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For the past 50 years, significant progress has been made in understanding Seasonal Hyperacute Panuveitis (SHAPU), a mysterious blinding disease first reported in Nepal in 1975. Predominantly affecting Nepalese children, SHAPU occurs cyclically every odd year from September to December. While initially misclassified as endophthalmitis, SHAPU is set apart by its lack of trauma or surgery, failure to grow organisms in most intraocular fluid cultures, and its hallmark presentation of a "white pupil in red eye" with an association with moth exposure. Recent findings have expanded SHAPU's geographical scope beyond Nepal, with cases reported in Bhutan. Moreover, sporadic summer outbreaks have emerged, showing worse clinical outcomes than the typical cases. Recent studies suggest that the Gazalina moth may play a role, although the exact cause of SHAPU remains undetermined. Despite the increased understanding of SHAPU's geographic distribution and cyclical patterns, it is possible that some cases remain misdiagnosed or underreported. Continued research is critical to further exploring the aetiology of SHAPU, which will lead to better diagnostic tools, targeted treatments, and improved outcomes. The inclusion of SHAPU under more general categories such as endophthalmitis underscores the need for a clearer distinction of this condition to prevent blindness in at-risk populations.

Purpose: To report a case of biopsy-proven sarcoidosis in a patient with panuveitis and a positive interferon-gamma release assay (IGRA) from a non-endemic tuberculosis (TB) country., Methods: Case report., Results: A 26-year-old male from the United Arab Emirates (UAE) presented with granulomatous panuveitis characterized by mutton-fat keratic precipitates, anterior chamber and vitreous cells, and retinal vasculitis. Laboratory evaluation revealed positive IGRA, normal serum angiotensin-converting enzyme level, and chest imaging showing lung nodularity along with hilar lymphadenopathy. Endobronchial ultrasound-transbronchial needle aspiration (EBUS-TBNA) identified non-caseating granulomas and an elevated CD4+/CD8+ ratio. Both vitreous biopsy and EBUS-TBNA tested negative for TB polymerase chain reaction, leading to a definitive diagnosis of sarcoidosis. The patient was successfully managed with systemic immunosuppression and achieved sustained quiescence., Discussion: Patients from non-endemic TB countries can present with a positive IGRA in the context of sarcoidosis. Invasive biopsy evaluation is crucial for establishing an accurate diagnosis and initiating appropriate treatment.

Purpose: We report three cases of occlusive vasculitis following intravitreal rituximab therapy for biopsy-proven primary vitreoretinal lymphoma (PVRL), one of which was following an injection of the biosimilar Riabni (rituximab-arrx, AmGen) and two of which were following an injection of Rituxan (rituximab, Genentech)., Methods: Case series., Results: Three cases of occlusive vasculitis confirmed with fluorescein angiography are reported 5 days, 8 days, and 3.5 weeks following intravitreal injection of rituximab. The initial vision was poor (20/500, 20/150, and light perception), but vision recovered to baseline in two cases, and remained poor in the case of combined artery and vein occlusion., Conclusion: Occlusive vasculitis is a rarely reported but potential complication of intravitreal rituximab therapy in patients who have been previously treated with the agent and may have delayed onset. A low threshold for fluorescein angiography as a diagnostic test for post-injection vision loss and prompt treatment with topical and/or oral steroids should be considered.

Purpose: To provide a comprehensive overview of the diagnostic and therapeutic journey of a pediatric patient with persistent sarcoid-associated panuveitis over a 10-year period, who ultimately developed bilateral macular subretinal fibrosis and visual loss., Methods: Retrospective case report., Results: The patient was diagnosed with sarcoidosis after undergoing a transbronchial biopsy. She was followed up because of granulomatous panuveitis, multifocal choroiditis, and papillitis bilaterally. She maintained a stable condition, and visual acuity was 0.3 RE and 0.5 LE. Immunomodulatory therapy included prednisone, methotrexate, and adalimumab. The patient was lost to follow-up for 20 months because of the COVID-19 pandemic. She was represented with active uveitis and was not responding to TNF-ɑ inhibitors (adalimumab and infliximab). Ultimately, the patient's intraocular inflammation was successfully controlled by using intravitreal steroids (Triamcinolone and Fluocinolone acetonide implant). However, the visual outcome was guarded because of bilateral subretinal fibrosis., Conclusion: 10% of patients with sarcoidosis-associated uveitis risk blindness in one eye. The index case progressed to sight-robbing bilateral subretinal fibrosis, a rare complication of ocular sarcoidosis despite a combination of conventional and biologic anti-inflammatory therapies. There is a pressing need to develop new treatment agents for refractory non-infectious uveitis.

Sympathetic ophthalmia (SO) is a rare, bilateral, granulomatous, panuveitis following perforating trauma or surgical intervention in one eye. Here, and to the best of knowledge, we report the first case of SO following an evisceration surgery in Pakistan. A 32-year-old, Pakistani, female presented with pain and decreased visual acuity in her right eye, at Civil Hospital, Karachi, 1 week after an evisceration surgery was performed on her left eye, following corneal perforation due to unresolved keratitis. Diagnosis of SO was made on the basis of clinical presentation and confirmed by slit lamp examination which revealed mutton fat keratic precipitates, posterior synechiae, and optic disc swelling. She was administered high-dose corticosteroids to which she did not respond. She was then prescribed methotrexate which resolved the inflammation and improved visual acuity. Initiating prompt treatment for the sympathizing eye led to effective management of the patient and prevented complete blindness., Competing Interests: The authors declare no conflicts of interest., (© 2024 The Author(s). Clinical Case Reports published by John Wiley & Sons Ltd.)

Background: Chronic myeloid leukemia (CML) can manifest ocular complications stemming from hematologic irregularities or direct infiltration of neoplastic cells. This article details the case of a patient with newly diagnosed CML exhibiting elevated platelet counts (PLT) who developed panuveitis accompanied by retinal vascular occlusion., Case Presentation: A 52-year-old woman experienced a notable decline in vision in her left eye over a 2-week period. Classical anterior uveitis, vitreous cavity opacity, optic nerve edema, and retinal vascular obstruction were observed. The right eye exhibited papilledema and retinal vein tortuosity. Despite admission, the condition of both eyes deteriorated, accompanied by a continuous increase in PLT. She was diagnosed with CML based on bone marrow biopsy and chromosomal examination. Following platelet apheresis therapy and chemotherapy, the condition of her right eye significantly improved, but the left eye's condition remained irreversible., Conclusions: This is a rare case of newly diagnosed CML presenting with diverse ocular manifestations in both eyes. The disparate outcomes in eyes with varying lesion stages underscore the importance of prompt diagnosis., (© 2024. The Author(s).)

Purpose: To present a treatment regimen for Brucellosis endophthalmitis that resulted in a good visual outcome. Additionally, we conducted a literature review on the treatment and visual prognosis of related cases., Case Presentation: A 49-year-old woman with the chief complaint of decreased vision and redness in the right eye was initially diagnosed with noninfectious uveitis and prescribed high-dose steroids which led to transient improvement followed by a decline in vision. An infectious cause was suspected. Metagenomic next-generation sequencing of vitreous fluid and serological testing confirmed Brucella melitensis infection. The patient underwent vitrectomy combined with six intravitreal injections of ceftazidime in the right eye in addition to systemic antibiotic treatment. The intraocular inflammation was completely resolved, and the visual acuity recovered to 20/25, which is the best-documented recovery in Brucella endophthalmitis cases, as revealed by the literature review., Conclusion: Vitrectomy combined with repeated intravitreal injections of ceftazidime can enhance the treatment for brucellosis endophthalmitis and achieve a better visual prognosis.

