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Keratic Precipitates: An Overlooked Sign of Microsporidial Stromal Keratitis? 角膜沉淀物:被忽视的小孢子菌基质角膜炎征兆?
IF 2.6 4区 医学 Q2 OPHTHALMOLOGY Pub Date : 2024-11-01 Epub Date: 2024-03-21 DOI: 10.1080/09273948.2024.2329805
Ipsita Muni, Smruti Rekha Priyadarshini, Srikant Kumar Sahu, Sujata Das

Purpose: To elucidate a distinctive clinical feature in cases of microsporidial stromal keratitis (MSK).

Method: A retrospective observational study of cases with a histopathological and/or microbiological diagnosis of MSK on corneal biopsy or host corneal button between 2016 and 2022 was conducted.

Results: Eighteen cases with a confirmed histopathological and/or microbiological diagnosis of MSK were detected. Careful review of slit-lamp photographs revealed the presence of pigmented keratic precipitates (KPs) beyond the area of stromal keratitis in five out of eighteen cases (27.7%).

Conclusion: The presence of pigmented KPs beyond the area of lesion can alert the clinician to keep microsporidia as a differential cause for stromal keratitis. Management can be tailored accordingly for a better outcome.

目的:阐明小孢子菌基质性角膜炎(MSK)病例的独特临床特征:方法:对2016年至2022年间通过角膜活检或宿主角膜扣针进行组织病理学和/或微生物学诊断为MSK的病例进行回顾性观察研究:结果:发现18例经组织病理学和/或微生物学确诊为MSK的病例。仔细查看裂隙灯照片发现,18 例病例中有 5 例(27.7%)在基质角膜炎区域之外存在色素性角膜沉淀(KPs):结论:病变区域以外出现色素性角膜沉淀物可提醒临床医生将微孢子虫作为基质性角膜炎的一个鉴别病因。临床医生可据此采取相应的治疗措施,以获得更好的疗效。
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引用次数: 0
Bilateral Retinal Venous Occlusion in Atypical Hemolytic-Uremic Syndrome Due to Complement Factor H Mutation. 补体因子 H 基因突变导致非典型溶血性尿毒症综合征双侧视网膜静脉闭塞
IF 2.6 4区 医学 Q2 OPHTHALMOLOGY Pub Date : 2024-11-01 Epub Date: 2024-03-27 DOI: 10.1080/09273948.2024.2333401
Saban Gonul, Serhat Eker

Purpose: Atypical hemolytic uremic syndrome (aHUS) is a rare progressive thrombotic microangiopathy caused by overactivation in the alternative complement pathway. A wide spectrum of environmental triggers, such as viruses, vaccination, drugs, pregnancy, neoplasms, transplant, and autoimmune diseases can cause aHUS in genetically susceptible individuals. In this report, the diagnosis and treatment process of aHUS and bilateral retinal venous occlusion (RVO) will be presented.

Methods: Single-case, retrospective management of ophthalmological and systemic manifestations.

Results: A 28-year-old G2P2 female with acute blurred vision and history of acute renal failure. She was diagnosed with preeclampsia in her gestation history. After the laboratory work-up, the diagnosis of aHUS was confirmed. She was treated with eculizumab following 14 days of plasmapheresis. However, her visual acuity was 20/20 on the right and 20/60 on the left at the time of admission. Retinal examination revealed flame-shaped hemorrhages, exudation, and macular edema. The patient was diagnosed with branch RVO in the right eye. Subsequently, central RVO was occurred in the left eye. Intravitreal dexamethasone implant was administered for both eyes since there was no reasonable regression in retinal findings with bevacizumab treatment. She went into remission and her BCVA reached 20/25 during the 12-month follow-up period under the eculizumab therapy.

Conclusion: Diagnosis of aHUS is challenging especially during pregnancy and the postpartum period. Although ocular involvement is quite rare, we described bilateral RVO in aHUS case with homozygous nonsense mutation (c.2134 G > T p.G712). Dexamethasone implant should be considered for the treatment of RVO in aHUS cases.

