular BD with posterior segment involvement and 30 age-and sex-matched healthy controls were included in the study. Plasma protein C, free protein S, antithrombin III and activated protein C resistance were measured in the studied groups., Results: Activated protein C resistance and antithrombin III levels were significantly altered in both active and inactive ocular BD patients when compared with those in the controls (p<0.05), whereas protein C and free protein S levels were not significantly changed within both groups (p>0.05). There was no difference among the active and inactive groups of BD for all of the studied blood tests (p>0.05)., Conclusion: Activated protein C resistance together with increased antithrombin III plasma levels are associated with the posterior segment involvement in ocular BD based on the assessment of basic thrombophilic tests.

Purpose: To investigate a clinical survey of uveitis in southern Kyushu of Japan, where human T-lymphotropic virus type 1 (HTLV-1) and toxoplasmosis is highly endemic., Methods: The clinical records of patients with uveitis between 1975 and 2007 at Miyata Eye Hospital were reviewed., Results: A total number of 1338 patients (2012 eyes), consisting of 526 men and 812 women with mean age of 50.5 years old, were analyzed. The most common clinical entity was HTLV-1 uveitis (17.1%), followed by Vogt-Koyanagi-Harada disease (9.9%), sarcoidosis (7.2%), toxoplasmosis (7.1%), Behçet's disease (4.3%) and others. Unclassified uveitis comprised 41.1% in the series. Anterior uveitis was seen in 30.8%, intermediate uveitis in 17.3%, posterior uveitis in 9.3%, and pan-uveitis in 42.6%., Conclusions: HTLV-1 uveitis and toxoplasmosis were the major clinical entities in southern Kyushu of Japan. This relates to the high seroprevalence of the infectious agents in this region of Japan.

Involvement of the orbit and posterior segment of the eye in early-onset sarcoidosis may necessitate prompt and aggressive immunosuppressive treatment to prevent a poor visual outcome., (Copyright 2009, SLACK Incorporated.)

Diverse manifestations of ocular syphilis may involve any structure in the eye, at any stage of the disease. Posterior uveitis in the form of posterior placoid chorioretinitis has been described in secondary- and tertiary-acquired syphilis. In this case report, we present a 47-year-old man with late latent syphilitic infection and fundoscopic, as well as angiographic findings consistent with acute syphilitic posterior placoid chorioretinitis. To our knowledge this form of patchy multifocal choroiditis has never been described in the latent stage of the disease.

Purpose: To estimate the risk of structural ocular complications and loss of visual acuity (VA) in cases of Behçet disease (BD) and to evaluate potential risk and protective factors for these events., Design: Retrospective cohort study., Methods: A total of 168 consecutive patients with BD-associated ocular inflammation treated at five academic center ocular inflammation subspecialty practices were included. Clinical data for these patients were ascertained by standardized chart review. Main outcome measures included VA, structural ocular complications of inflammation, and intraocular pressure (IOP)., Results: Over a median follow-up of 1.05 years, the incidence of specific structural complications and IOP disturbances were common: the incidence rate of any ocular complication was 0.45 per eye-year (EY). Rates of loss of VA to 20/50 or worse and to 20/200 or worse were 0.12 per EY and 0.09 per EY, respectively. Risk factors for loss of VA during follow-up were persistent inflammatory activity, presence of posterior synechiae, presence of hypotony, and presence of elevated IOP. In a time-dependent analysis, current activity of ocular inflammation was associated with an increased risk of loss of VA to 20/50 or worse (relative risk [RR], 2.45; 95% confidence interval [CI], 1.1 to 5.5; P = .03) and to 20/200 or worse (RR, 2.67; 95% CI, 1.2 to 5.8; P = .01)., Conclusions: Loss of VA and occurrence of ocular complications were common in patients with ocular inflammation associated with BD, even with aggressive therapy. Ongoing inflammation during follow-up, presence or occurrence of posterior synechiae, hypotony, and elevated IOP were associated with an increased risk of loss of VA.

Aim: To describe the clinical profile, management, and outcome in seven patients with posterior segment recurrence in Vogt-Koyanagi-Harada (VKH) disease., Material and Methods: Retrospective chart review of all the patients of VKH disease presenting to our institute between 2001 and 2005 was carried out. Patients with a documented posterior segment recurrence during follow-up were included in this study. Their history, systemic associations, ocular findings, investigations, management strategies, and treatment response were noted., Results: Out of 82 VKH patients screened, documented posterior segment recurrence was seen in seven patients who included five women and two men, with a mean age of 33.7 years (range 22-55 years). At onset, two patients had incomplete and five had probable VKH disease. At the time of recurrence, four patients were on maintenance dose (5-10 mg/day) of oral corticosteroid while the remaining three were off treatment. The mean interval between the primary episode and posterior segment recurrence was 33.7 weeks (range 16-52 weeks). All posterior segment recurrences were bilateral and were characterized by vitritis (eight eyes), papillitis (14 eyes), multiple yellow-white oval subretinal lesions (six eyes), and exudative retinal detachment (10 eyes). The recurrences were managed with oral corticosteroids (1.0-1.5 mg/day) and Azathioprine (2.0-2.5 mg/day). Subsequently, three patients experienced a second episode of posterior segment recurrence, which also responded to the corticosteroid-Azathioprine combination., Conclusions: Recurrences in VKH disease may involve predominantly the posterior segment, and respond well to the standard treatment.

