masquerade syndrome

化妆舞会综合征
  • 文章类型: Journal Article
    目的:报告并描述与原发性中枢神经系统淋巴瘤(PCNSL)相关的无症状玻璃体视网膜淋巴瘤(VRL)的眼部特征,通过检查临床和多模式影像学特征并与症状性VRL进行比较。
    方法:回顾性横断面研究。
    方法:纳入细胞学或分子学证实的VRL患者。患者分为三组:原发性VRL(PVRL),与PCNSL(PCNSL-S)相关的症状性VRL,或与PCNSL(PCNSL-AS)相关的无症状VRL。涵盖人口统计的数据,视觉症状,视敏度(VA),并收集影像学特征。使用单向ANOVA和多项线性回归分析对各组之间的定量和分类变量进行了横截面分析。
    结果:该研究包括56例VRL患者的104只眼。29例患者(52%)被诊断为PVRL,27例患者(48%)被诊断为与PCNSL相关的VRL。其中,17例(63%)报告了视觉症状(PCNSL-S),10例(37%)无症状(PCNSL-AS)。PCNSL-AS患者的VA优于PVRL患者(0.11vs.0.76LogMAR,p=0.04)和不同的临床特征,眼前节受累率较低(比值比[OR]=0.02;95%置信区间[CI]0.12-0.84;p<0.01)和玻璃体炎(OR=0.32;95CI0.11-0.91;p=0.03)。与PVRL(OR=0.14;95CI0.02-1.11;p=0.06)和PCNSL-S(OR:0.08;95CI0.01-0.69p=0.05)相比,PCNSL-AS病例的视网膜下浸润较少,并且与较差的VA相关(估计值=0.55LogMAR;95CI0.29-0.8;p<0.01)。
    结论:本研究描述了与PCNSL相关的无症状VRL的独特临床和影像学特征,特点是更好的VA和较少严重的眼部受累。这些发现强调了多模态成像在促进VRL在PCNSL分期中的早期检测中的关键作用。未来的PCNSL管理指南应考虑诊断无症状VRL患者的必要性。
    OBJECTIVE: To report and characterize ocular features of asymptomatic vitreoretinal lymphoma (VRL) associated with primary central nervous system lymphoma (PCNSL), by examining clinical and multimodal imaging characteristics and comparing with symptomatic VRL.
    METHODS: Retrospective cross-sectional study.
    METHODS: Patients with cytologically or molecularly confirmed VRL were included. Patients were classified into three groups: primary VRL (PVRL), symptomatic VRL associated with PCNSL (PCNSL-S), or asymptomatic VRL associated with PCNSL (PCNSL-AS). Data encompassing demographics, visual symptoms, visual acuity (VA), and imaging characteristics were collected. Cross-sectional analyses of quantitative and categorical variables among groups were performed with one-way ANOVA and multinomial linear regression analyses.
    RESULTS: The study included 104 eyes from 56 patients with VRL. Twenty-nine patients (52%) were diagnosed with PVRL, and 27 patients (48%) were diagnosed with VRL associated with PCNSL. Among these, 17 (63%) reported visual symptoms (PCNSL-S), whereas 10 (37%) were asymptomatic (PCNSL-AS). PCNSL-AS patients exhibited better VA than PVRL patients (0.11 vs 0.76 LogMAR, P = 0.04) and distinct clinical features, with lower rates of anterior segment involvement (odds ratio [OR] = 0.02; 95% confidence interval [CI] 0.12-0.84; P < .01) and vitritis (OR = 0.32; 95% CI 0.11-0.91; P = .03). Subretinal infiltration was less common in PCNSL-AS cases compared to PVRL (OR = 0.14; 95% CI 0.02-1.11; P = 0.06) and PCNSL-S (OR: 0.08; 95% CI 0.01-0.69 P = 0.05) and was associated with worse VA (estimate = 0.55 LogMAR; 95% CI 0.29-0.8; P < .01).
    CONCLUSIONS: This study describes distinctive clinical and imaging features of asymptomatic VRL associated with PCNSL, characterized by better VA and less severe ocular involvement. The findings highlight the pivotal role of multimodal imaging in facilitating early detection of VRL in the staging of PCNSL. Future guidelines for PCNSL management should consider the necessity of diagnosing patients with asymptomatic VRL.
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  • 文章类型: Case Reports
    在并发肺肉瘤样反应和肺腺癌的背景下,将眼部肉瘤样反应描述为副肿瘤综合征的独特表现。
    单例报告和叙述性回顾。
    一名59岁的男性患者,有一年的视力下降和体重减轻的病史。临床检查显示全葡萄膜炎和多发性脉络膜视网膜病变。胸部CT扫描显示纵隔和肺门淋巴结肿大以及恶性的右下肺结节。随后的支气管镜检查和活检证实前哨淋巴结中有肺腺癌和非干酪性肉芽肿。
    尽管肺肉芽肿反应可以在肺部恶性肿瘤中看到,眼部类肉瘤样反应可能表现为系统性恶性肿瘤的副肿瘤表现,伴随的肺和眼部类肉瘤样反应的存在区分了这种情况。这些发现强调了对有体质症状的患者进行系统性检查的重要性,因为副肿瘤综合征和转移性疾病可能模拟葡萄膜炎。识别副肿瘤结节病作为潜在的临床表现是至关重要的,尤其是有慢性病指标的患者,需要对恶性肿瘤进行全面评估。
    UNASSIGNED: To describe ocular sarcoid-like reaction as a unique manifestation of paraneoplastic syndrome in the context of concurrent pulmonary sarcoid-like reaction and lung adenocarcinoma.