目的:非典型溶血性尿毒症综合征(aHUS)是一种罕见的进行性血栓性微血管病,由替代补体途径过度激活引起。病毒、疫苗接种、药物、妊娠、肿瘤、移植和自身免疫性疾病等多种环境诱因均可导致遗传易感者发生非典型溶血性尿毒症。本报告将介绍 aHUS 和双侧视网膜静脉闭塞(RVO)的诊断和治疗过程:方法:对眼科和全身表现进行单病例回顾性管理:一名 28 岁的 G2P2 女性,急性视力模糊,有急性肾衰竭病史。她的妊娠史被诊断为子痫前期。经过实验室检查,确诊为 aHUS。在进行了 14 天的血浆置换后,她接受了依库珠单抗治疗。然而,入院时她的右眼视力为 20/20,左眼视力为 20/60。视网膜检查发现了火焰状出血、渗出和黄斑水肿。患者被诊断为右眼分支性 RVO。随后,左眼也出现了中心性 RVO。由于贝伐珠单抗治疗后视网膜症状没有得到合理的缓解,因此对患者双眼进行了玻璃体内地塞米松植入治疗。在接受依库珠单抗治疗的 12 个月随访期间,她的病情得到缓解,BCVA 达到 20/25:结论:诊断 aHUS 具有挑战性,尤其是在妊娠期和产后。尽管眼部受累相当罕见,但我们描述了在患有同基因无义突变(c.2134 G > T p.G712)的 aHUS 病例中出现的双侧 RVO。在治疗 aHUS 病例的 RVO 时,应考虑植入地塞米松。
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引用次数: 0
Surgical Outcomes in Uveitic Glaucoma: Long-Term Evaluation of Trabeculectomy, Non-Penetrating Deep Sclerectomy, Ex-PRESS Shunt and Ahmed Glaucoma Valve. A 3-Year Follow-Up Study. 葡萄膜炎性青光眼的手术效果:小梁切除术、非穿透性深巩膜切除术、Ex-PRESS 分流术和 Ahmed 青光眼瓣膜的长期评估。三年随访研究。
IF 2.6 4区 医学 Q2 OPHTHALMOLOGY Pub Date : 2024-11-01 Epub Date: 2024-02-21 DOI: 10.1080/09273948.2024.2315194
David Aragón-Roca, David Oliver-Gutierrez, Sandra Banderas García, Jaume Rigo, Antonio Dou, Marta Castany

Purpose: Uveitis can lead to secondary glaucoma, a condition with challenging management that can carry irreversible visual loss. Filtering surgery has demonstrated a higher failure rate, increased incidence of postoperative complications and reinterventions in uveitic patients. There is no consensus on the optimal surgical approach for uveitic glaucoma (UG) due to limited data comparing various intraocular pressure (IOP)-lowering surgeries. This retrospective cohort aims to assess the clinical outcomes of trabeculectomy (TBT), non-penetrating deep sclerectomy (NPDS), Ex-PRESS shunt and Ahmed glaucoma valve, providing additional insights into the long-term IOP control and safety of filtering surgeries in UG.

Methods: The filtering surgery was performed on 32 eyes of 27 UG patients. Complete success was defined as IOP ≤ 18 mmHg or a 30% reduction. Qualified success allowed topical hypotensive treatment.

Results: Complete success was 40.63% (13/32) at 12 months and 36.67% (11/30) at 36 months. Qualified success was 84.38% (27/32) at 12 months and 63.33% (19/30) at 36 months. In the survival analysis, both NPDS and Ex-PRESS demonstrated decreased failure rates compared to TBT (NPDS vs TBT: HR = 0.20, p = 0.049; Ex-PRESS vs TBT: HR = 0.28, p = 0.13). One or more reinterventions were required in 34.38% (11/32) of the eyes. NPDS had the lowest incidence of hypotony. Secondary cataract was a common complication in all groups.

Conclusion: Various filtering surgeries are safe and effective procedures for lowering IOP and reducing the requirement of topical antihypertensives in UG at 36 months. However, one-third of the patients will require another IOP-lowering procedure.

目的:葡萄膜炎可导致继发性青光眼,这种疾病的治疗具有挑战性,可造成不可逆的视力损失。在葡萄膜炎患者中,滤过手术的失败率较高,术后并发症和再次干预的发生率增加。由于各种降低眼压(IOP)手术的比较数据有限,目前尚未就葡萄膜炎性青光眼(UG)的最佳手术方法达成共识。这项回顾性队列研究旨在评估小梁切除术(TBT)、非穿透性深巩膜切除术(NPDS)、Ex-PRESS分流术和Ahmed青光眼瓣膜的临床疗效,为UG长期眼压控制和滤过手术的安全性提供更多见解:对 27 名 UG 患者的 32 只眼睛进行了滤过手术。完全成功的定义是眼压≤18 mmHg或降低30%。结果:手术完全成功率为 40.63%(18 mmHg):12个月时完全成功率为40.63%(13/32),36个月时完全成功率为36.67%(11/30)。12个月时合格成功率为84.38%(27/32),36个月时合格成功率为63.33%(19/30)。在生存分析中,NPDS 和 Ex-PRESS 的失败率均低于 TBT(NPDS vs TBT:HR = 0.20,p = 0.049;Ex-PRESS vs TBT:HR = 0.28,p = 0.13)。34.38%的眼睛(11/32)需要进行一次或多次再干预。NPDS 的低眼压发生率最低。继发性白内障是所有组别中常见的并发症:各种滤过手术都是安全有效的方法,可在 36 个月内降低 UG 患者的眼压,减少对局部降压药的需求。结论:各种滤过手术是降低眼压和减少 UG 患者在 36 个月内对局部降压药需求的安全而有效的方法,但三分之一的患者需要再次接受降低眼压手术。
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引用次数: 0
Periodontal Ligament Stem Cell-Derived Exosomes Regulate Muc5ac Expression in Rat Conjunctival Goblet Cells via Regulating Macrophages Toward an Anti-Inflammatory Phenotype. 牙周韧带干细胞衍生的外泌体通过调节巨噬细胞形成抗炎表型来调控大鼠结膜上皮细胞中 Muc5ac 的表达
IF 2.6 4区 医学 Q2 OPHTHALMOLOGY Pub Date : 2024-11-01 Epub Date: 2024-02-16 DOI: 10.1080/09273948.2024.2311981
Yiqian Ren, Yani Wang, Na An, Xianghua Xiao, Shiyin Pan, Bei Wang, Xianning Liu, Yao Wang

