Anterior and posterior uveitis associated with juvenile idiopathic arthritis -case report.

Anterior uveitis is the most common extra-articular manifestation in children diagnosed with Juvenile idiopathic arthritis (JIA). It is typically a non-granulomatous, chronic, and asymptomatic uveitis. The lack of acute symptoms often delays the diagnosis with the incidence of severe ocular complications. Chorioretinitis lesions have been described in only 1% of cases. The absence of fundus changes can be explained by the impossibility of performing fundoscopy through the cloudy ocular media, secondary to inflammation. A 7-year-old female with a 3-month history of painless reduced vision came to have an eye examination. An initial diagnosis of bilateral anterior granulomatous uveitis complicated with glaucoma and cataract was formulated. Because of the concomitant diagnosis of COVID-19 disease (same day as the eye examination), the child was hospitalized in a hometown COVID-19 patient ward, so both local and general treatment, monitorization, and investigations were discontinued. The following eye examination revealed the persistence of anterior uveitis, inflammatory glaucoma, cataract, and the appearance of band keratopathy. Fundoscopy revealed numerous disseminated lesions of choroiditis. Further examinations established JIA-associated uveitis diagnosis, so systemic corticosteroids were initiated followed by Methotrexate and Adalimumab. Monitoring with fundoscopy in a patient diagnosed with JIA-U is necessary to detect possible chorioretinal or vascular damage. Abbreviations: BVA = best visual acuity, CVA = corrected visual acuity, CS = corticosteroids, IOP = Intraocular pressure, JIA = Juvenile idiopathic arthritis, JIA-U = Juvenile idiopathic arthritis associated uveitis, LE = left eye, MTX = Methotrexate, OU = both eyes, OCT = Optical Coherence Tomography, RE = right eye, TNF = tumor necrosis factor.

Clinical Features and Multimodal Imaging in Atypical Posterior Uveitis Secondary to Bartonella Henselae Infection.

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.

Posterior Uveitis Associated with Large Vessel Giant Cell Arteritis.

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 18FDG-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.

MONTHLY INTRAVITREAL INFLIXIMAB IN BEHÇET'S DISEASE ACTIVE POSTERIOR UVEITIS: A Long-Term Safety Study.

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.

Pseudoretinitis pigmentosa due to syphilis: a case report and literature review / Pseudorretinose pigmentária por sífilis: relato de caso e revisão de literatura

ABSTRACT Syphilis is a sexually transmitted infection caused by the spirochete Treponema pallidum. Ocular involvement can occur at any time, and it may affect 10% of patients in the secondary stage, and from 2% to 5% in the tertiary stage. Uveitis is the most common presentation of ocular syphilis, affecting 0.4% to 8% of patients with systemic disease. Chorioretinitis is the most common posterior alteration. We present the case of a 53-year-old male patient, presenting with bilateral low visual acuity and nyctalopia for 3 years. His physical examination revealed decreased pupillary reflex, anterior vitreous cells, physiologic papillae, arteriolar attenuation, reduced foveal reflex, diffuse retinal pigment epithelium atrophy, peripapillary and perivascular punctate pigment accumulation and peripheral chorioretinitis. Full-field electroretinogram was extinct in both eyes. Treponemal syphilis test was positive. Previously diagnosed as retinitis pigmentosa, evolved to blindness, despite proper treatment. Our case shows syphilis as a significant cause of blindness. Atypical presentations of retinitis pigmentosa must warn ophthalmologists to etiologies of pseudoretinitis pigmentosa, such as syphilis.

RESUMO A sífilis é uma infecção sexualmente transmissível causada pela espiroqueta Treponema pallidum. A sífilis ocular pode ocorrer em qualquer estágio da doença, chegando a 10% na forma secundária e a 2% a 5% em sua forma terciária. A uveíte é a manifestação ocular mais comum, ocorrendo em 0,4% a 8% dos pacientes com a doença sistêmica. A coriorretinite é a manifestação mais comum do segmento posterior. Apresentamos o caso de um paciente do sexo masculino, 53 anos, com queixa de baixa acuidade visual e nictalopia há ٣ anos. Seu exame físico revelou lentificação dos reflexos pupilares, celularidade no vítreo anterior, papilas fisiológicas, atenuação arteriolar, redução do reflexo foveal, atrofia difusa do epitélio pigmentar da retina, acúmulo punctato de pigmento em regiões peripapilar e perivascular e coriorretinite periférica. Eletrorretinograma de campo total extinto em ambos os olhos. O teste treponêmico foi positivo. Foi previamente diagnosticado como portador de retinose pigmentar, evoluindo com cegueira, a despeito do tratamento correto instituído. Esse caso mostra a sífilis como importante causadora de cegueira. Casos atípicos de retinose pigmentar devem alertar o oftalmologista para causas de pseudorretinose pigmentar, como a sífilis.

Case Report: Posterior Uveitis after Divalent Human Papillomavirus Vaccination in an Asian Female.

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.

Risk Factors Associated with Persistent Anterior Uveitis after Cataract Surgery.

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.

Ocular Autoimmune Systemic Inflammatory Infectious Study - Report 3: Posterior and Panuveitis.

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.

Two cases of primary vitreoretinal lymphoma: a diagnostic challenge : The supporting role of multimodal imaging in the diagnosis of primary vitreoretinal lymphoma.

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.

Role of Ultra-Wide Field Imaging in the Management of Tubercular Posterior Uveitis.

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.

Retinal involvement in uveitis associated with Hodgkin disease.

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.

Mesenchymal stem cell therapy unable to rescue the vision from advanced Behcet's disease retinal vasculitis: report of three patients.

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.

Long-term clinical outcomes in patients with refractory uveitis associated with Behçet disease treated with infliximab.

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.

Clinical aspects of posterior uveitis in ocular sarcoidosis.

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.

Intravitreal infliximab for sight-threatening relapsing uveitis in Behçet disease: a pilot study in 15 patients.

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.

[Endogenous endophthalmitis as a first clinical manifestation of Klebsiella sepsis. The importance of an early diagnosis]. / Primera manifestación oftalmológica de una sepsis por Klebsiella. Importancia de la sospecha diagnóstica precoz.

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.

Toxoplasmosis ocular / Ocular toxoplasmosis

La toxoplasmosis ocular es una enfermedad producida por el parásito toxoplasma gondii. Es la causa más frecuente de uveítis posterior, es una enfermedad de distribución universal, al menos 500 millones de personas están infectadas en todo el mundo, ocasionando disminución de la visión y ceguera en muchas de ellas. Por tal motivo, realizamos una revisión actualizada sobre, la situación actual a nivel mundial, la historia de la enfermedad, la prevención, formas clínicas y el control de la toxoplasmosis. Se tratan otros aspectos de interés como el modo de transmisión, los hospederos (definitivos e intermediarios) y las manifestaciones clínicas más notables

The ocular toxoplasmosis is an illness caused by Toxoplasma gondii parasite. It is the most frequent cause in posterior uveitis, and it spreads worldwide since at least 500 million people are infected in the entire world, causing decrease of vision and blindness in many of them. This is the reason why we made a literature review, the current situation worldwide, the history, the prevention, the clinical forms and the control of toxoplasmosis. Other interesting aspects were the channel of transmission, the hosts (intermediary and final) and the most remarkable clinical manifestations