Ocular Immunology and Inflammation最新文献

Purpose: Case report.

Methods: Melanoma-associated retinopathy (MAR) is a rareparaneoplastic autoimmune disorder characterized by retinal dysfunction inpatients with cutaneous melanoma. While typically presenting with photopsias, nyctalopia, and peripheral vision loss, diagnosis remains challenging due tooften normal fundoscopic findings and variable autoantibody detection. Thisreport describes a unique case of recurrent MAR managed successfully withintravitreal dexamethasone over a decade-long follow-up.

Results: A 48-year-old man with stage IV cutaneousmelanoma with BRAF V600E mutation achieved complete systemic remission with vemurafenib. During ophthalmological screening, he reported nyctalopia andphotopsias; clinical examination and optical coherence tomography wereunremarkable. Full-field electroretinography (ERG) revealed an electronegativewaveform (reduced b-wave), confirming MAR despite negative antiretinalantibodies. Sub-Tenon's triamcinolone and intravenous immunoglobulin failed toimprove symptoms, but bilateral intravitreal dexamethasone implants (Ozurdex) resolved visual disturbances and normalized ERG. Over ten years, eight MAR recurrences occurred - each treated successfully with Ozurdex - without melanoma relapse.

Conclusion: MAR can be presented as the first sign of melanoma relapse. This case underscores MAR's potential for recurrence despite durable melanoma remission and highlights intravitreal dexamethasone as an effective long-term therapy. The absence of autoantibodies and late flares challenges current diagnostic paradigms, emphasizing ERG's critical role. Proactive ophthalmologic surveillance and individualized local therapy canpreserve vision in this underrecognized condition.

Purpose: To investigate trends in the diagnostic approach to nonparaneoplastic autoimmune retinopathy (npAIR).

Methods: We queried PubMed for clinical reports on npAIR published between January 2016 and September 2025. Articles were assessed to determine criteria used to establish diagnosis of npAIR using a standardized grading system. Articles were categorized as case reports (≤3 patients) or case series (>3 patients).

Results: 36 case reports and 41 case series met eligibility criteria (755 total cases). Author subspecialty included 34% uveitis, 20% inherited retinal disease (IRD), 16% general retina, 10% miscellaneous, and 19% unknown specialty. Over 80% of publications reported electroretinography and anti-retinal antibody testing for diagnosis of npAIR. Fundus autofluorescence (FAF) was performed in 67% of case reports and at least one patient in 51% of case series. Widefield FAF was used in 19% of case reports and in at least one patient in 20% of case series. Genetic testing was reported in 22% of case reports and in at least one patient in 27% of case series. Studies with an IRD specialist as first or last author most commonly used genetic testing (35%).

Conclusions: Literature on npAIR is hampered by variability in classification schemes and incomplete reporting. Nonspecific electroretinography testing and antiretinal antibody testing are widely employed while widefield autofluorescence testing and genetic testing are not commonly used. Expanded access to these tools provides an opportunity to update diagnostic criteria of npAIR. Improved classification will permit us to better understand the natural history of disease.

Paediatric neoplastic uveitis masquerade syndrome (UMS) is a rare but serious condition in which intraocular malignancies mimic inflammatory uveitis. Children may present with symptoms of intraocular inflammation such as redness, pain, light sensitivity, and blurred vision. The diagnostic challenge is compounded by the difficulty of examining younger patients and the absence of systemic warning signs. Accurate diagnosis is essential because the therapies for uveitis and ocular neoplasms vary fundamentally, and delayed recognition can be life-threatening.Although the most frequent neoplastic masquerades are acute leukaemia and retinoblastoma (RB), medulloepithelioma and metastatic tumours may also occur. Acute lymphoblastic leukaemia, the most common childhood malignancy, can manifest as anterior pseudohypopyon, iris infiltration or neovascularization, or posterior segment findings, including haemorrhages, Roth spots, and serous retinal detachment. Ocular involvement may indicate relapse. RB, the most common primary intraocular cancer in children, usually presents with leukocoria or strabismus. However, its diffuse infiltrating variant can masquerade as intraocular inflammation with pseudohypopyon, iris neovascularization, vitreous seeds, and vitreous haemorrhage. Unlike classic RB, this variant often lacks a discrete calcified mass, further complicating the diagnosis, which frequently results in misdiagnosis of uveitis.Multimodal ophthalmic imaging, including enhanced depth optical coherence tomography, fluorescein angiography, and ultrasonography, may aid in the diagnosis. Computed tomography or magnetic resonance imaging, combined with a comprehensive history and laboratory evaluation, can help distinguish neoplastic masquerades from inflammatory diseases. Early recognition, prompt referral to an oncology or ocular oncology department, and multidisciplinary care are critical because a timely diagnosis can preserve both vision and life.