Background: Several studies have reported the protective effects of mesenchymal stem cell-derived exosomes (MSC-Exos) in reducing inflammation and decreasing conjunctival goblet cell (CGC) loss in dry eye disease. However, whether MSC-Exos provide anti-inflammatory profiles in macrophages, thus contributing to CGC protection, has remained elusive.

Methods: Macrophages were incubated with PKH26-labeled periodontal ligament mesenchymal stem cell-derived exosomes (PDLSC-Exos) for 12 h, and uptake of PDLSC-Exos by macrophages was observed by a confocal fluorescence microscope. The mRNA expression of TNF-α, IL-10, and Arg1 was detected by quantitative real-time polymerase chain reaction (qRT-PCR). The protein expression of TNF-α and IL-10 were quantified using western blotting. Then, CGCs were exposed to different macrophage supernatants and qRT-PCR was used to detect the Muc5ac mRNA expression of CGCs in response to or absence of cholinergic stimulation. ELISA was used to determine the Muc5ac secretion of CGCs in response to cholinergic stimulation.

Results: The uptake of PDLSC-Exos by M1 macrophages facilitates M2 macrophage polarization with the elevated expressions of IL-10 and Arg1. In macrophage supernatant-treated CGCs systems, PDLSC-Exo-treated M1 macrophage supernatant significantly enhanced the Muc5ac expression of CGCs in response to, or in the absence of, cholinergic stimulation, while the addition of PDLSC-Exos to the control macrophage supernatant did not generate a change in Muc5ac expression. Conversely, the addition of PDLSC-Exos to the diluted control macrophage supernatant induced a significant increase in Muc5ac expression.

Conclusion: PDLSC-Exos could protect CGCs against M1 macrophage-mediated inflammation, and the protective effects of PDLSC-Exos are partly attributable to their effects on M1 macrophages.

背景:一些研究报告称,间充质干细胞衍生的外泌体(MSC-Exos)在减少干眼症患者的炎症和结膜上皮细胞(CGC)损失方面具有保护作用。然而,间充质干细胞外泌体是否能在巨噬细胞中提供抗炎特征,从而促进CGC保护,这一点仍不明确:方法:用PKH26标记的牙周韧带间充质干细胞衍生外泌体(PDLSC-Exos)培养巨噬细胞12小时,用共聚焦荧光显微镜观察巨噬细胞对PDLSC-Exos的吸收。实时定量聚合酶链反应(qRT-PCR)检测了 TNF-α、IL-10 和 Arg1 的 mRNA 表达。TNF-α 和 IL-10 的蛋白表达采用 Western 印迹法进行定量。然后,将 CGCs 暴露于不同的巨噬细胞上清液,用 qRT-PCR 检测胆碱能刺激或无胆碱能刺激时 CGCs 的 Muc5ac mRNA 表达。用ELISA检测CGCs在胆碱能刺激下的Muc5ac分泌量:结果:M1巨噬细胞摄取PDLSC-Exos促进了M2巨噬细胞的极化,使IL-10和Arg1的表达升高。在巨噬细胞上清液处理的CGCs系统中,PDLSC-Exo处理的M1巨噬细胞上清液在胆碱能刺激或无胆碱能刺激的情况下都能显著增强CGCs的Muc5ac表达,而在对照组巨噬细胞上清液中加入PDLSC-Exos并不会改变Muc5ac的表达。相反,在稀释的对照组巨噬细胞上清液中加入 PDLSC-Exos 会导致 Muc5ac 表达量显著增加:结论:PDLSC-Exos能保护CGCs免受M1巨噬细胞介导的炎症,PDLSC-Exos的保护作用部分归因于其对M1巨噬细胞的影响。
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引用次数: 0
Anterior Segment Optical Coherence Tomography in Uveitis-Glaucoma-Hyphema Syndrome. 葡萄膜炎-青光眼-红斑综合征的前眼球光学相干断层扫描。
IF 2.6 4区 医学 Q2 OPHTHALMOLOGY Pub Date : 2024-11-01 Epub Date: 2024-03-04 DOI: 10.1080/09273948.2024.2323094
L De Simone, L Mautone, R Aldigeri, P Gentile, E Ragusa, F Gozzi, E Bolletta, C Adani, M Vecchi, A Invernizzi, L Cimino