Purpose: To document the long-term visual outcomes in patients with Blau syndrome., Methods: A retrospective institutional cohort study was conducted, and 13 patients with genetically confirmed Blau syndrome were included. Demographic and clinical data were collected from standardised medical charts. Baseline was defined as the first detected uveitis and data were recorded onwards at intervals of 1, 3, 5, 10, 15 and 20 years., Results: Anterior uveitis was the most common classification at baseline (57.1%). Among patients with documented uveitis lasting 10 years or more, all of them developed panuveitis. Median logMAR visual acuity at baseline was 0 (range -0.5; 0.7), 0.19 (range 0; 1.5) at year 5, and 0.7 (range 0.1 - no perception of light) at year 20, as recorded in 13, 16, and 10 eyes, respectively. All patients received treatment with topical and oral steroids, and multiple systemic immunosuppressants including biologics. Disease control, defined as having cells <1+ in both eyes and using topical steroid eye drops less than twice daily, was achieved in 14.3% to 37.5% of patients at the different time points. Cataract surgery was performed in 12 eyes of 8 patients, 3 eyes of 3 patients necessitated glaucoma surgery, and 4 eyes of 4 patients required surgery for retinal detachment., Conclusion: Uveitis associated with Blau syndrome commonly leads to severe, chronic panuveitis, requiring long-term systemic immunosuppression. Early diagnosis and timely initiation of biologics may prevent significant visual impairment.

Background: Behcet's disease (BD) is an inflammatory disorder known for various symptoms, including oral and genital ulcers and ocular inflammation. Panuveitis, a severe eye condition, is rare as the first sign of BD., Case Summary: We present an unusual case of a 30-year-old man who developed panuveitis after receiving the mRNA-based coronavirus disease 2019 (COVID-19) vaccine (Moderna). Laboratory tests ruled out infections, but he had a positive HLA-B51 result and a history of genital ulcer and oral ulcers, leading to a BD diagnosis. Treatment with corticosteroids improved his condition. Interestingly, he had another episode of panuveitis after the second mRNA vaccine dose, which also responded to corticosteroids., Conclusion: This case highlights the rare onset of BD following mRNA COVID-19 vaccination, suggesting a potential link between these vaccines and BD's eye symptoms, emphasizing the importance of quick treatment in similar cases., Competing Interests: Conflict-of-interest statement: All authors declare that there are no conflicts of interest for this paper., (©The Author(s) 2024. Published by Baishideng Publishing Group Inc. All rights reserved.)

Purpose: To report a case of bilateral exudative retinal detachments and panuveitis in a patient with multiple myeloma (MM)., Case Report: A 54-year-old patient with non-proliferative diabetic retinopathy was referred with blurred vision and scotomas in both eyes (OU). Three months prior to the onset of ocular symptoms, he was diagnosed with systemic MM and was receiving chemotherapy. Clinical examination revealed best-corrected visual acuities of 20/80 OU, rare anterior chamber cell, 2+ vitreous cell, diffuse intraretinal hemorrhages, and exudative retinal detachments (RD). Optical coherence tomography of the macula showed central subretinal fluid with cystic intraretinal fluid OU. The findings were consistent with panuveitis and exudative RD in the setting of MM. He reported symptomatic improvement after plasmapheresis and oral prednisone initiation., Conclusion: Extensive, bilateral exudative RD and panuveitis are rare but potentially sight-threatening findings in patients with MM.

Introduction: Syphilis exhibits a wide range of clinical presentations, mimicking various systemic and ocular diseases. Ocular syphilis, in particular, manifests with multiple presentations, ranging from anterior uveitis to panuveitis, making it a potential differential diagnosis for Behçet's uveitis. Here, we present a unique case of Behçet's panuveitis that was undergoing immunomodulatory therapy and was complicated by ocular syphilis. Notably, this case also featured rare cutaneous manifestations associated with secondary syphilis, commonly known as malignant syphilis., Case Presentation: A 38-year-old Thai man with refractory end-stage Behçet's panuveitis reported a maculopapular rash accompanied by increased intraocular inflammation. The escalation of immunomodulatory therapy, intended to manage the provisional diagnosis of active ocular and cutaneous Behçet's disease, resulted in clinical deterioration, with the rash transforming into multiple noduloulcerative lesions. Despite negative serologic tests for syphilis at baseline before initiating immunomodulatory therapy, syphilis infection was eventually diagnosed following reevaluation and the observation of spirochetes in a skin biopsy specimen. The patient was treated with intravenous penicillin G, resulting in an improvement in intraocular inflammation and resolution of noduloulcerative rashes., Conclusion: Intraocular inflammation and mucocutaneous lesions in syphilis can mimic the presentation of Behçet's disease. The introduction of immunomodulatory therapy may alter the clinical picture, leading to a severe and atypical presentation. A high index of suspicion for reevaluating serologic tests or performing tissue biopsies is warranted for an accurate diagnosis., Competing Interests: The authors have no conflicts of interest to declare., (© 2024 The Author(s). Published by S. Karger AG, Basel.)

Purpose: To report a case of isolated conjunctival inflammation as initial manifestation of IgG4-related disease and subsequent development of panuveitis., Case Report: A 75-year-old female presented with a diffuse mass lesion in the temporal area of the left eye, involving the conjunctiva, and an abscessed corneal ulcer. An incisional biopsy was diagnostic of IgG4-related disease with an elevated IgG4/IgG ratio (>40%) and the presence of >10 cells that tested positive for IgG4/CGA. No other ocular, orbital or systemic manifestations were noted at the time of diagnosis. After a year of treatment with topical dexamethasone, oral prednisone, and methotrexate, the patient developed panuveitis, which was controlled by increasing steroids and switching to rituximab., Conclusion: IgG4-related disease is a rare entity that can be particularly challenging to diagnose if it manifests in an atypical manner. Continuous follow-up of patients is crucial as relapses and worsening of symptoms can occur despite treatment.

Objective: To describe a case of bilateral retinal vasculitis due to presumed sarcoidosis and rickettsial retinitis complicated with neovascularization with tear biomarker analysis., Methods: A retrospective case report., Results: A 16-year-old male presented with bilateral retinal vasculitis and retinitis in both eyes with inferotemporal quadrant neovascularization in the right eye. Multimodal imaging revealed the presence of active inflammation in both eyes. Weil Felix test was positive with raised ACE levels. This patient was treated with local and systemic steroids, doxycycline, and laser photocoagulation followed by oral methotrexate therapy which resulted in clinical resolution with recovery of visual acuity. Tear biomarker analysis showed raised sICAM-1 and MMP-9 levels in both eyes which significantly reduced following treatment., Conclusion: Ocular sarcoidosis with rickettsial infection is a rare association. Tear biomarkers correlated well with clinical and imaging manifestations. High index of suspicion and aggressive anti-inflammatory therapy can help control inflammation and restore good vision., Competing Interests: Declaration of conflicting interestsThe authors declared no potential conflicts of interest with respect to the research, authorship, and/or publication of this article.

Purpose: To report the management and outcome of VKH disease in a 4-year-old boy with a review of literature on VKH in preschool children (3 to 5 years)., Method: Retrospective chart and literature review., Result: A 4-year-old boy presented with visual acuity of light perception in both eyes with panuveitis, secondary cataracts and intraocular pressure (IOP) of ≥40 mmHg in both eyes. He was started on oral corticosteroid, immunosuppressive and underwent glaucoma filtering surgery and lensectomy with vitrectomy after adequate control of intraocular inflammation. Over time his IOP was again uncontrolled even with maximum anti-glaucoma medications, and he underwent Ahmed glaucoma valve (AGV) implantation, and he gained a visual acuity of 20/60 in both eyes with aphakic correction at the last visit., Conclusion: Paediatric VKH follows an aggressive disease phase and requires a comprehensive multispeciality management approach.