Purpose: Intermediate uveitis (IU) can occur secondary to systemic autoinflammatory disorders, such as juvenile idiopathic arthritis and multiple sclerosis. In contrast, pars planitis (PP) specifically refers to an idiopathic form of IU, characterized by the absence of any identifiable underlying systemic condition. The frequency of the association between PP and CNS demyelination is unknown in children.

Methods: A retrospective analysis of paediatric PP patients' clinical and brain magnetic resonance imaging (MRI) data was conducted to investigate the coexistence of PP and CNS demyelinating pathologies in children.

Results: The cohort comprised 65 paediatric patients with PP, mean age 13.25 ± 3.1, (range 6-18, median 13) years, 41.5% female, who had at least one brain MRI. The mean follow-up was 4.02 ± 2.76 (range 0.5-11, median 4) years. Demyelinating lesions on MRI were visible in 5/65 (7.7%) patients. None reported neurological symptoms nor had abnormal findings on neurological examination. Three patients who had been undergoing adalimumab (ADA) treatment for a period of between three months and four years had their ADA therapy stopped when a demyelinating lesion was observed on MRI. Additionally, mycophenolate mofetil or methotrexate treatment was maintained as a maintenance therapy. The other two patients had not received any immunomodulatory treatment when demyelinating lesions were identified, and MRI findings were taken into consideration when treatment was planned.

Conclusions: Demyelinating lesions of the CNS can be detected in paediatric PP patients at a rate similar to adults. Clinicians should be aware of the presence of silent demyelination in PP and plan the anti-inflammatory treatment accordingly.

Purpose: Non-infectious uveitis (NIU) is a major cause of visual loss among young adults, and the available therapies are limited. Adalimumab (ADA), an antibody targeting tumour necrosis factor α (TNF-α), is an effective treatment. This review aims to examine immunogenicity of ADA, and its association with serum ADA trough levels (SATL) and the risk of treatment failure.

Methods: A systematic review of the literature was conducted, following PRISMA guidelines. Studies published between 2019 and 2023 were included. After applying the inclusion criteria, 10 articles were selected. The risk of bias was evaluated using the most appropriate method for each type of study.

Results: In total 10 studies were finally included. Most of the investigation reported the formation of anti-ADA antibodies (AAA), which was associated with low SATL and poor treatment response. Some studies also distinguished between transient and permanent AAA, with transient AAA linked to a higher risk of treatment failure. Risk factors for AAA development were explored, with many studies highlighting the benefits of combined therapy with ADA and other immunosuppressants compared with ADA monotherapy.

Conclusion: The association between low SATL, AAA, and poor response to ADA treatment is well established. However, further high-quality investigations are needed to strengthen the evidence in this area. Therapeutic monitoring strategies appear to be valuable tools for providing personalized management for patients with NIU.

Aim: To evaluate the safety and preliminary efficacy of low-dose subconjunctival adalimumab in patients with non-infectious uveitis (NIU), refractory to conventional immunomodulatory therapy (IMT).

Methods: Prospective, noncomparative, interventional case series. Five patients with bilateral panuveitis (four post-therapeutic vitrectomy) and persistent intraocular inflammation on conventional IMT for > 2 years received three doses of subconjunctival adalimumab 5 mg/0.1 mL at two-week intervals and followed up for 24 weeks.

Results: No cases of persistent conjunctival congestion, corneal erosions or infection, or intraocular pressure > 21 mmHg were noted. Improvement in inflammatory scores and best-corrected visual acuity (BCVA) were noted in three patients each and worsening in none. None of the three patients who showed initial improvement in visual acuity had any baseline cystoid macular edema or vitreous haze to account for the improvement in BCVA.

Conclusion: Subconjunctival administration of 5 mg adalimumab is a safe and effective therapy for NIU refractory to conventional IMT.

Purpose: To assess the efficacy and safety of weekly adalimumab after dose escalation in patients with chronic refractory non-infectious uveitis.

Methods: A retrospective analysis was conducted on patients aged 18 years and older with persistent chronic uveitis despite bi-weekly adalimumab treatment. Data collected included uveitis diagnosis, anatomical site of involvement, history of immunosuppressive treatments, relapse frequency, and other relevant clinical parameters.