Purpose: Uveitis-Glaucoma-Hyphema (UGH) syndrome results from contact between the intraocular lens (IOL) and the iris or ciliary body, leading to uveal structure erosion and blood-aqueous barrier breakdown. Treatment involves various drugs, with IOL removal often being necessary. Diagnosis relies on clinical signs, but imaging techniques like ultrasound biomicroscopy (UBM) or anterior segment optical coherence tomography (AS-OCT) are crucial. AS-OCT accurately depicts IOL position and potential contact, emerging as a primary alternative to UBM in the diagnosis. Our study aimed to correlate AS-OCT findings with clinically detectable iris atrophy in pseudophakic patients with IOL-iris chafing and UGH syndrome.

Methods: The study retrospectively analyzed patients diagnosed with UGH syndrome presenting at the Ocular Immunology Unit of Reggio Emilia, Italy, from January 2019 to August 2023. Patients' data were collected. Ophthalmological exams and imaging were performed. The peephole sign in AS-OCT images was evaluated. Statistical analyses were conducted, with a significance level of p ≤ 0.05.

Results: The study reviewed 22 eyes of 22 patients with UGH syndrome. Four eyes were excluded, leaving 18 patients (8 females, 10 males). Common misdiagnoses included idiopathic anterior uveitis (55.5%) and herpetic anterior uveitis (16.7%). All patients had iris transillumination defects, mostly focal (77.8%). AS-OCT revealed IOL chafing in all the eyes, with peephole sign correlation. More peephole signs occurred with IOL in the sulcus (p-value = 0.08).

Conclusion: The study recommends AS-OCT for UGH syndrome confirmation and UBM when IOL-iris chafing is not observed on AS-OCT scans.

目的:葡萄膜炎-青光眼-红斑(UGH)综合征(Uveitis-Glaucoma-Hyphema,UGH)是由于眼内晶状体(IOL)与虹膜或睫状体接触,导致葡萄膜结构侵蚀和血水屏障破坏。治疗涉及各种药物,通常需要摘除人工晶体。诊断依赖于临床症状,但超声生物显微镜(UBM)或前节光学相干断层扫描(AS-OCT)等成像技术也至关重要。AS-OCT 能准确描述 IOL 的位置和潜在接触,是 UBM 诊断的主要替代方法。我们的研究旨在将 AS-OCT 发现与假性眼球患者中可临床检测到的虹膜萎缩相关联,这些患者患有 IOL 虹膜摩擦症和 UGH 综合征:本研究回顾性分析了2019年1月至2023年8月期间在意大利雷焦艾米利亚眼部免疫科确诊为UGH综合征的患者。收集了患者数据。进行了眼科检查和成像。评估了 AS-OCT 图像中的窥孔标志。进行统计分析,显著性水平为 p≤ 0.05:研究共对 22 名 UGH 综合征患者的 22 只眼睛进行了检查。结果:研究对 22 名 UGH 综合征患者的 22 只眼睛进行了复查,其中 4 只眼睛被排除在外,剩下 18 名患者(8 名女性,10 名男性)。常见误诊包括特发性前葡萄膜炎(55.5%)和疱疹性前葡萄膜炎(16.7%)。所有患者都有虹膜透光缺陷,大部分为局灶性(77.8%)。AS-OCT 显示所有眼球都有人工晶体皴裂,并与窥孔征象相关。人工晶体位于沟内时会出现更多的窥孔征象(p 值 = 0.08):本研究建议在 AS-OCT 扫描未观察到 IOL 与虹膜摩擦时,将 AS-OCT 用于 UGH 综合征的确认和 UBM。
{"title":"Anterior Segment Optical Coherence Tomography in Uveitis-Glaucoma-Hyphema Syndrome.","authors":"L De Simone, L Mautone, R Aldigeri, P Gentile, E Ragusa, F Gozzi, E Bolletta, C Adani, M Vecchi, A Invernizzi, L Cimino","doi":"10.1080/09273948.2024.2323094","DOIUrl":"10.1080/09273948.2024.2323094","url":null,"abstract":"<p><strong>Purpose: </strong>Uveitis-Glaucoma-Hyphema (UGH) syndrome results from contact between the intraocular lens (IOL) and the iris or ciliary body, leading to uveal structure erosion and blood-aqueous barrier breakdown. Treatment involves various drugs, with IOL removal often being necessary. Diagnosis relies on clinical signs, but imaging techniques like ultrasound biomicroscopy (UBM) or anterior segment optical coherence tomography (AS-OCT) are crucial. AS-OCT accurately depicts IOL position and potential contact, emerging as a primary alternative to UBM in the diagnosis. Our study aimed to correlate AS-OCT findings with clinically detectable iris atrophy in pseudophakic patients with IOL-iris chafing and UGH syndrome.</p><p><strong>Methods: </strong>The study retrospectively analyzed patients diagnosed with UGH syndrome presenting at the Ocular Immunology Unit of Reggio Emilia, Italy, from January 2019 to August 2023. Patients' data were collected. Ophthalmological exams and imaging were performed. The peephole sign in AS-OCT images was evaluated. Statistical analyses were conducted, with a significance level of <i>p</i> ≤ 0.05.</p><p><strong>Results: </strong>The study reviewed 22 eyes of 22 patients with UGH syndrome. Four eyes were excluded, leaving 18 patients (8 females, 10 males). Common misdiagnoses included idiopathic anterior uveitis (55.5%) and herpetic anterior uveitis (16.7%). All patients had iris transillumination defects, mostly focal (77.8%). AS-OCT revealed IOL chafing in all the eyes, with peephole sign correlation. More peephole signs occurred with IOL in the sulcus (<i>p</i>-value = 0.08).</p><p><strong>Conclusion: </strong>The study recommends AS-OCT for UGH syndrome confirmation and UBM when IOL-iris chafing is not observed on AS-OCT scans.</p>","PeriodicalId":19406,"journal":{"name":"Ocular Immunology and Inflammation","volume":" ","pages":"2085-2091"},"PeriodicalIF":2.6,"publicationDate":"2024-11-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"140022325","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
Epidemiology of Orbital Inflammatory Disease: An AAO IRIS Registry Study. 眼眶炎症的流行病学:AAO IRIS 注册研究。
IF 2.6 4区 医学 Q2 OPHTHALMOLOGY Pub Date : 2024-11-01 Epub Date: 2024-03-15 DOI: 10.1080/09273948.2024.2322013
Jonathan E Lu, Connor Ross, Alexander Ivanov, Nathan Hall, Alice Lorch, Joan Miller, Michael K Yoon