Introduction: Blau syndrome (BS) is a rare autoimmune disease. We report here an atypical case of BS., Case Presentation: We present a case of late-onset eye manifestations in a Chinese girl of 18 years old with sporadic BS, presenting with panuveitis. We performed comprehensive ocular examinations including fluorescein fundus angiography and indocyanine green angiography for her. The oral hormone plus local anti-inflammatory eye drops have well controlled the inflammation of her eyes., Conclusion: Our case highlights the necessity of systemic medical history inquiry for every eye discomfort., Competing Interests: The authors declare that there is no conflict of interest., (© 2024 The Author(s). Published by S. Karger AG, Basel.)

Background: To determine the prevalence, clinical characteristics, and independent predictors of uveitic macular edema (UME) in patients with intermediate, posterior and panuveitis., Methods: We retrospectively reviewed the records of patients with intermediate, posterior, and panuveitis who underwent macular assessment using optical coherence tomography between January 2015 and February 2020. The prevalence of UME and clinical characteristics of the patients were described. Predictors of UME were identified using multivariate regression analysis., Results: A total of 349 patients were included. The mean age was 41 years, female: male ratio was 1.3:1. The prevalence of UME was 51.9%. UME was found in 33.9%, 56.9%, and 54.1% of the intermediate, posterior, and panuveitis cases, respectively. Among patients with UME, 47% had infectious uveitis, 32.6% had idiopathic uveitis, and 20.4% had immune-mediated uveitis. Diffuse macular edema was the most frequently observed pattern (36.5%). Multivariate analysis showed that factors independently associated with UME included age at uveitis onset (adjusted odds ratio [aOR] 1.01, 95% confidence interval [CI] 1.00-1.03, P = 0.036), PU and panuveitis compared with intermediate uveitis (aOR 2.09, 95% CI 1.14-3.86, P = 0.018), and infectious uveitis compared with noninfectious uveitis (aOR 2.13, 95% CI 1.34-3.37, P = 0.001)., Conclusions: Increasing age at uveitis onset, posterior/panuveitis, and infectious etiology are predictive factors for UME in patients with intermediate, posterior and panuveitis., (© 2024. The Author(s).)

Rosai-Dorfman disease (RDD) is a rare non-Langerhans cell histiocytosis characterized by massive lymphadenopathy and systemic extranodal lesions. We present the case of a 28-year-old woman who presented with recurrent blurred vision in her right eye for 3 months. She developed blindness and atrophy in her left eye a decade prior to presentation. She subsequently developed headache, fever, and impaired mental status. Cranial magnetic resonance imaging indicated hypertrophic pachymeningitis (HP), and 18 F-fluoro-2-deoxy-2-d-glucose (FDG) positron emission tomography/computed tomography revealed significant FDG uptake in the left dura mater. Autoimmune testing revealed elevated anti-nuclear, anti-SS-A, and anti-SS-B antibody levels. Incisional biopsy of the atrophic eyeball revealed RDD with marked polyclonal plasmacytosis. The patient was diagnosed with RDD accompanied by multisystem involvement, including Sjögren's syndrome (SS), panuveitis, and HP. Treatment with methylprednisolone for several weeks resulted in significant improvement. This is the first reported case of RDD presenting with SS in combination with panuveitis and HP. Although RDD is rarely diagnosed in young patients, interdisciplinary collaboration is essential to prevent a delayed diagnosis., Competing Interests: Declaration of conflicting interestsThe authors declare no competing interests.

Introduction: Panuveitis is a serious inflammatory disease. Setae are fibers produced by many insects and plants. Many case reports have described caterpillar and spider fibers entering the eye. These hairs are covered with tiny barbs that help them enter and migrate into the eye tissue, leading to severe inflammation. Normally, they are buried mainly in the conjunctiva and cornea. However, in the present case, they entered the posterior segment of the eye, which is very rare., Case Description: A female patient presented with a complaint of repeated foreign body sensation, redness, pain, and photophobia in the left eye for 9 years following initial exposure to caterpillars. She visited the doctor in January after aggravation of symptoms. Slit-lamp examination, ultrasound biomicroscopy, ultrasound B-scan, fluorescein angiography, and indocyanine green angiography revealed the presence of a foreign body in the vitreous, which caused discomfort in the patient's left eye and subsequently, panuveitis of the left eye. The symptoms improved significantly after foreign body removal using vitrectomy. Microscopic examination revealed that the foreign body was caterpillar setae., Conclusion: Clinicians should be more vigilant about history of contact with caterpillars while examining uveitis that has recurred for many years. Identification of the root cause of the disease can ensure better treatment., Competing Interests: Declaration of conflicting interestsThe author(s) declared no potential conflicts of interest with respect to the research, authorship, and/or publication of this article.

Background: Syphilis has historically been referred to as "the great imitator", for the extent of disease manifestations secondary to infection. Ocular manifestations include a wide range of intra-ocular inflammation., Methods: In this study, we report the case of a 52 years-old male patient with syphilitic hemorrhagic necrotizing retinitis., Results: The patient presented to the emergency room for rapid and progressive vision loss and ocular redness lasting three weeks and was under immunosuppressive treatment. The diagnosis was syphilitic hemorrhagic necrotizing retinitis mimicking the typical clinical picture of retinitis caused by Cytomegalovirus infection in immunocompromised patients., Conclusions: The presented case highlights the need to consider ocular syphilis as a great masquerader even in the presence of atypical presentations such as hemorrhagic retinitis. Syphilis should be tested for treponemal and non-treponemal tests, and it should be ruled out as an etiological agent in every case of new-onset intra-ocular inflammation., Competing Interests: Declaration of conflicting interestsThe author(s) declared no potential conflicts of interest with respect to the research, authorship, and/or publication of this article.

Coronavirus disease 2019 (COVID-19) can manifest with ocular symptoms. These symptoms can be divided into isolated events attributed to COVID-19, and those occurring in multisystem inflammatory syndrome in children (MIS-C), a newly diagnosed disease entity associated with COVID-19 infection. Currently, the literature lacks specific guidelines and treatment regimens for COVID-19 ocular symptoms, especially in children. The authors present the case of a 14-and-a-half-year-old boy with bilateral uveitis of the anterior and posterior segments along with vasculitis and optic neuritis associated with SARS-CoV-2 infection. The authors also perform an up-to-date review of all available publications on the treatment of post-COVID-19 uveitis in children described in the literature between 2020 and 2023. In the case described by the authors, the treatment involved a Depo-Medrol 40 mg/mL injection uder the Tenon capsule, with two subconjunctival injections of epinephrine, topical steroid therapy and non-steroidal anti-inflammatory drugs: dexamethasone 0.1%; diclofenac eye drops. In addition, acetylsalicylic acid (150 mg) and pentoxifylline (100 mg, orally) were administered throughout the course of the disease as well as up to 12 months after its termination, until a complete improvement in visual acuity and the withdrawal of ocular lesions were achieved. It can be assumed that this type of treatment is far more beneficial for pediatric patients, with an effect comparable to systemic steroid administration with a preserved improvement in retinal-vascular circulation, without exposing the child to systemic post-steroid complications.

