A chronic myeloid leukemia presenting as panuveitis combined with retinal and choroidal vascular occlusion: a case report.

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.

Behcet's disease-related panuveitis following COVID-19 vaccination: A case report.

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.

Vitrectomy Combined with Repeated Intravitreal Injection of Ceftazidime for the Treatment of Brucellosis Endophthalmitis: A Case Report and Literature Review.

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.

Bilateral Panuveitis After Endometrial Cancer Treatment with Dostarlimab: A Case Report.

PURPOSE: To report a case of bilateral panuveitis following Dostarlimab therapy for endometrial cancer. METHODS: Case report. RESULTS: A 73-year-old woman with a history of endometrial cancer, previously treated with Dostarlimab (an immune checkpoint inhibitor), presented to the ophthalmology emergency department with decreased visual acuity in her left eye. Her medical history included immune-mediated thyroid involvement, which led to the discontinuation of Dostarlimab. Subsequently, she developed pneumonitis and pancreatitis. On initial examination in the emergency room, she had poor vision (hand motion in the right eye and 20/200 on the Snellen chart in the left eye), an inflammatory reaction in the anterior chamber, with 1+ cells, small keratic precipitates and broad posterior synechiae in both eyes. Examination of the posterior pole revealed 3+ vitritis, which made it difficult to evaluate the retina. Treatment with oral and topical corticosteroids was initiated, resulting in an improvement in symptoms and the resolution of intraocular inflammation in several weeks. CONCLUSION: Immune checkpoint inhibitors are used in the treatment of various types of cancer, with their efficacy demonstrated in different clinical trials. However, they are also associated with a range of adverse reactions. To date, no ocular adverse reactions related to the use of Dostarlimab have been published. A case of bilateral panuveitis potentially induced by Dostarlimab is described.

Categories and Profiles of Uveitis in Vogt-Koyanagi-Harada Syndrome With Systemic Correlation: Inferences From a Tertiary Multispecialty Hospital.

Introduction Vogt-Koyanagi-Harada (VKH) syndrome is a granulomatous, autoimmune panuveitis, affecting the eyes, ears, skin, and meninges. It can cause choroiditis and can progress to the retina and optic disc causing visual loss. Imaging using fundus fluorescein angiography (FFA), indocyanine green angiography (ICGA), and enhanced depth imaging-ocular coherence tomography (EDI-OCT) is required for clinical evaluation and management. Steroids and immunosuppression are the treatment modalities used. Aim The aim of this study is to report the correlation and severity of uveitis in relation to systemic manifestations. Method A retrospective study including 100 patients with VKH syndrome was carried out. They were classified based on clinical manifestations and investigations such as FFA, ICGA, B-scan ultrasonography (USG), and ocular coherence tomography (OCT). Patients were characterized as complete, incomplete, and probable VKH syndrome. Laboratory investigations were performed, and statistical analysis was done. Results Probable VKH syndrome was found to be the most common form of presentation in our study population. Defective vision was the most common complaint among the patients. Extraocular manifestations included tinnitus, vertigo, alopecia, headache, fatigue, and vitiligo and were seen in 33% of the patients. Disc edema and serous retinal detachment were seen in 85% of the patients. Improvement was noted in 25% of the patients with the use of corticosteroids. Conclusion Response to treatment with systemic corticosteroids and immunosuppression in the acute phase of uveitis is better compared to chronic uveitis. The ophthalmologist is usually first consulted in VKH syndrome due to presenting ocular complaints. A multidisciplinary approach is key to providing holistic management.

Severe Cutaneous Manifestation of Malignant Syphilis in a Patient with Behçet's Uveitis: A Case Report.

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.

Occlusive Vasculitis Following Intravitreal Rituximab Injection for Primary Vitreoretinal Lymphoma.

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.

Subretinal Fibrosis Developing 10 Years After First Presentation with Chronic Ocular Sarcoidosis in a Child.

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.

Ocular inflammation in tsutsugamushi disease - a case report.

A 64-year-old male, working at a mountain site in Taitung County, suffered from primary open angle glaucoma (POAG) post trabeculectomy with well-controlled intraocular pressure (IOP) in both eyes (OU). He presented with headache accompanied by red eyes (OU) for 10 days. Physical examination revealed fever up to 38.2°C, neck stiffness, one eschar at the left forearm and another at the left ankle. Abnormal laboratory data indicated bacterial infection with central nervous system involvement. Ophthalmic examination showed elevated IOP, moderate conjunctival congestion, subconjunctival hemorrhage, anterior uveitis, cotton-wool spots on the retina and multiple white dots on the temporal retina (OU). Under the impression of uveitis in tsutsugamushi disease with atypical meningitis, oral doxycycline, anti-glaucoma and anti-inflammation eye drugs were prescribed. IOP returned to 12 mmHg and anterior uveitis subsided. The lesions of cotton-wool spots on the retina disappeared within 2 weeks, but multiple white dots remained persistently on the temporal retina.

Full-Thickness Macular Hole: An Exceptional Case Presentation in Behçet's Disease.

Behçet's disease (BD) is a vascular disorder affecting a variety of organ systems. It is an auto-immune disease with inflammatory vasculitis that is systemic in nature, the exact etiology of which is unknown. Obliterative vasculitis, recurrent aphthous ulcers, mucocutaneous manifestations, recurrent genital ulcerations, and intraocular inflammation, especially chronic relapsing uveitis, are the characteristic features of BD. The case report presents a unique manifestation of BD in a 20-year-old Pakistani male who presents with a one-year-old history of viral encephalitis, after which he developed a blurring of vision. On examination, he had recurrent aphthous ulcers, recurrent ulcerations of genitalia, and a history of lesions of the skin. After making the patient undergo a cascade of investigations for evaluating and assessing the various signs and symptoms, a diagnosis of BD with bilateral panuveitis and a full-thickness macular hole (FTMH) in the right eye was established. Immuno-suppressants, steroids, and azathioprine were used as treatment options, following which the state of remission was attained.

Prevalence, clinical characteristics, and independent predictors of uveitic macular edema in an Asian population: a retrospective cohort study.

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.

Late-Onset Panuveitis in a Chinese Girl with Sporadic Blau Syndrome: A Case Report.

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.

Isolated Depo-Medrol Administration under Tenon's Capsule for Post-COVID-19 Uveitis in a Child: A Case Report and Literature Review.

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.

Extranodal Rosai-Dorfman disease manifesting as Sjögren's syndrome combined with panuveitis and hypertrophic pachymeningitis: a case report and review of literature.

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 18F-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.

Vogt-Koyanagi-Harada Disease: The Clinical Spectrum and Management of Case Series in a Tertiary Eye Centre in Northern Part Of Malaysia.

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.

Long-Term Visual Outcome of Patients with Blau Syndrome.

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.

A case of panuveitis caused by caterpillar setae.

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.

Clinical Features and Course in Patients Diagnosed with Ocular Sarcoidosis without Previously Known Systemic Disease at a Tertiary Referral Center in Turkey.

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.