Purpose: The current study queries the American Academy of Ophthalmology (AAO) Intelligent Research in Sight (IRIS) registry for data on the epidemiology, work-up, and management patterns of autoimmune orbital inflammation.

Methods: Analysis and description of patient data from the IRIS registry between 2013 and 2019 reviewing patients with autoimmune or idiopathic orbital inflammation with filters based on International Classification of Disease (ICD) and Current Procedural Terminology (CPT) codes. Patients with thyroid eye disease, orbital cellulitis, and orbital abscess were excluded.

Main outcome measures: Demographic descriptions included gender, age, geographic region, and treatment. Sub-analysis was performed by assessing rates of imaging, biopsy, lab work-up, and diagnostic categories.

Results: In a final cohort of 20,584 patients, the mean age of onset of orbital inflammation was 51.7 years; 67% female; and 63% Caucasian, 21% unknown, 12% Black, 2.6% Asian, and 1.5% other. Only 49 had imaging, 78 had laboratory work-up, and 1,411 had biopsy codes. Treatment results showed 166 patients receiving antibiotics, 224 patients receiving steroids, and 35 patients receiving both.

Conclusions: This study assessed the epidemiology, diagnostic patterns, and treatment patterns for orbital inflammation through the AAO IRIS registry. Practise patterns suggest a relatively low overall rate of imaging and laboratory studies compared to biopsies, although this certainly under-represents the actual number of imaging and laboratory studies and exemplifies the inherent imprecision of using a large database. However, the methodology of this study provides a framework of approaching the IRIS registry for oculoplastic research.