Purpose: To present the long-term clinical outcomes of patients with sympathetic ophthalmia (SO)., Methods: Retrospective review of patients' medical files between 2002 and 2022., Results: Included were seven patients (four males). The mean ± SD age at presentation was 37.9 ± 22.5 years. Four patients had co-morbidities: three had diabetes mellitus type 2 and one had Turner Syndrome. Trauma was the inciting event in six patients and postoperative endophthalmitis in one patient. Decreased visual acuity (VA) was the leading symptom in the sympathizing eye and all of the patients presented with panuveitis. The mean ± SD interval between the triggering incident and the onset of SO in six cases was 4.3 ± 4.2 months. One case presented 30 years following the eye injury. Five patients underwent enucleation/evisceration of the exciting eye. The mean ± SD presenting LogMAR BCVA in the sympathizing eye was 0.57 ± 0.82, and the final LogMAR BCVA was 0.61 ± 0.95. Inflammation was completely controlled in 5 patients at a mean ± SD of 8.55 ± 9.21 months following the institution of immunomodulatory therapy, and it was partially controlled in 2 patients. VA deteriorated in all 3 diabetic patients and improved or remained stable in the 4 young and healthy patients. The mean ± SD follow-up period after achieving drug-free remission was 28 ± 22.8 months. The mean ± SD follow-up time was 6.8 ± 5.6 years., Conclusions: SO is one of the most sight-threatening conditions, affecting the healthy eye. In this cohort, the favorable visual outcome was especially seen in young and healthy individuals. Visual prognosis is directly related to prompt diagnosis and treatment., (© 2024. The Author(s).)

Background: Acute retinal necrosis (ARN) is a progressive necrotizing retinitis caused by viral infection. Optimal management strategies have not been established for this detrimental disease. Previous literature published suggests that Varicella-zoster virus (VZV) and Herpes simplex virus-1 (HSV1) are the most common promoters of acute retinal necrosis (ARN)., Aims: The purpose of our study was to investigate the viral distribution, demographic, and treatment outcomes of ARN., Methods: A retrospective chart review evaluated data from PCR-positive ARN patients diagnosed between 2009 and 2018., Results: Analysis of fourteen eyes from 12 patients found CMV and VZV as the commonest causes of ARN. Patients on 1 g of valacyclovir three times a day (V1T) had worse vision between first and final visits (mean difference of 1.25 ± 0.65, n = 2) compared with patients treated with 2 g of valacyclovir three times a day (V2T), or 900 mg twice a day of valganciclovir (V9B) (mean difference of - 0.067 ± 0.13, n = 6, and 0.067 ± 0.067, n = 6, respectively). Both V1T patients developed retinal detachments (RD). Both CMV patients treated with intravitreal triamcinolone developed ARN, elevated IOP, and one developed multiple RD., Conclusions: Our review found increased incidence of CMV-positive ARN. Patients with zone 1 disease had worse initial visual acuity. Moreover, patients had more favorable outcomes with V2T and V9B compared to V1T. CMV-positive patients clinically worsened after intravitreal steroid injections, further underscoring the value of a PCR diagnosis to tailor the patients' treatment plan accordingly., (© 2023. The Author(s), under exclusive licence to Royal Academy of Medicine in Ireland.)

Purpose: To evaluate the ocular features, course, and prognosis of uveitis in the ocular sarcoidosis (OS) subgroups without previously known systemic sarcoidosis., Methods: Ninety-one eyes of 49 patients were included. Definite OS patients were classified as group 1 ( n = 15), presumed OS patients as group 2 ( n = 15), and probable OS patients as group 3 ( n = 19)., Results:  The most common presentation of OS was panuveitis in all groups. During the follow-up, bilateral ocular involvement was observed in 85.7% of the patients. The most common extraocular involvement was pulmonary involvement, which was detected in 61.2% of the patients at the time of diagnosis., Conclusion: Ophthalmologists should be aware that uveitis may be the first sign of systemic sarcoidosis. In all subgroups of OS, uveitis tends to be bilateral and the most common type is panuveitis. Therefore, the eye without obvious clinical findings should be carefully evaluated clinically and angiographically.

This is a unique case of retinoschisis as an ocular manifestation of brucellosis. A 38-year-old male presented with recurrent episodes of bilateral eye redness, predominately in his left eye. His visual acuity was not affected, and he did not report any other symptoms. On slit lamp examination, binocular Koeppe nodules of the iris and cells in the left anterior chamber were observed. Fundoscopy followed by meticulous multimodal imaging confirmed left inferior retinoschisis. The patient was diagnosed with panuveitis, and a series of laboratory examinations revealed positive anti-IgM Brucella antibodies. Ocular brucellosis can cause variable, atypical, and serious presentations, hence, early diagnosis is paramount to avoid complications., Competing Interests: The authors declare that they have no conflict of interest., (Thieme. All rights reserved.)

Purpose: To examine the long-term efficacy and safety of adalimumab (ADA) in patients with Behçet uveitis (BU)., Methods: A systematic review and meta-analysis of observational studies was performed. Pooled results are presented as mean difference or standardized mean difference (std diff) and 95% confidence intervals (CI). Visual acuity (VA), intraocular inflammation grade, central macular thickness, corticosteroid (CS) sparing effect and adverse events were evaluated., Results: Ten studies were included finally for quantitative and qualitative synthesis. ADA therapy resulted in 0.124 (95%CI: 0.084, 0.165) logMAR improvement in VA. In addition, ADA therapy resulted in decreased grade of intraocular inflammation [std diff, -1.187 (95%CI: -1.508, -0.866)] and macular thickness [std diff, -0.564 (95%CI: -0.843, -0.286)] and caused a decrease in CS dosage [std diff, -1.809 (95%CI: -2.420, -1.198)]. The pooled rate of overall adverse events for ADA in 301 patients was 8.5% (95%CI: 0.039, 0.177)., Conclusion: ADA is an efficient therapy that improves VA and controls intraocular inflammation, macular edema and retinal vasculitis. As the disease exposure time increased, improvement in VA was less. The safety and CS-sparing effect of ADA were demonstrated with few adverse effects. The results provided evidence that ADA can be used safely and efficiently as the first-line drug in patients with BU.

Aims: We present the clinical spectrum, the initial clinical presentation with management trends in treating 14 Vogt-Koyanagi-Harada (VKH) disease cases in a tertiary center in the Northern part of Malaysia., Case Series: There were 14 cases of Vogt-Koyanagi-Harada (VKH) disease retrospectively reviewed over five years (from 2015 to 2020). The mean age at presentation was 37.7 years (range 21-64 years), with female predominance (85.7%). All cases presented with acute uveitic stage and bilateral eye involvement. Of them, 11 (78.6%) were probable VKH, and three (21.4%) were incomplete VKH. All patients attended with acute panuveitis at first presentation. The main posterior segment involvement was disc edema in 57.1% (16 out of 28 eyes) and exudative retinal detachment (ERD) in 35.7% (10 out of 28 eyes). Most of them presented with blindness (3/60 and worse) and moderate visual impair- ment (6/18-6/60); 35.71% each, followed by mild visual impairment (6/12-6/18), and severe visual impairment (6/60-3/60); 7.1% each. Ten patients (71.4%) required combination second-line immunomodulatory treatment during subsequent visits, and only four patients (28.6%) responded well to corticosteroid therapy. Most of the cases achieved no visual impairment (64.3%), followed by mild visual impairment (21.4%) and moderate visual impairment (14.3%), and none were severe or blind at the end of follow-up., Conclusion: VKH is a potentially blinding illness if there is inadequate control of the disease in the acute stage. Most of our patients achieved good visual outcomes with early immunomodulatory treatment and systemic corticosteroids.

