Ocular Immunology and Inflammation最新文献

Purpose: To assess appeal outcomes and factors influencing those outcomes in insurance-mandated non-medical switching (NMS) from adalimumab (Humira®) to biosimilars in patients with uveitis and ocular inflammatory diseases.

Methods: This retrospective observational study reviewed the electronic medical records of patients receiving Humira® for non-infectious uveitis and ocular inflammatory diseases prior to pharmacy benefit manager (PBM) formulary changes effective April 1, 2024, at the University of Texas Southwestern Medical Center (UTSW) and University of Texas Health Austin (UTHA). Data collected included PBM assignment, treatment duration, remission status, incidence of NMS mandates, appeal outcomes, and final treatment status. Appeals at UTSW cited Texas Senate Bill 680, which allows exemptions from new step-therapy protocols. Logistic regression assessed associations between patient characteristics, PBM, and NMS outcomes.

Results: Of the 75 patients, 30 (40%) received NMS mandates. CVS Caremark patients were more likely to receive mandates for NMS (86.4% vs 20.8%; p < 0.01) and had a lower appeal success rate (14.3% vs 90.9%; p < 0.01) compared to non-CVS Caremark patients. Ultimately, 18 (24%) patients (94% CVS Caremark patients, p < 0.01) were forcibly switched. Median appeal time was 11 days (range 1-35), and processing biosimilar transitions added considerable administrative burden, even in the absence of appeals.

Conclusion: PBM-driven formulary changes disrupted treatment continuity for patients with non-infectious ocular inflammation. Enforcement of NMS mandates varied by PBM and often disregarded clinical stability and physician input, underscoring the urgent need for policy reform and uveitis-specific biosimilar outcome data.

Purpose: To present two rare pediatric cases of bilateral acute occlusive retinal vasculitis (ORV) associated with respiratory viral infections.

Methods: This retrospective review of two cases included a comprehensive clinical evaluation and detailed examinations, including wide-field RetCam color fundus photography, fundus fluorescein angiography, B-ultrasound imaging, aqueous humor cytokine analysis, and laboratory testing for viral infections. Treatments consisted of antivirals, corticosteroids, and anti-VEGF therapy.

Results: Both patients presented with severe vision loss, retinal hemorrhages, retinal vascular occlusion, and exudative detachment. Elevated intraocular VEGF and IL-6 suggested an immune-mediated response. Despite treatment, both had poor visual recovery with optic atrophy and retinal vessel attenuation.

Conclusion: Respiratory viral infections may trigger severe pediatric ORV via immune-mediated inflammation. In these two cases, early diagnosis, viral screening, and prompt treatment were applied, but the prognosis remains poor. Further research is needed to refine management strategies.

Purpose: To describe a rare case of pediatric granulomatosis with polyangiitis (GPA) initially presenting as peripheral ulcerative keratitis (PUK), followed by orbital inflammation, highlighting the diagnostic challenges and importance of early recognition.

Methods: A 12-year-old boy with a prior history of allergic conjunctivitis presented with unilateral peripheral corneal opacities. The condition was initially misdiagnosed and treated as infectious keratitis and allergic conjunctivitis without improvement. Comprehensive clinical, serologic, and imaging evaluations were performed, including ANCA testing, chest and orbital CT scans, and infectious serologies.

Results: Laboratory workup revealed elevated inflammatory markers, positive p-ANCA, and high anti-myeloperoxidase (MPO) antibody titers. Chest CT showed pulmonary nodules consistent with vasculitic involvement. The patient subsequently developed orbital pseudotumor and recurrent epistaxis, consolidating the diagnosis of GPA despite MPO-ANCA positivity, which is more commonly linked to microscopic polyangiitis. Immunosuppressive therapy with systemic corticosteroids and methotrexate led to complete remission of ocular inflammation, resolution of orbital involvement, and preservation of 20/20 visual acuity bilaterally after one year of follow-up.

Conclusion: This case represents an exceptional pediatric presentation of GPA, with PUK as the sole initial manifestation preceding systemic disease. It underscores the need for heightened clinical suspicion in children with atypical peripheral keratitis unresponsive to standard therapy. Early recognition and multidisciplinary management are crucial to prevent irreversible visual loss and systemic complications.

Purpose: To report the first documented case of endophthalmitis caused by Letendraea helminthicola following a penetrating globe injury from a cat scratch.