目的:本研究询问了美国眼科学会(AAO)视力智能研究(IRIS)登记处有关自身免疫性眼眶炎症的流行病学、检查和管理模式的数据:分析和描述2013年至2019年期间IRIS登记处的患者数据,回顾自身免疫性或特发性眼眶炎症患者,根据国际疾病分类(ICD)和当前程序术语(CPT)代码进行筛选。排除甲状腺眼病、眼眶蜂窝织炎和眼眶脓肿患者:人口统计学描述包括性别、年龄、地理区域和治疗方法。通过评估影像学检查、活组织检查、实验室检查和诊断类别的比例进行子分析:在最终的 20,584 例患者中,眼眶炎症的平均发病年龄为 51.7 岁;67% 为女性;63% 为白种人,21% 为不明国籍,12% 为黑人,2.6% 为亚洲人,1.5% 为其他族裔。只有 49 人进行了影像学检查,78 人进行了实验室检查,1,411 人进行了活组织检查。治疗结果显示,166 名患者接受了抗生素治疗,224 名患者接受了类固醇治疗,35 名患者同时接受了两种治疗:本研究通过AAO IRIS登记册评估了眼眶炎症的流行病学、诊断模式和治疗模式。实践模式表明,与活组织检查相比,影像学和实验室检查的总体比例相对较低,但这肯定没有充分反映影像学和实验室检查的实际数量,也体现了使用大型数据库固有的不精确性。不过,这项研究的方法为眼部整形研究提供了一个利用 IRIS 登记册的框架。
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引用次数: 0
Familial Mediterranean Fever-Associated Retinal Vasculitis: A Rare Manifestation Successfully Managed with IL-1 Pathway Inhibitors. 家族性地中海热相关视网膜血管炎:IL-1通路抑制剂成功治疗的罕见病例
IF 2.6 4区 医学 Q2 OPHTHALMOLOGY Pub Date : 2024-11-01 Epub Date: 2024-02-16 DOI: 10.1080/09273948.2024.2317978
Natalia Anglada-Masferrer, Yann Bertolani, Liliana Gutuleac, Júlia Angrill Valls, Laura Distefano, Eric Kirkegaard-Biosca

Purpose: To investigate the rare manifestation of retinal vasculitis in Familial Mediterranean fever (FMF) and its correlation with specific gene mutations, particularly the MEFV gene, with a focus on the severity of phenotypes and systemic vasculitis.

Methods: A case report of a 45-year-old Armenian patient with FMF history and dual mutations (M680I and M694V) was analyzed. Clinical assessments, including ocular examinations, were conducted at various stages of the disease. Treatment modalities, including prednisone, Anakinra, and Canakinumab, were administered and their effectiveness was assessed.

Results: The patient presented with bilateral ocular pain and decreased vision, exhibiting acute anterior uveitis, perivascular hemorrhages resembling Roth spots, and subsequent features of persistent vascular sheathing and cotton-wool spots. Dual mutations, especially M694V, were associated with a severe phenotype and systemic vasculitis. Treatment with prednisone induced remission, and IL-1 pathway inhibition with Anakinra and Canakinumab successfully managed relapses.

Conclusion: This case underscores the rarity of retinal vasculitis in FMF, particularly involving arteries, and highlights the correlation between specific gene mutations (M680I, M694V) and disease severity. The successful management with IL-1 pathway inhibitors suggests a potential therapeutic approach. Increased clinical awareness, further research, and reporting are crucial for optimizing the understanding and treatment of FMF-related ocular manifestations.

目的:研究家族性地中海热(FMF)中视网膜血管炎的罕见表现及其与特定基因突变(尤其是 MEFV 基因)的相关性,重点是表型的严重程度和全身性血管炎:方法: 分析了一例 45 岁亚美尼亚患者的病例报告,该患者有 FMF 病史和双重基因突变(M680I 和 M694V)。在疾病的不同阶段进行了临床评估,包括眼部检查。对包括泼尼松、安纳金拉和卡纳库单抗在内的治疗方法进行了治疗,并评估了其疗效:患者出现双侧眼痛和视力下降,表现为急性前葡萄膜炎、类似罗斯斑的血管周围出血,随后出现持续性血管鞘和棉絮斑。双重突变(尤其是 M694V)与严重的表型和全身性血管炎有关。使用泼尼松治疗可诱导病情缓解,使用安纳金拉和卡那单抗抑制IL-1通路可成功控制复发:本病例强调了FMF视网膜血管炎的罕见性,尤其是涉及动脉的视网膜血管炎,并突出了特定基因突变(M680I、M694V)与疾病严重程度之间的相关性。IL-1 通路抑制剂的成功治疗提示了一种潜在的治疗方法。提高临床认识、进一步研究和报告对于优化对 FMF 相关眼部表现的理解和治疗至关重要。
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引用次数: 0
Automated Machine Learning versus Expert-Designed Models in Ocular Toxoplasmosis: Detection and Lesion Localization Using Fundus Images. 眼弓形虫病中的自动机器学习与专家设计模型:使用眼底图像进行检测和病变定位
IF 2.6 4区 医学 Q2 OPHTHALMOLOGY Pub Date : 2024-11-01 Epub Date: 2024-02-27 DOI: 10.1080/09273948.2024.2319281
Daniel Milad, Fares Antaki, Allison Bernstein, Samir Touma, Renaud Duval

Purpose: Automated machine learning (AutoML) allows clinicians without coding experience to build their own deep learning (DL) models. This study assesses the performance of AutoML in detecting and localizing ocular toxoplasmosis (OT) lesions in fundus images and compares it to expert-designed models.