Key Clinical Message: Ocular brucellosis is a potential cause of endogenous endophthalmitis in endemic areas, which can be associated with sight-threatening complications., Abstract: To report a patient with unusual fulminant endogenous endophthalmitis due to Brucella melitensis . A 25-year-old woman with a history of fever and right shoulder pain from 4 months ago and a positive Wright test presented with acute panuveitis in her right eye. All laboratory tests were unremarkable except for the positive polymerase chain reaction (PCR) test of the vitreous sample for B . melitensis . Despite the therapeutic efforts, including multiple vitreoretinal surgeries, and intravitreal and systemic antibiotics, the patient's final follow-up examination after 6 months revealed hand motion vision, hypotonia, and pre-phthisis bulbi status. The fellow eye was entirely normal. Brucella endogenous endophthalmitis can be fulminant and result in poor visual outcomes. It is suggested to consider ocular brucellosis as a potential cause of endogenous endophthalmitis in endemic areas., Competing Interests: The authors declare that they have no competing interests., (© 2023 The Authors. Clinical Case Reports published by John Wiley & Sons Ltd.)

Case Histories: Medical records of four dogs diagnosed with protothecosis in New Zealand were reviewed. The dogs were aged between 4 and 9 years and three of the four dogs were female. Breeds were one Labrador, one Miniature Schnauzer and two crossbreeds. The reasons for initial veterinary evaluation were a cough and opaque appearance of the right eye (Case 1), diarrhoea (Cases 2 and 3), and cutaneous disease (Case 4)., Clinical Findings: The ocular signs were characterised by panuveitis, retinal detachment and secondary glaucoma. Gastrointestinal signs included chronic haemorrhagic diarrhoea due to colitis. Three cases had disseminated infection and developed both bilateral, blinding, ocular disease and chronic gastrointestinal disease. Cutaneous signs consisted of draining fistulae over the olecranon, multifocal cutaneous nodules, and ulceration and tracts of the foot pads. Disseminated protothecosis was confirmed by histopathology of biopsied ocular tissues in Cases 1 and 2 and by gastrointestinal biopsies in Case 3. Prototheca spp. were also identified in cytological specimens from Cases 1 and 4 and recovered by culture in Cases 2 and 4. Cutaneous protothecosis was diagnosed in Case 4 initially by cytology and histopathology of skin lesions, and Prototheca zopfii was confirmed by PCR of cultured organisms., Treatment and Outcome: Prior to diagnosis of protothecosis, a variety of treatments were prescribed to treat the gastrointestinal and ocular signs. After diagnosis, only Cases 2 and 4 received medication aimed at treating the protothecal infection, which was itraconazole in both cases. Following the progression of clinical signs and concerns about quality of life, all four dogs were euthanised., Diagnosis: Disseminated protothecosis in three dogs, cutaneous protothecosis in one dog., Clinical Relevance: Canine protothecosis is rarely reported, despite the ubiquity of the causal algae, and the disease usually carries an extremely grave prognosis when infection is generalised. In New Zealand, protothecosis should be considered as a differential diagnosis in dogs with panuveitis, chorioretinitis or retinal detachment, colitis, or nodular, ulcerative or fistulating cutaneous lesions.

Objective: To develop evidence-based expert-consensus recommendations for the management of non-infectious, non-neoplastic, non-demyelinating disease associated uveitis., Methods: Clinical research questions relevant to the objective of the document were identified, and reformulated into PICO format (patient, intervention, comparison, outcome) by a panel of experts selected based on their experience in the field. A systematic review of the available evidence was conducted, and evidence was graded according to GRADE (Grading of Recommendations Assessment, Development, and Evaluation) criteria. Subsequently, recommendations were developed., Results: Three PICO questions were constructed referring to uveitis anterior, non-anterior and complicated with macular edema. A total of 19 recommendations were formulated, based on the evidence found and/or expert consensus., Conclusions: Here we present the first official recommendations of the Spanish Society of Rheumatology for the treatment of non-infectious and non-demyelinating disease associated uveitis. They can be directly applied to the Spanish healthcare system as a tool for assistance and therapeutic homogenisation., (Copyright © 2023 Elsevier España, S.L.U. and Sociedad Española de Reumatología y Colegio Mexicano de Reumatología. All rights reserved.)

Background: Pediatric uveitis is a severe inflammatory ocular condition that can lead to sight-threatening complications and can negatively impact quality of life. The retinal microcirculation is often affected in intermediate uveitis and panuveitis. Here, we examined the extraocular (i.e., systemic) microcirculation in pediatric uveitis cases and healthy controls using nailfold capillaroscopy (NFC)., Methods: We performed NFC in 119 children with noninfectious uveitis and 25 healthy pediatric controls, and assessed the following parameters: capillary density (number of capillaries/mm), dilated capillaries (apex > 20 µm), avascular area, the presence of microhemorrhages, and capillary morphology. Differences in NFC parameters between cases and controls were calculated using regression analysis after adjusting for age and sex., Results: The mean (± SD) age of the patient group was 13.7 (± 3) years, with 56% females; 46%, 18%, and 36% of cases presented as anterior uveitis, intermediate uveitis, and panuveitis, respectively, with an overall mean disease duration of 4.7 (± 4.0) years. Compared to the control group, the pediatric uveitis cases had a significantly higher number of dilated capillaries/mm and a higher prevalence of ramified capillaries. Moreover, compared to the control group the intermediate uveitis cases had a significantly higher number of dilated capillaries, whereas the anterior uveitis cases had a lower capillary density and a higher prevalence of ramified capillaries., Conclusions: Children with uveitis without systemic disease can present with changes in systemic microcirculation. These changes vary amongst the subtypes of uveitis., (© 2023. BioMed Central Ltd., part of Springer Nature.)

Introduction: VKH is a primary stromal choroiditis. Studies with indocyanine green angiography (ICGA) have shown that inflammation begins at the choroid and may persist without clinically apparent inflammation. Thus, systemic therapy must target choroidal inflammation and be maintained until the choroiditis resolves, as proven by ICG and/or EDI-OCT imaging studies., Discussion: Aggressive therapy with oral corticosteroids, with or without "pulsed" intravenous methylprednisolone, has been the mainstay of VKH therapy in the acute phase for many years. However, there is convincing evidence that corticosteroid monotherapy is insufficient to prevent progression onto the chronic recurrent form of the disease, which is associated with poor visual outcomes., Conclusion: Numerous studies suggest that combined therapy with systemic corticosteroids and immunosuppressants is necessary to prevent the progression toward chronic recurrent disease, avoiding complications, get a better control of inflammation and visual outcomes in VKH patients seen at the uveitic phase of the disease.

Purpose: Clinical trials in uveitis have led to the expansion of therapeutic options for the management of non-infectious uveitis. The purpose of this systematic review is to investigate why some clinical trials have yielded successful results and regulatory approval of new therapies, and some have not., Methods: A systematic literature search of the Pubmed/MEDLINE database and clinicaltrials.gov was performed from 2006 to 2021, according to the PRISMA guidelines. Phase III clinical trials of systemic and local therapies in adults with non-infectious intermediate, posterior, and panuveitis were included., Results: A total of 79 clinical trials were collected from ClinicalTrials.gov and PubMed/MEDLINE database search. Based on the inclusion and exclusion criteria, 14 clinical trials were included., Conclusion: This review summarizes the study design, outcome measures, and results of recent phase III trials in non-infectious uveitis, in the interest of understanding limitations and rethinking new methods of defining endpoints in clinical trial design.