Intraocular inflammation, or uveitis, incorporates a diverse group of infectious and immune-mediated disorders. In addition, some conditions masquerade as uveitis. However, classifying uveitis according to anatomic location in adult and pediatric populations, and appreciating the effect of immune status and regional differences, refines the list of potential causes. In this way, a select few investigations can be performed, rather than a nondirected battery of tests.

Purpose of Review: Diagnostic vitrectomy is performed in cases of posterior segment inflammation that are suspicious for malignancy or infection and require tissue confirmation. Advances in vitrectomy technique and laboratory tests have improved the diagnostic yield in these challenging cases. The methods for obtaining vitreous specimens and the diagnostic tests used to analyze them are discussed., Recent Findings: Ancillary tests have been invaluable in addressing the diagnostic limitations of traditional histopathologic and microbiologic analyses. Flow cytometry, gene rearrangement studies, and cytokine measurements are useful adjuncts to cytology for the diagnosis of malignancy, in particular primary intraocular lymphoma. Microbial DNA amplification by polymerase chain reaction and intraocular antibody measurement has been shown to detect the presence of infection by organisms that are difficult to culture., Summary: When performed appropriately, diagnostic vitrectomy with carefully selected ancillary testing can lead to a definitive diagnosis in a large proportion of cases.

Purpose: To determine the aetiology and clinical characteristics of patients with uveitis in a tertiary ophthalmology centre in northern Thailand., Methods: Standard ophthalmological examination and laboratory screening blood tests were performed in 200 consecutive new patients with uveitis. Patients were classified according to the location and aetiology of the uveitis. Specific clinical characteristics were recorded., Design: Prospective case series., Results: The case series included 106 male and 94 female patients with a mean age of 38 years. HIV-associated uveitis was noted in 31% (62/200), and included mostly patients with cytomegalovirus retinitis (85%, 53/62). In the non-HIV group, the most common anatomical type was anterior uveitis (34%, 47/138). Infectious uveitis was diagnosed in 22% (30/138) of non-HIV patients, and toxoplasmosis was the most common infection (12/138, 8.7%). The most common non-infectious clinical entities were Vogt-Koyanagi-Harada disease (20%, 22/108) and HLA-B27-associated acute anterior uveitis (9%, 10/108)., Conclusions: The spectrum of uveitis in northern Thailand included 27% of HIV-infected patients with cytomegalovirus retinitis. Causes of non-HIV uveitis were similar to those often observed in the Far East, but the specific prevalences of these disorders were distinct from that found in India and Japan.

Objective: To describe the cytopathological method used in the analysis of vitreous samples in the diagnosis of primary intraocular lymphoma (PIOL)., Participants: Seven patients with refractory posterior uveitis referred to a regional ocular inflammatory service were diagnosed as having PIOL between 1999 and 2006., Methods: Clinical features of the uveitis and cytopathological preparation of the samples were described. All patients underwent vitrectomy and samples were placed in formal saline or prepared fresh. Following paraffin embedding generating a cell block, immunostaining, and polymerase chain reactions were performed., Results: Five women (71.4%) and two men (28.6%) (mean age 67.7 years) were included. Five patients had diagnostic vitrectomy performed within 6 months of presentation, but in two patients diagnosis was delayed up to 2 years. Uveitis was bilateral in two patients. Cytologic and immunohistochemical staining prepared from the vitreous specimens showed PIOL in all patients, and PCR displayed single band of immunoglobulin heavy chain rearrangement in five out of six samples tested., Conclusions: Diagnosis of PIOL is difficult due to small volume of sample with low number of malignant cells and inadequate preparation of samples. Our method of analysis with fresh samples together with immunohistochemistry and PCR analysis demonstrates a high yield of diagnosis reducing diagnostic delay.

We evaluated the efficacy and safety of high-dose intravenous methylprednisolone (1 g x 3 days) pulse therapy followed in the management of acute posterior segment uveitis attacks in 25 patients with Behçet's disease. High-dose intravenous methylprednisolone pulse therapy may be beneficial in the prompt control of severe ocular inflammation and in preventing visual loss.