Methods: Ophthalmology trainees without coding experience designed AutoML models using 304 labelled fundus images. We designed a binary model to differentiate OT from normal and an object detection model to visually identify OT lesions.

Results: The AutoML model had an area under the precision-recall curve (AuPRC) of 0.945, sensitivity of 100%, specificity of 83% and accuracy of 93.5% (vs. 94%, 86% and 91% for the bespoke models). The AutoML object detection model had an AuPRC of 0.600 with a precision of 93.3% and recall of 56%. Using a diversified external validation dataset, our model correctly labeled 15 normal fundus images (100%) and 15 OT fundus images (100%), with a mean confidence score of 0.965 and 0.963, respectively.

Conclusion: AutoML models created by ophthalmologists without coding experience were comparable or better than expert-designed bespoke models trained on the same dataset. By creatively using AutoML to identify OT lesions on fundus images, our approach brings the whole spectrum of DL model design into the hands of clinicians.

目的:自动机器学习(AutoML)允许没有编码经验的临床医生建立自己的深度学习(DL)模型。本研究评估了 AutoML 在眼底图像中检测和定位眼弓形虫病(OT)病变的性能,并将其与专家设计的模型进行了比较:方法:没有编码经验的眼科受训人员使用 304 张标记的眼底图像设计了 AutoML 模型。我们设计了一个二元模型来区分 OT 和正常眼,并设计了一个对象检测模型来直观地识别 OT 病变:结果:AutoML 模型的精确度-召回曲线下面积(AuPRC)为 0.945,灵敏度为 100%,特异度为 83%,准确度为 93.5%(定制模型的准确度、特异度和准确度分别为 94%、86% 和 91%)。AutoML 物体检测模型的 AuPRC 为 0.600,精确度为 93.3%,召回率为 56%。使用多样化的外部验证数据集,我们的模型正确标注了 15 张正常眼底图像(100%)和 15 张 OT 眼底图像(100%),平均置信度分别为 0.965 和 0.963:没有编码经验的眼科医生创建的 AutoML 模型与在相同数据集上训练的专家设计的定制模型相当或更好。通过创造性地使用 AutoML 来识别眼底图像上的 OT 病变,我们的方法将 DL 模型设计的整个范围带到了临床医生的手中。
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引用次数: 0
Ocular Involvement in Systemic Sclerosis: Updated Review and New Insights on Microvascular Impairment. 系统性硬化症的眼部受累:系统性硬化症的眼部受累:微血管损伤的最新回顾和新见解。
IF 2.6 4区 医学 Q2 OPHTHALMOLOGY Pub Date : 2024-11-01 Epub Date: 2024-03-11 DOI: 10.1080/09273948.2024.2308030
Matteo Mario Carlà, Gloria Gambini, Tomaso Caporossi, Federico Giannuzzi, Francesco Boselli, Emanuele Crincoli, Matteo Ripa, Stanislao Rizzo

Systemic sclerosis (SSc) is a chronic multisystemic disease characterized by immunological activation, diffuse vasculopathy, and generalized fibrosis exhibiting a variety of symptoms. A recognized precursor of SSc is Raynaud's phenomenon, which is part of the very early disease of systemic sclerosis (VEDOSS) in combination with nailfold videocapillaroscopy (NVC) impairment. The pathophysiology of ocular involvement, alterations in internal organs, and body integumentary system involvement in SSc patients are complicated and poorly understood, with multiple mechanisms presumptively working together. The most prevalent ocular symptoms of SSc are abnormalities of the eyelids and conjunctiva as well as dry eye syndrome, due to fibroblasts' dysfunction and inflammation of the ocular surface. In particular, lagophthalmos, blepharophimosis limitation of eyelid motion, eyelid telangiectasia, and rigidity or tightening of the lids may affect up to two-third of the patients. In addition, reduction in central corneal thickness, iris defects and higher rates of glaucoma were reported. In the first reports based on retinography or fluorescein angiography, about 50% of SSc patients showed signs of vascular disease: peripheral artery occlusion, thinning of retinal pigment epithelium and choroidal capillaries, ischemic areas surrounded by intraretinal extravasation and microaneurysms, and peripheral capillary non-perfusion. Successively, thanks to the advent of optical coherence tomography angiography (OCTA), several studies highlighted significant impairment of either the choriocapillaris and retinal vascular plexuses, also correlating with NVC involvement and skin disease, even in VEDOSS disease. Given the sensitivity of this technique, ocular micro-vasculopathy may act as a tool for early SSc identification and discriminate between disease stages.