Purpose: To investigate the diagnostic value of using retinal nerve fiber layer thickness measured on optical coherence tomography (OCT-RNFL) to diagnose papillitis in pediatric uveitis., Design: Retrospective cohort study., Methods: Demographic and clinical data were collected retrospectively for 257 children with uveitis (with 455 affected eyes). Receiver operating characteristic (ROC) analysis was performed to compare fluorescein angiography (FA, the diagnostic gold standard for papillitis) to OCT-RNFL in a subgroup of 93 patients. An ideal cut-off value for OCT-RNFL was then determined by calculating the highest Youden index. Finally, a multivariate analysis was applied to the clinical ophthalmological data., Results: Based on a subset of 93 patients who underwent both OCT-RNFL and FA, the ideal cut-off OCT-RNFL for diagnosing papillitis was >130 µm, with 79% sensitivity and 85% specificity. Among the entire cohort, the prevalence of OCT-RNFL >130 µm was 19% (27/141), 72% (26/36), and 45% (36/80) in patients with anterior uveitis, intermediate uveitis, and panuveitis, respectively. Our multivariate analysis of the clinical data revealed that OCT-RNFL >130 µm was associated with a higher prevalence of cystoid macular edema, active uveitis, and optic disc swelling on fundoscopy, with odds ratios of 5.3, 4.3, and 13.7, respectively (all P < .001)., Conclusions: OCT-RNFL can be a useful noninvasive additional imaging tool for diagnosing papillitis in pediatric uveitis with relatively high sensitivity and specificity. OCT-RNFL was >130 µm in approximately one-third of all children with uveitis and was particularly prevalent in cases of intermediate uveitis and panuveitis., (Copyright © 2023 The Author(s). Published by Elsevier Inc. All rights reserved.)

Background: Post-streptococcal uveitis syndrome is a rare inflammatory sequela following streptococcus infection most commonly occurring in young patients. Patients most typically present with bilateral anterior nongranulomatous uveitis, but posterior involvement occurs frequently., Case Presentation: We report an immunosuppressed patient presenting with asymmetrically bilateral acute onset, panuveitis with diffuse multifocal retinitis resulting from post-streptococcal uveitis syndrome., Conclusion: Post-streptococcal uveitis syndrome can present as a panuveitis with multifocal retinitis. A high clinical suspicion is needed for this diagnosis especially in patients under the age of 30 years old with bilateral non-granulomatous uveitis.

Ocular and systemic syphilis are well known to mimic other clinical entities making them challenging to diagnose in many cases. Syphilis testing plays an important role in diagnosis and timely treatment. Here we describe a patient with untreated human immunodeficiency virus (HIV) infection who presented with bilateral panuveitis with repeatedly negative syphilis serologies. In light of worsening retinitis while on aggressive anti-viral treatment and in consideration of the clinical suspicion for syphilitic uveitis, intravenous penicillin was initiated empirically. The patient demonstrated significant improvement subjectively and objectively after treatment. We also review and discuss syphilis testing reliability generally and in HIV co-infected patients in particular. Empiric intravenous penicillin should be considered in patients with clinical features of ocular syphilis despite negative serologic testing, especially in those with HIV co-infection.

Background: Uveitis is a term used to describe a group of intraocular inflammatory diseases. Uveitis is the fifth most common cause of vision loss in high-income countries, with the highest incidence of disease in the working-age population. Corticosteroids are the mainstay of treatment for all subtypes of non-infectious uveitis. They can be administered orally, topically with drops, by periocular (around the eye) or intravitreal (inside the eye) injection, or by surgical implantation., Objectives: To determine the efficacy and safety of steroid implants in people with chronic non-infectious posterior uveitis, intermediate uveitis, and panuveitis., Search Methods: We searched CENTRAL (which contains the Cochrane Eyes and Vision Trials Register), MEDLINE Ovid, Embase, PubMed, LILACS, and three trials registries to November 2021., Selection Criteria: We included randomized controlled trials comparing either fluocinolone acetonide (FA) or dexamethasone (DEX) intravitreal implants with standard-of-care therapy or sham procedures, with at least six months of follow-up after treatment. We included studies that enrolled participants of all ages, who had chronic non-infectious posterior uveitis, intermediate uveitis, or panuveitis with vision that was better than hand-motion., Data Collection and Analysis: We applied standard Cochrane methodology., Main Results: We included data from four trials (683 participants, 907 eyes) that compared corticosteroid implants with either sham or standard-of-care therapy. Study characteristics and risk of bias Of the two trials that compared corticosteroid implants with sham procedure, one examined a 0.18 mg FA implant, and the other, a 0.7 mg DEX implant. The other two trials compared a 0.59 mg FA implant with standard-of-care therapy, which included systemic corticosteroids and immunosuppressive medications, if needed. Considering improvement in visual acuity, we assessed the four trials to be at either low risk, or with some concerns of risk of bias across all domains. Findings Using sham procedure as control, combined results at the six-month primary time point suggested that corticosteroid implants may decrease the risk of uveitis recurrence by 60% (relative risk [RR] 0.40, 95% confidence interval [CI] 0.30 to 0.54; 2 trials, 282 participants; low-certainty evidence); and lead to a greater improvement in best-corrected visual acuity (BCVA; mean difference [MD] 0.15 logMAR, 95% CI 0.06 to 0.24; 1 trial, 153 participants; low-certainty evidence). Evidence based on a single-study report (146 participants) suggested that steroid implants may have no effects on visual functioning quality of life, measured on the National Eye Institute 25-Item Visual Function Questionnaire (MD 2.85, 95%CI -3.64 to 9.34; 1 trial, 146 participants; moderate-certainty evidence). Using standard-of care therapy as control, combined estimates at the 24-month primary time point suggested that corticosteroid implants were likely to decrease the risk of recurrence of uveitis by 54% (RR 0.46, 95% CI 0.35 to 0.60; 2 trials, 619 eyes). Combined estimates at 24 months also suggested that steroid implants may have little to no effects on improving BCVA (MD 0.05 logMAR, 95% CI -0.02 to 0.12; 2 trials, 619 eyes; low-certainty evidence). Evidence based on a single-study report (232 participants) suggested that steroid implants may have minimal clinical effects on visual functioning (MD 4.64, 95% CI 0.13 to 9.15; 1 trial, 232 participants; moderate-certainty evidence); physical functioning (SF-36 physical subscale MD 2.95, 95% CI 0.55 to 5.35; 1 trial, 232 participants; moderate-certainty evidence); or mental health (SF-36 mental subscale MD 3.65, 95% CI 0.52 to 6.78; 1 trial, 232 participants; moderate-certainty evidence); but not on EuroQoL (MD 6.17, 95% CI 1.87 to 10.47; 1 trial, 232 participants; moderate-certainty evidence); or EuroQoL-5D scale (MD 0.02, 95% CI -0.04 to 0.08; 1 trial, 232 participants; moderate-certainty evidence). Adverse effects Compared with sham procedures, corticosteroid implants may slightly increase the risk of cataract formation (RR 2.69, 95% CI 1.17 to 6.18; 1 trial, 90 eyes; low-certainty evidence), but not the risk of cataract progression (RR 2.00, 95% CI 0.65 to 6.12; 1 trial, 117 eyes; low-certainty evidence); or the need for surgery (RR 2.98, 95% CI 0.82 to 10.81; 1 trial, 180 eyes; low-certainty evidence), during up to 12 months of follow-up. These implants may increase the risk of elevated intraocular pressure ([IOP] RR 2.81, 95% CI 1.42 to 5.56; 2 trials, 282 participants; moderate-certainty evidence); and the need for IOP-lowering eyedrops (RR 1.85, 95% CI 1.05 to 3.25; 2 trials, 282 participants; moderate-certainty evidence); but not the need for IOP-lowering surgery (RR 0.72, 95% CI 0.13 to 4.17; 2 trials, 282 participants; moderate-certainty evidence). Evidence comparing the 0.59 mg FA implant with standard-of-care suggested that the implant may increase the risk of cataract progression (RR 2.71, 95% CI 2.06 to 3.56; 2 trials, 210 eyes; low-certainty evidence); and the need for surgery (RR 2.98, 95% CI 2.33 to 3.79; 2 trials, 371 eyes; low-certainty evidence); along with the risk of elevated IOP (RR 3.64, 95% CI 2.71 to 4.87; 2 trials, 605 eyes; moderate-certainty evidence); and the need for medical (RR 3.04, 95% CI 2.36 to 3.91; 2 trials, 544 eyes; moderate-certainty evidence); or surgical interventions (RR 5.43, 95% CI 3.12 to 9.45; 2 trials, 599 eyes; moderate-certainty evidence). In either comparison, these implants did not increase the risk for endophthalmitis, retinal tear, or retinal detachment (moderate-certainty evidence)., Authors' Conclusions: Our confidence is limited that local corticosteroid implants are superior to sham therapy or standard-of-care therapy in reducing the risk of uveitis recurrence. We demonstrated different effectiveness on BCVA relative to comparators in people with non-infectious uveitis. Nevertheless, the evidence suggests that these implants may increase the risk of cataract progression and IOP elevation, which will require interventions over time. To better understand the efficacy and safety profiles of corticosteroid implants, we need future trials that examine implants of different doses, used for different durations. The trials should measure core standard outcomes that are universally defined, and measured at comparable follow-up time points., (Copyright © 2023 The Cochrane Collaboration. Published by John Wiley & Sons, Ltd.)