Methods: A 32-year-old male presented with post-traumatic endophthalmitis after a penetrating ocular injury caused by a cat scratch. Clinical evaluation, serial ophthalmic imaging, microbiological investigations, and polymerase chain reaction (PCR) analysis of conjunctival tissue were performed.

Results: Despite initial improvement with empiric broad-spectrum antibiotics, the patient developed progressive vitritis requiring multiple pars plana vitrectomies. Conventional microbiological cultures remained negative; however, PCR identified L. helminthicola from conjunctival tissue. Targeted antifungal therapy with systemic and intravitreal voriconazole led to infection control, although visual recovery was limited due to macular involvement.

Conclusion: L. helminthicola can cause post-traumatic fungal endophthalmitis following ocular injuries involving animal scratches. Molecular diagnostics are critical for identifying rare pathogens, and prompt targeted antifungal therapy is essential for optimizing outcomes.

Background: Sjögren's syndrome (SS) is a chronic autoimmune disorder primarily affecting exocrine glands, although it may also involve multiple organs, including the eye. The choroid, the most vascularized ocular structure, is affected by systemic diseases. The choroidal vascularity index (CVI), a parameter that reflects vascular damage, decreases as vascular injury increases.

Method: A total of 40 SS patients, aged 18-75 years, and 40 healthy controls were enrolled. Disease activity was assessed using the Sjögren's Syndrome Disease Activity Index (SSDAI); patients with an SSDAI score ≥5 were classified as having active disease, while those with scores <5 were considered in remission. Enhanced depth imaging optical coherence tomography (EDI-OCT) images were analyzed using ImageJ software to measure the luminal area (LA) and total choroidal area (TCA), with CVI calculated as the ratio LA/TCA.

Results: SS patients exhibited significantly lower CVI values (mean: 0.62 ± 0.04) compared to controls (mean: 0.69 ± 0.02; p < 0.001). No significant correlation was found between CVI and SS disease activity as measured by the SSDAI. Additionally, CVI did not significantly differ with respect to gender, age, disease duration, anti-SS-A, anti-SS-B, or rheumatoid factor (RF) positivity. However, CVI was significantly lower in patients with positive antinuclear antibodies. In multivariable analysis, ANA positivity and CRP ≥5 mg/L were independently associated with lower CVI, whereas ESR showed no association with CVI.

Conclusion: Our findings indicate that SS patients exhibit reduced CVI, reflecting ocular vascular involvement, which was not associated with disease activity. As a non-invasive method, CVI measurement may serve as a useful tool for assessing microvascular damage induced by inflammatory processes.

Purpose: To demonstrate the efficacy and safety of intravenous golimumab infusion in treating juvenile idiopathic arthritis-associated anterior uveitis.

Methods: This study was a retrospective observation case series. Electronic records of patients diagnosed with juvenile idiopathic arthritis-associated anterior uveitis who received intravenous golimumab infusion were examined.

Results: A total of 24 eyes of 13 patients were included in this study. During 12 months before starting intravenous golimumab, the median grade of anterior chamber reaction was 1 (range: 0.5-3), and the median number of flare-ups was 1 (1-3). During 12 months following the start of intravenous golimumab, the median grade of anterior chamber reaction was 0 (range: 0-1), and the median number of flare-ups was 0 (range: 0-1). Before starting intravenous golimumab, the average number of immunomodulatory agents was 2.6 ± 1.0 with a range of 2 to 5. The average age of patients at the time of starting intravenous golimumab was 13.69 ± 5.23 years (range between 5 and 22). A total of 11 (84.6%) patients responded to intravenous golimumab. The medication was discontinued in one patient due to ineffectiveness and in another patient due to the development of psoriasis as an adverse effect. Cystoid macular edema was present in six eyes of three patients which resolved in all six eyes after starting intravenous golimumab.

Conclusion: Intravenous golimumab proves to be efficacious and safe for inducing and sustaining remission in JIA and JIA-associated uveitis. Nonetheless, further robust studies with larger sample sizes are needed to substantiate our findings.

Background: Retinal vasculitis (RV) is a complex inflammatory condition that affects the retinal vessels, often presenting as perivascular sheathing, vascular leakage, and occlusion. Despite its well-documented clinical and angiographic features, the definition and classification of RV remain inconsistent across clinical and research settings. This lack of uniformity has led to challenges in diagnosis, management, and understanding of research findings. Thus, this scoping review aims to objectively identify any ambiguity in the terminology of RV and discuss possible reasons for it, laying the groundwork for a subsequent panel of experts to provide consensus definitions for RV through a Delphi process.