Results: This study included 30 patients (18 females and 12 males) with a median age of 33.5 (22-57) years and a mean follow-up period of 62.6 ± 25.2 months. Median visual acuity was 0.29 logMAR (0.22-0.40) with bi-weekly adalimumab treatment and improved to 0.20 logMAR (0.10-0.36) with weekly adalimumab treatment (p = 0.009). A significant reduction in central macular thickness was observed with weekly adalimumab treatment compared to the bi-weekly regimen (259.67 µm vs. 336.47 µm, p = 0.001). The frequency of relapses was 1.9 ± 0.66 with bi-weekly treatment and 0.53 ± 0.33 with weekly treatment (p = 0.001). The proportion of patients exhibiting active ocular inflammation requiring systemic steroids was 60% in the bi-weekly treatment, compared to 24.3% in the weekly treatment. In terms of adverse effect profile, both treatment modalities exhibited similar characteristics. Weekly adalimumab is associated with a significantly reduced risk of relapse compared with bi-weekly ADA (HR = 0.267, p = 0.001).

Conclusion: Weekly adalimumab treatment is a viable option for managing inflammation in refractory uveitis, providing enhanced efficacy to the standard dose in terms of visual and anatomical outcomes, while maintaining a comparable side effect profile.

Purpose: This study aimed to evaluate the efficacy of methotrexate (MTX) treatment in pediatric non-infectious uveitis (NIU) cases.

Methods: Patients diagnosed with pediatric NIU and initiated on subcutaneous MTX at a dose of 10 mg/m²/week between 2023 and 2025 were included in the study. The patients' age, age at uveitis diagnosis, anatomical localization and etiology of uveitis, baseline and final best-corrected visual acuity (BCVA), and anterior and posterior segment complications detected at the initial visit were recorded.

Results: A total of 127 eyes from 64 patients were included in the study. Of these patients, 39 were female, and 25 were male. The mean age was 9.89 ± 3.56 years (3-17 years). At the initial visit, at least one ocular complication in at least one eye was detected in 49 patients. Remission was achieved in 23 cases with MTX treatment, whereas 41 patients did not achieve remission. Among the 49 patients with at least one ocular complication, remission was achieved in 13 with MTX treatment. In contrast, among the 15 patients without any complications, remission was achieved in 10 (p = 0.005). The risk of non-responsiveness to MTX treatment was found to be 10.7 times higher in patients with at least one ocular complication at diagnosis.

Conclusion: MTX is an effective and safe treatment for pediatric NIU. However, in a significant proportion of patients, particularly those with ocular complications, MTX alone may be insufficient, necessitating the addition of other immunosuppressive agents.

Purpose: To investigate the clinical characteristics, risk factors, and prognostic factors of cytomegalovirus retinitis (CMVR) after hematopoietic stem cell transplantation (HSCT).

Methods: A retrospective cohort study was conducted involving 473 HSCT patients (193 allogeneic, 280 autologous) from 2019 to 2023.

Results: In this cohort, 12 patients (21 eyes) developed CMVR. The cumulative incidence was 2.54%. CMVR was bilateral in 75% of cases, with a mean onset of 136.4 ± 59.1 days post-transplantation. CMV DNA was detected in 88.9% (16/18) of aqueous humor samples. Retinal detachment was the most common complication, affecting 52.4% of eyes. All patients had three or more risk factors. Median visual acuity declined from 20/80 (range, hand motion-20/20) initially to 20/125 (range, no light perception-20/25) at final follow-up. CMVR was the first symptom of CMV infection in 16.7% of cases. The recurrence rate was 25.00% (3/12), with recurrent cases linked to EBV viremia episodes. Correlation analysis revealed that CMVR type and initial BCVA were significantly associated with RD (p < 0.05).

Conclusions: These findings highlight the critical importance of the first year post-HSCT for CMVR detection. Increased ophthalmic screening is advised for patients with multiple risk factors and hemorrhagic types to improve outcomes.

Purpose: To report a case of Ocular Whipple's Disease (WD) that presented as chronic bilateral uveitis and to highlight the essential role of molecular diagnostics in reaching a definitive diagnosis.

Methods: This report describes the case of a 67-year-old female with chronic, refractory uveitis. The patient underwent a diagnostic vitrectomy. The collected vitreous sample was analyzed using histopathology with Periodic Acid-Schiff (PAS) staining, transmission electron microscopy (EM), and polymerase chain reaction (PCR) for Tropheryma whipplei.

Results: Histopathology of the vitreous revealed bubbly histiocytes containing PAS-positive granules, and EM confirmed the presence of Tropheryma whipplei bacteria. PCR analysis of fresh vitreous fluid yielded a positive result for T. whipplei 16S rRNA. In contrast, systemic evaluation, including a duodenl biopsy, was negative for WD. Following treatment with appropriate antibiotics, the patient's ocular inflammation resolved, and visual acuity improved significantly.

Conclusion: Ocular WD should be considered in the differential diagnosis for chronic, unexplained uveitis, even when systemic symptoms are absent. PCR analysis of vitreous fluid is an invaluable tool for confirming the diagnosis and is crucial for guiding appropriate, sight-saving therapy.