Purpose: Analyse and describe the anatomical and etiological classification, clinical and epidemiological characteristics and most frequent symptoms of uveitis cases in our population., Patients and Method: Descriptive, retrospective observational study of uveitis cases treated in the Ophthalmology Department of Virgen del Rocío University Hospital in 2021. The demographic and clinical characteristics were studied., Results: A total of 109cases of uveitis were studied, 46 men and 63 women, with a mean age of 45.43±16.11 years. The most frequent symptoms were pain (74.31%), hyperemia (73.39%) and blurred vision (65.14%). The most frequent anatomical classification was anterior (55.96%), followed by panuveitis (18.35%), posterior (12.84%) and intermediate uveitis (7.34%). Regarding etiology, most uveitis were idiopathic (42.99%), followed by non-infectious (38.32%) and finally infectious (18.69%)., Conclusions: In southern España, the characteristics of uveitis are similar to other studies in Western countries., (Copyright © 2023 Elsevier España, S.L.U. All rights reserved.)

Purpose: To present a case of panuveitis with granulomatous intraocular inflammation and vasculitis related to coronavirus disease 2019 (COVID-19)., Case Report: A 46-year-old female patient had blurred vision in both eyes during 8 days after COVID-19 symptoms. Anterior segment examination revealed anterior chamber cells in the both eyes, mutton fat keratic precipitates and posterior synechiae in the left eye. Small peripheral iris nodules and mild vitritis were accompanying in both eyes. Fundus examination revealed multiple superficial retinal infiltrate lesions in the peripheral retina with retinal periphlebitis in both eyes. Spectral domain optical coherence tomography showed hyperreflective superficial retinal infiltrates. Fluorescein angiography (FA) and indocyanine green angiography demonstrated hypofluorescent foci due to blockade of retinal infiltrates. Peripheral vascular leakage was detected on FA in both eyes., Conclusion: This case showed that COVID-19 infection can cause granulomatous panuveitis. Autoimmune mechanisms related to COVID-19 may lead to ocular inflammation.

Purpose: To identify the characteristics and outcome of panuveitis of undetermined origin (PUO) after diagnostic vitrectomy., Methods: Retrospective analysis of all patients who underwent vitrectomy for diagnostic/therapeutic purposes from 2013 to 2020, whose vitreous biopsies turned out negative and final diagnoses were not clinically supported., Results: Of 122 operated eyes, 36 eyes (29.5%) were defined as PUO (67.8 ± 14.9 years). The presenting clinical picture revealed a predominantly bilateral condition (70% of eyes) with significant posterior segment involvement: 3.1 ± 0.6 vitritis, 61.1% of eyes with retinal vasculitis, 44.4% with macular edema, and 30.6% with exudative retinal detachment. Presenting visual acuity was 1.2 ± 0.7 logMAR, and up to 90% remained stable or improved vision over a ~ 3.5 year observation period. None of the presenting clinical features turned out to be predictive of final visual outcome or survival., Conclusions: PUO is present in up to 30% of cases after diagnostic/therapeutic vitrectomy. This mainly bilateral condition shows chronic and overall stable long-term outcome, generally with retained steady visual function., (© 2023. The Author(s), under exclusive licence to Springer Nature B.V.)

Purpose: To report panuveitis with exudative retinal detachments in a healthy 27-year-old woman with biallelic mutations in the RPE65 gene, who underwent bilateral sequential gene therapy with subretinal administration of voretigene neparvovec-rzyl., Observations: Visual acuity improved for 30 days after surgery as oral corticosteroids were tapered. At postoperative week 6, vision declined due to sudden onset uveitis and exudative retinal detachments in both eyes. HLA Class II typing revealed the haplotype associated with sympathetic ophthalmia and Vogt-Koyanagi-Harada (VKH). The inflammation improved after corticosteroid, mycophenolate mofetil, and adalimumab therapy while vision remained poor., Conclusions and Importance: Surgically-induced sympathetic ophthalmia is a plausible explanation for the clinical findings; surgery of both eyes within one week would conceal the inciting eye. VKH or inflammation related to the gene therapy are other possible etiologies but severe bilateral panuveitis has not been reported with voretigene neparvovec-rzyl. Informed consent for gene therapy surgery should include a discussion of the rare complication of sympathetic ophthalmia following vitrectomy surgery., Competing Interests: The authors declare that they have no known competing financial interests or personal relationships that could have appeared to influence the work reported in this paper. BLL, NZG, and JLD have participated in clinical trials of gene therapy funded by NightStar, Biogen, AGTC, Nanoscope, Ocugen. NZG has participated in clinical trial and has consulted for Gyroscope Therapeutics., (© 2023 The Authors.)

A 40-year-old Asian Indian woman, diagnosed as having idiopathic panuveitis (elsewhere) 3 years earlier and being treated with oral steroids (20 mg/day) and methotrexate (25 mg/week), presented to us with worsening vision in both eyes. Her best corrected visual acuity (BCVA) was perception of light in her right eye and counting fingers close to face in her left eye. A slit lamp examination showed an anterior chamber (AC) reaction (1+) in both eyes with posterior synechia, a total cataract in her right eye, and pseudophakia in her left eye. The left fundus showed vitritis, vitreous membranes, chorioretinitis, multifocal areas of retinitis, and retinal vascular sheathing. A systemic examination showed extensive multifocal areas of tinea corporis on the hands and torso. Owing to the leukocytosis (22,000 cells/mm 3 ), diagnostic vitrectomy was initially deferred and 100 mg of oral itraconazole was given twice a day for 3 months. The vitritis improved a little and her total white blood cell (WBC) count improved with treatment of the skin infection. Following a diagnostic vitrectomy later in her left eye, resolving areas of retinitis were seen. Complete resolution of eye inflammation was seen at the end of 6 weeks. At the 6-month follow-up, her BCVA was 6/18 in left eye and she was off oral steroids and methotrexate, with no recurrence of inflammation. We speculate a probable association between the ocular inflammation and extensive tinea corporis based on the therapeutic response to itraconazole., Competing Interests: The authors declare that the research was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest., (Copyright © 2023 Konana and Babu.)

