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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. [ABSTRACT FROM AUTHOR]

PurposeMethodsResultsDiscussionTo 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.Case report.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.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. [ABSTRACT FROM AUTHOR]

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. [ABSTRACT FROM AUTHOR]

Abstract 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.

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. [ABSTRACT FROM AUTHOR]

PurposeCase presentationConclusionTo 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.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.Vitrectomy combined with repeated intravitreal injections of ceftazidime can enhance the treatment for brucellosis endophthalmitis and achieve a better visual prognosis. [ABSTRACT FROM AUTHOR]

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. [ABSTRACT FROM AUTHOR]

Acute retinal necrosis (ARN) is a profound infection of the retina, marked by acute panuveitis, retinal periarteritis, and widespread necrotizing retinitis. The etiology of ARN involves human herpesviruses, such as herpes simplex virus (HSV) and varicella-zoster virus (VZV), which can lead to severe visual impairment or even blindness. Diagnosis of ARN is based on clinical characteristics and disease progression according to the standard diagnostic criteria established by the American Uveitis Society (AUS) in 1994. The polymerase chain reaction (PCR) of aqueous specimens can enable identification of the type of virus. Early initiation of antiviral medication is essential for treatment efficacy to stop lesion progression, accelerate the healing process, and prevent contralateral eye involvement. Ocular complications of ARN include atrophic retina, multiple retinal breaks, rhegmatogenous retinal detachment (RRD), tractional retinal detachment (TRD), optic atrophy, macular edema, epiretinal membrane (ERM), and retinal and optic disc neovascularization. This review summarizes the clinical features, diagnostic criteria, and recently recommended ARN management. [ABSTRACT FROM AUTHOR]

Here, we describe a case of Lyme disease presenting as bilateral panuveitis. A 25-year-old woman presented to our clinic with decreased visual acuity of 20/320 and 20/160 in the right and left eye, respectively. An ophthalmic examination revealed the presence of anterior chamber cells 3+, vitreous cells 1+, vitreous haziness 2+/1+, and retinal infiltration in both eyes. She also had fever, headache, and difficulty in breathing. An initial blood analysis did not detect infection; however, high levels of erythrocyte sedimentation rate and C-reactive protein were noted. Pleural and pericardial effusions, and multiple reactive arthritis lesions were observed on chest computed tomography and bone scans, respectively. Oral steroids (30 mg/day) and steroid eye drops were initiated. Ten days later, she was diagnosed with Lyme disease, based on an indirect immunofluorescence antibody test. Ceftriaxone (2 g) was intravenously administered for 2 weeks followed by administration of oral trimethoprim-sulfamethoxazole (400 mg/80 mg/day) for 1 week. Thereafter, she received a 4-week course of doxycycline (100 mg) twice daily. Her symptoms and ocular findings improved; however, a gradually increasing dose of oral steroid was needed to control retinal lesions for some time, since multiple retinitis lesions developed in the peripheral retina after tapering the oral steroid dose to 5 mg/day. In conclusion, panuveitis can occur in patients with Lyme disease and can be treated with systemic antibiotics and steroids [ABSTRACT FROM AUTHOR]

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. [ABSTRACT FROM AUTHOR]

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.

Sympathetic ophthalmia (SO) is a rare bilateral diffuse granulomatous intraocular inflammation, which occurs days or months following surgery or trauma to one eye. After penetrating ocular injuries and during intraocular surgery, the incidence of SO varies from 0.2% to 0.5% and 0.01%, respectively. Other risk factors include cyclodestructive operations and vitreoretinal surgery. With 80% of cases appearing within three months following injury to the exciting eye and 90% occurring within one year, the temporal interval between ocular injury and the onset of SO varies widely, ranging from a few days to decades. The diagnosis is based on clinical findings. It manifests as bilateral diffuse uveitis. In the sympathetic, uninjured eye, patients describe a sneaky beginning of blurred vision, discomfort, epiphora and photophobia. Typically, this is accompanied by conjunctival injection and a granulomatous anterior chamber reaction, resulting in mutton fat keratic precipitates (KPs). The degree of inflammation in the posterior chamber can differ. One of the initial lines of treatment for SO is systemic corticosteroids. Long-term immunomodulatory therapy may involve azathioprine, mycophenolate mofetil, cyclosporine or other immunosuppressive drugs if patients do not respond to steroid therapy or if their side effects are clinically substantial.

Purpose: To report a case of unilateral cytomegalovirus (CMV) panuveitis with occlusive vasculitis after injection of intravitreal dexamethasone implant in a patient with type 2 diabetes mellitus. Methods: Observational case report. Results: A 60-year-old immunocompetent man with well-controlled type 2 diabetes mellitus was treated with intravitreal dexamethasone implant for recurrent uveitis that was responsive to steroids. Three months after implantation, the patient develops panuveitis with occlusive vasculitis. Anterior chamber tap confirms diagnosis of cytomegalovirus retinitis. Intravitreal foscarnet and oral valganciclovir led to quiescent disease. Conclusions: Patients treated with local immunosuppressants should be monitored carefully to assess treatment response and complications, even in the absence of frank immunodeficiency. Quantitative viral PCR can be an effective way to monitor treatment response to antiviral therapy. [ABSTRACT FROM AUTHOR]