系统性硬化症(SSc)是一种慢性多系统疾病,以免疫激活、弥漫性血管病变和全身纤维化为特征,表现出多种症状。雷诺现象是公认的系统性硬化症的前兆,它是系统性硬化症极早期疾病(VEDOSS)的一部分,同时伴有甲襞视乳头镜(NVC)损伤。系统性硬化症患者的眼部受累、内脏器官的改变和全身皮肤系统受累的病理生理学非常复杂,人们对其了解甚少,推测是多种机制共同作用的结果。由于成纤维细胞功能障碍和眼表炎症,SSc 最常见的眼部症状是眼睑和结膜异常以及干眼症。其中,眼睑下垂、睑外翻、眼睑活动受限、眼睑毛细血管扩张、眼睑僵硬或紧缩等症状可能会影响多达三分之二的患者。此外,还有报道称中央角膜厚度减少、虹膜缺损和青光眼发病率升高。根据视网膜造影术或荧光素血管造影术的首批报告,约 50%的 SSc 患者出现血管病变迹象:外周动脉闭塞、视网膜色素上皮细胞和脉络膜毛细血管变薄、缺血区被视网膜内渗出物和微动脉瘤包围,以及外周毛细血管无灌注。随后,由于光学相干断层血管造影术(OCTA)的出现,一些研究强调了绒毛膜和视网膜血管丛的明显损伤,这也与 NVC 受累和皮肤疾病有关,甚至在 VEDOSS 疾病中也是如此。鉴于这种技术的敏感性,眼底微血管病变可作为早期 SSc 识别和区分疾病分期的工具。
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引用次数: 0
Uveitic Flare-Ups After Gonioscopy-Assisted Transluminal Trabeculotomy (GATT) in Patients with Uveitic Glaucoma. 葡萄膜炎性青光眼患者接受球镜辅助透镜小梁切开术 (GATT) 后的葡萄膜炎复发。
IF 2.6 4区 医学 Q2 OPHTHALMOLOGY Pub Date : 2024-11-01 Epub Date: 2024-03-12 DOI: 10.1080/09273948.2024.2316760
Raz Gepstein, Tamar Laytman Klein, Liron Naftali Ben Haim, Avner Belkin

Purpose: To assess the risk of gonioscopy-assisted transluminal trabeculotomy (GATT) inducing an uveitic flare-up in patients with uveitic glaucoma (UG).

Methods: This retrospective study included consecutive UG patients who underwent GATT at a single medical center between June 2020 and September 2022. The main outcome measure was the occurrence of a surgery-related uveitic complication defined as either an uveitic flare-up defined by the Standardization of Uveitis Nomenclature (SUN) criteria, or the appearance of cystoid macular edema (CME) from 2 weeks to 3 months after surgery.

Results: A total of 25 eyes of 22 patients were included in the study. Age ranged from 10-78 and 64% were women. The most common uveitic etiologies were juvenile idiopathic arthritis (JIA, 24%) and herpetic infection (24%). A total of 48%of the patients were on systemic immunosuppressants prior and unrelated to surgery. Eight eyes (32%) had severe glaucomatous damage prior to surgery, and 20% of the patients had undergone previous glaucoma surgery. Two cases (8%) demonstrated uveitic flare-up in the early postoperative period: a case of mild anterior chamber reaction and a case of CME with persistent edema prior to surgery. Average intraocular pressure (IOP) was reduced from 26.7 mm Hg on four medications to 12.2 on 1.1 after 1 year. One patient required reoperation for IOP control.

Conclusions: With careful pre and postoperative care, GATT seems to be a low-risk procedure for uveitic flare-ups in patients with UG.

目的:评估葡萄膜炎性青光眼(UG)患者接受球镜辅助腔内小梁切开术(GATT)诱发葡萄膜炎复发的风险:这项回顾性研究纳入了2020年6月至2022年9月期间在一家医疗中心接受GATT手术的连续UG患者。主要结果指标是手术相关葡萄膜并发症的发生情况,即根据葡萄膜炎命名标准化(SUN)标准定义的葡萄膜炎发作或术后2周至3个月出现囊样黄斑水肿(CME):研究共纳入了 22 名患者的 25 只眼睛。年龄在 10-78 岁之间,64% 为女性。最常见的葡萄膜炎病因是幼年特发性关节炎(JIA,24%)和疱疹病毒感染(24%)。48%的患者在手术前服用过全身免疫抑制剂,但与手术无关。八只眼睛(32%)在手术前有严重的青光眼损伤,20%的患者曾接受过青光眼手术。两例(8%)患者在术后早期出现葡萄膜炎复发:一例是轻度前房反应,一例是手术前持续水肿的CME。平均眼压(IOP)从使用四种药物时的 26.7 mm Hg 降至一年后的 12.2 mm Hg,使用 1.1 种药物时的 12.2 mm Hg。一名患者需要再次手术才能控制眼压:结论:通过术前和术后的精心护理,GATT 似乎是 UG 患者葡萄膜炎复发的低风险手术。
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引用次数: 0
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Ocular Immunology and Inflammation
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