Methods: This review included 97 studies. The authors extracted and summarised data on the definitions and diagnostic criteria used for RV, the definitions used for subtypes of RV (e.g. isolated, idiopathic, undifferentiated, primary RV), the imaging modalities used for RV diagnosis, and any references to existing guidelines.

Results: The results confirmed a significant degree of ambiguity in RV terminology. Among the 17 papers that explicitly defined RV in their methodology, four distinct definitions emerged. We also found that the terms' idiopathic,' 'primary,' and 'undifferentiated' RV were often used interchangeably, despite reflecting distinct aspects of the disease. Notably, all 52 studies that noted the diagnostic modalities used included fluorescein angiography, although the usage of other modalities was contentious.

Conclusion: There are significant inconsistencies and ambiguities in defining and classifying RV, highlighting the need for a standardized, consensus-based framework to improve clarity and consistency in both research and clinical practice.

Purpose: Peripheral ulcerative keratitis (PUK) is a rare, vision-threatening disorder that typically occurs in adults with underlying autoimmune disease or infection. Few cases of pediatric PUK have been reported, making clinical guidelines rare.

Methods: Case report.

Results: A 13-year-old female with Trisomy 21 and well-controlled Graves' Disease and no history of ocular trauma presented with ten days of right eye pain refractory to treatment with topical antibiotics and corticosteroids. Visual acuity was count fingers and slit lamp examination showed marked conjunctival injection, as well as a fibrin band in the anterior chamber. Corneal examination demonstrated substantial inferior peripheral stromal thinning and overlying epithelial defect concerning for PUK, prompting hospital admission for management and systemic disease workup. Effusive asymmetric arthritis of the left lower ankle, left knee, and left hand was suspicious for juvenile idiopathic arthritis (JIA) and laboratory studies showed elevated CRP and ESR levels. Despite medical treatment with IV methylprednisolone 1000 mg daily and fortified antibiotic eye drops four times per day, a corneal descemetocele formed, prompting urgent surgical intervention. The patient underwent two interventions with amniotic membrane transplantation. Four months after initial presentation, visual acuity in the affected eye had improved to 20/25, same as fellow eye, and slit lamp examination showed corneal re-epithelialization, healed descemetocele, and ocular inflammatory control on methotrexate monotherapy. The final ocular diagnosis was PUK and anterior uveitis secondary to JIA.

Conclusion: Based on our clinical course, we recommend early systemic treatment to manage rapidly progressive corneal thinning in pediatric PUK. Early surgical intervention can be considered to avoid perforation.

Purpose: Fusarium is an uncommon cause of fungal endogenous endophthalmitis (FEE), especially in immunocompromised patients. So far, only two species of Fusarium (Fusarium solani and dimerium) have been associated with FEE. To the best of our knowledge, Fusarium oxysporum has not been reported as a cause of FEE previously. We describe a case of FEE due to Fusarium oxysporum without associated disseminated fusariosis in a patient of hematopoietic stem cell transplantation (HCT).

Methods: A young male patient presented with a sudden diminution of vision in the left eye (LE). Ocular examination of the LE revealed a best-corrected visual acuity of 20/120, 2+ cells, mild vitritis, and superficial necrotizing retinitis-like lesions at the posterior pole. The right eye was unremarkable. Systemic history revealed a recent history of HCT for aplastic anemia. He was on systemic immunosuppressants for prophylaxis of graft versus host disease.

Results: An intravitreal ganciclovir was injected initially, suspecting LE CMV retinitis. However, the vitreous tap microbiological examination and PCR were negative. Detailed laboratory evaluations were unremarkable. As the LE condition continued to worsen, he underwent pars plana vitrectomy and intravitreal antibiotics and antifungals in LE. Vitreous biopsy culture revealed Fusarium oxysporum. He was aggressively treated with topical, repeat intravitreal, and systemic anti-fungal medications. Despite multifaceted treatment, the LE progressed to total retinal detachment without any salvageable vision.

Conclusion: This report illustrates the first case of Fusarium oxysporum-associated FEE in an HCT patient and highlights the diagnostic challenges, need for early aggressive treatment, and poor outcome in such cases.