Purpose: To report a severe bilateral frosted branch angiitis associated with acute onset of systemic dermatomyositis., Method: History and clinical examination, laboratory evaluation, fundus' and skin's color pictures, fluorescein angiography (FA), optical coherence tomography (OCT)., Results: A 32-year-old female was referred for a bilateral frosted branch angiitis and vitreitis, with skin rash, muscle pain, weakness, severe headache, compatible with dermatomyositis. After an initial improvement following an aggressive treatment by systemic steroids and mycophenolate mofetil, she was switched to intravenous infliximab (RemicadeTM, Janssen Biotech, Inc. USA) due to lack of efficacy. Nine months later, her visual acuity was improved up to 20/20 in OU, with both retinal vasculitis and vitreitis being solved., Conclusion: Dermatomyositis is a severe multiorgan disease which might severely involve eye structures. Anti-TNF-Alpha agents, particularly Infliximab, might offer a rapid control and long-term remission.

Introduction: Sympathetic ophthalmia (SO) is rare, bilateral granulomatous panuveitis that typically occurs following penetrating or perforating ocular trauma or surgery. This review aims to provide an update on the etiopathogenesis, clinical presentations, diagnosis and treatment of SO., Methods: Reports cited in MEDLINE database, that analyzed SO in at least 5 patients, published prior to December 1st, 2021 were included., Results: Initially, SO was associated with penetrating ocular trauma, however, various studies reported an increased incidence of SO after surgical procedures including vitreoretinal surgeries. Multimodal imaging including fluorescein and indocyanine green angiography, optical coherence tomography (OCT) and OCT angiography have added further insights into the understanding of SO. While pulse dose corticosteroids & immunosuppressive drugs are still the treatment of choice, TNF-α blockers & other biologic drugs represent new promising agents., Conclusion: There is a growing pool of evidence in understanding the pathogenesis of SO. Novel treatment options have provided better prognosis for this potentially blinding condition.

Choroidal caverns (CCs) have been described in association with age-related macular degeneration and pachychoroid disease. However, it is unknown if caverns are found in patients with chronic non-infectious uveitis (NIU). Herein, we evaluated patients with NIU who had optical coherence tomography and indocyanine green angiography for CCs. Clinical and demographic characteristics were extracted from the chart review. Univariate and multivariate mixed-effects logistical models were used to assess the association between clinical and demographic factors and the presence of CCs. One hundred thirty-five patients (251 eyes) met the inclusion criteria: 1 eye had anterior uveitis, 5 had intermediate uveitis, 194 had posterior uveitis, and 51 had panuveitis. The prevalence of CCs was 10%. CCs were only observed in patients with posterior and panuveitis, with a prevalence of 10.8% and 7.8%, respectively. Multifocal choroiditis (MFC) was the type of uveitis where CCs were most frequently observed, with 40% of eyes with MFC having CCs. In addition, male sex ( p = 0.024) was associated with CCs. There was no significant difference in the degree of intraocular inflammation or mean subfoveal choroidal thickness between CC+ and CC- eyes. This is the first study to describe CCs in uveitis. Overall, these findings suggest that caverns may be a sequela of structural and/or vascular perturbations in the choroid from uveitis.

Aim: We report a case of a young male who developed bilateral panuveitis after extensive tattooing., Design: Case report., Methods: A 22-year-old male with a history of inflamed tattoos presented with pain in both eyes and blurred vision in the left eye. Clinical examination showed ciliary congestion, flare, vitreous cells in both eyes, and posterior synechiae in the left eye. Optic nerve was swollen in both eyes. OCT scans demonstrated subretinal blood, associated with neurosensory macular detachment in the left eye. The skin tattoo biopsy showed a granulomatous inflammation without evidence of sarcoidosis. Long-term corticosteroid therapy allowed a regression of clinical signs and symptoms with full recovery., Conclusion: TAttoo Granulomas with Uveitis (TAGU) is a syndrome with numerous clinical presentations. In our case, optic nerve head oedema and subretinal hemorrhage at the posterior pole were the presentation signs. Ophthalmologists should always consider TAGU as a diagnosis in patients with a history of inflamed tattoos.

Background: Varicella zoster reactivation is an increasingly recognised event following mRNA COVID-19 vaccination. In addition, various ocular inflammatory and infectious adverse events following COVID-19 vaccination have been described in the literature. This case report describes acute retinal necrosis (ARN) secondary to varicella zoster virus (VZV) reactivation following COVID-19 mRNA vaccination., Case Description: A 42-year-old immunocompetent man developed left ARN 12 days following first dose of Pfizer BioNTech mRNA COVID-19 vaccination. Aqueous and vitreous tap polymerase chain reaction testing was positive for VZV. Good visual outcome was achieved with combination therapy, including intravitreal foscarnet, oral valaciclovir and prednisolone, topical dexamethasone and atropine, and barrier retinal laser. Second dose of the vaccine is planned under cover of high-dose oral valaciclovir therapy., Conclusion: This case illustrates the possible association between COVID-19 vaccination and potentially blinding VZV reactivation. Therefore, prompt ophthalmic assessment is recommended in patients with visual disturbance following COVID-19 vaccination.

Purpose: To report a case of bilateral panuveitis and occlusive vasculitis following COVID-19 vaccination., Study Design: Case report., Results: A 41-year-old otherwise healthy male presented with progressive vision loss and floaters starting 48 hours after a first dose of COVID-19 vaccine. Examination initially showed bilateral anterior uveitis, but this evolved into bilateral panuveitis with occlusive vasculitis despite topical corticosteroids over two weeks. The patient underwent extensive testing for other etiologies which were excluded. He was successfully treated with a gradual taper of topical and systemic corticosteroids leading to improvement of signs and symptoms. Follow-up is maintained for observation of avascular zones with possible neovascularization which could require laser as needed., Conclusions: The temporal association between vaccine and presentation makes this a plausible etiology. This remains a rare adverse event, but clinicians should be aware of this possibility to include it in their differential diagnosis when confronted with idiosyncratic ocular presentations.

Purpose: To report sarcoid-like uveitis with or without tubulointerstitial nephritis (TIN) during coronavirus disease 2019 (COVID-19) and to discuss diagnostic evaluation and treatment., Methods: Detailed information on the subject's demographics, medical history, ophthalmic examination findings, and laboratory results were obtained from medical records. Fluorescein angiography (FA) and indocyanine green angiography (ICGA) images were evaluated., Results: All seven patients aged between 8 and 17 had bilateral granulomatous panuveitis. TIN preceded in four patients. Thorax computed tomography of patient 1 was found to be compatible with COVID-19, patients 2 and 3 were in contact with Severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) positive parents, patients 4 and 5 were found to be SARS-CoV-2 PCR positive, and patients 6 and 7 were positive for SARS-CoV-2 IgG antibodies. ICGA revealed hypofluorescent dots in six patients. Intraocular inflammation was controlled with corticosteroid and/or immunomodulatory therapy. Visual acuity was maintained or improved in all., Conclusion: SARS-CoV-2 infection may be related to sarcoid-like uveitis and acute tubulointerstitial nephritis.