An 84-year-old man presented with decreased right-eye visual acuity. Upon initial examination, the rightand left-eye visual acuities were 0.03 and 1.2, respectively; moreover, the right- and left-eye intraocular pressure was 12 mmHg and 13 mmHg, respectively. Examination revealed a shallow anterior chamber of the right eye, anterior chamber inflammation, vitreous opacity, and marked retinochoroidal detachment. Optical coherence tomography (OCT) revealed retinal detachment (RD) and choroidal folds; moreover, B-scan ultrasonography (B-scan) showed RD as well as thickened sclera with fluid in Tenon's space. Fluorescent fundus angiography revealed hyperfluorescence in the optic disc and vascular hyperpermeability in the right eye. The left eye lacked extra-ocular symptoms or abnormalities. The right ocular axis measured 23.4 mm with no apparent subretinal fluid migration due to positional changes. Accordingly, the patient was diagnosed with panuveitis associated with posterior scleritis and immediately started on 40 mg prednisolone, which improved his symptoms. However, at 3 post-treatment months, choroidal folds were observed and was restarted on 20 mg prednisolone. The choroidal folds subsequently disappeared, with a current visual acuity of 0.3 in the right eye and no recurrence. Our findings indicated the utility of accurate diagnosis of posterior scleritis by B-scan and prompt systemic steroid administration. [ABSTRACT FROM AUTHOR]

PurposeMethodsResultsConclusionWe 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).Case series.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.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. [ABSTRACT FROM AUTHOR]

PurposeMethodsResultsConclusionTo 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.Retrospective case reportThe 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.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. [ABSTRACT FROM AUTHOR]

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. [ABSTRACT FROM AUTHOR]

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Acute retinal necrosis (ARN) is a profound infection of the retina, marked by acute panuveitis, retinal periarteritis, and widespread necrotizing retinitis. The etiology of ARN involves human herpesviruses, such as herpes simplex virus (HSV) and varicella-zoster virus (VZV), which can lead to severe visual impairment or even blindness. A diagnosis of ARN is based on clinical characteristics and disease progression according to the standard diagnostic criteria established by the American Uveitis Society (AUS) in 1994. The polymerase chain reaction (PCR) of aqueous specimens can enable identification of the type of virus. Early initiation of antiviral medication is essential for treatment efficacy to stop lesion progression, accelerate the healing process, and prevent contralateral eye involvement. Ocular complications of ARN include atrophic retina, multiple retinal breaks, rhegmatogenous retinal detachment (RRD), tractional retinal detachment (TRD), optic atrophy, macular edema, epiretinal membrane (ERM), and retinal and optic disc neovascularization. This review summarizes the clinical features, diagnostic criteria, and recently recommended ARN management.

Abstract 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.

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.

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. [ABSTRACT FROM AUTHOR]

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. [ABSTRACT FROM AUTHOR]

To evaluate the ocular features, course, and prognosis of uveitis in the ocular sarcoidosis (OS) subgroups without previously known systemic sarcoidosis. 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). 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. 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. [ABSTRACT FROM AUTHOR]

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. [ABSTRACT FROM AUTHOR]

To report a case of isolated conjunctival inflammation as initial manifestation of IgG4-related disease and subsequent development of panuveitis. 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. 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. [ABSTRACT FROM AUTHOR]

To examine the long-term efficacy and safety of adalimumab (ADA) in patients with Behçet uveitis (BU). 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. 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). 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. [ABSTRACT FROM AUTHOR]

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. [ABSTRACT FROM AUTHOR]

Elaborar recomendaciones basadas en la evidencia disponible y el consenso de expertos para el manejo terapéutico de los pacientes con uveítis no infecciosas, no neoplásicas y no asociadas a enfermedad desmielinizante. Se identificaron preguntas clínicas de investigación relevantes para el objetivo del documento, reformuladas en formato PICO (paciente, intervención, comparación, outcome o desenlace) por un panel de expertos seleccionados en base a su experiencia en el área. Se realizó una revisión sistemática de la evidencia, graduándose de acuerdo a los criterios Grading of Recommendations Assessment, Development, and Evaluation (GRADE). Subsecuentemente, se formularon las recomendaciones. Se seleccionaron tres preguntas PICO, referentes a uveítis anteriores, no anteriores y complicadas con edema macular. Se formularon un total de 19 recomendaciones con base en la evidencia encontrada y/o en el consenso de expertos. Se presenta el primer documento oficial de la Sociedad Española de Reumatología de recomendaciones para el tratamiento de las uveítis. Pueden aplicarse directamente al sistema sanitario español como herramienta de ayuda y homogenización terapéutica. To develop evidence-based expert-consensus recommendations for the management of non-infectious, non-neoplastic, non-demyelinating disease associated uveitis. 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. 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. 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. [ABSTRACT FROM AUTHOR]

Key Clinical Message: Ocular brucellosis is a potential cause of endogenous endophthalmitis in endemic areas, which can be associated with sight‐threatening complications. 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. [ABSTRACT FROM AUTHOR]

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). 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. 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. Disseminated protothecosis in three dogs, cutaneous protothecosis in one dog. 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. [ABSTRACT FROM AUTHOR]

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.

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. 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. 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. 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. [ABSTRACT FROM AUTHOR]

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. [ABSTRACT FROM AUTHOR]

To present a case of panuveitis with granulomatous intraocular inflammation and vasculitis related to coronavirus disease 2019 (COVID-19). 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. This case showed that COVID-19 infection can cause granulomatous panuveitis. Autoimmune mechanisms related to COVID-19 may lead to ocular inflammation. [ABSTRACT FROM AUTHOR]

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. [ABSTRACT FROM AUTHOR]