    UNASSIGNED: Single case report and narrative review.
    UNASSIGNED: A 59-year-old male patient presented with a year-long history of diminished vision and weight loss. Clinical examination revealed panuveitis and multiple chorioretinal lesions. A CT scan of the chest revealed mediastinal and hilar lymphadenopathy as well as a spiculated right lower lung nodule concerning for malignancy. Subsequent bronchoscopy and biopsy confirmed lung adenocarcinoma and non-caseating granulomas in sentinel lymph nodes.
    UNASSIGNED: Although pulmonary granulomatous reaction can be seen in the setting of lung malignancy, and ocular sarcoid-like reaction may present as a paraneoplastic manifestation of systemic malignancy, the presence of concomitant pulmonary and ocular sarcoid-like reactions distinguishes this case. The findings underscore the importance of a systemic workup for patients with concerning constitutional symptoms, as paraneoplastic syndromes and metastatic diseases may mimic uveitis. Recognition of paraneoplastic sarcoidosis as a potential clinical manifestation is essential, especially in patients with chronic illness indicators, necessitating a comprehensive evaluation for malignancy.
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  • 文章类型: Case Reports
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  • 文章类型: Journal Article
    葡萄膜炎伪装综合征是一组不同的临床实体,由于存在炎症体征而模仿常规免疫介导的葡萄膜炎,但对抗炎治疗具有抗性。误诊会阻碍这些疾病的适当治疗,并可能导致不良结局。这篇综述讨论了常见的肿瘤和非肿瘤疾病过程,这些过程伪装成眼内炎症,重点是相关的临床特征和辅助研究,有助于达到正确的诊断。
    Uveitis masquerade syndromes are a diverse group of clinical entities which mimic conventional immune-mediated uveitis due to the presence of inflammatory signs but are resistant to anti-inflammatory therapy. Misdiagnosis hinders appropriate management in these conditions and may result in poor outcomes. This review discusses commonly encountered neoplastic and non-neoplastic disease processes that masquerade as intraocular inflammation with a focus on relevant clinical features and adjunctive investigations that are helpful in reaching a correct diagnosis.
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  • 文章类型: Case Reports
    眼部结核是各种葡萄膜炎实体的伟大模拟者。我们介绍了一例29岁的男性,他的左眼视力模糊和漂浮物。在检查中,左眼有与视网膜炎相关的前房细胞和玻璃体炎。他没有其他症状。最初的表现与眼部弓形虫病一致,他开始口服磺胺甲恶唑-甲氧苄啶,并对治疗表现出良好的反应。然而,检查结果显示弓形虫抗体滴度阴性,但结核分枝杆菌干扰素-γ释放试验和Mantoux试验阳性,使眼结核的诊断更有可能。病人被转移到抗结核治疗,最终导致炎症消退,视力恢复。眼结核的诊断需要详细的病史以及微生物学和免疫学研究。治疗眼科医生的高度怀疑指数对于揭示诊断是必要的。
    Ocular tuberculosis is a great mimicker of various uveitis entities. We present a case of a 29-year-old male who came in with blurring of vision and floaters in the left eye. On examination, the left eye had anterior chamber cells and vitritis associated with retinitis. He had no other symptoms. The initial presentation was consistent with ocular toxoplasmosis, and he was started on oral sulfamethoxazole-trimethoprim and showed a good response to the treatment. However, work-up revealed negative toxoplasma antibody titers but a positive M. tuberculosis interferon-gamma release assay test and Mantoux test, making the diagnosis of ocular tuberculosis more likely. The patient was shifted to antituberculous therapy, which eventually resulted in the resolution of the inflammation with a recovery of the visual acuity. The diagnosis of ocular tuberculosis requires a detailed medical history as well as microbiologic and immunologic studies. A high index of suspicion by the treating ophthalmologist is necessary to reveal the diagnosis.
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  • 文章类型: Case Reports
    肿瘤向视网膜的转移是相对罕见的。我们报告了一个具有临床和组织病理学相关性的系统性恶性肿瘤视网膜转移的独特病例。
    一名62岁女性,有IV期肺小细胞癌病史(SCC,化疗和维持免疫治疗后的状态)表现为手部动作视觉和玻璃体出血,既往玻璃体切除术和活检后的状态为非诊断性。她被发现患有单侧视网膜转移性肿瘤,并接受了重复的玻璃体辅助活检,证实了诊断。由于复发性非清除玻璃体出血和鬼细胞青光眼,眼睛变得失明和疼痛,并被摘除。对全球进行的详细组织病理学分析证实,小细胞癌转移到视网膜和玻璃体腔,并保留了脉络膜。
    该病例表明,对于已知有新的玻璃体视网膜病变的恶性肿瘤病史的患者,保持高度怀疑转移的重要性。
    UNASSIGNED: Tumor metastases to the retina are a relatively rare occurrence. We report a unique case of retinal metastasis of a systemic malignancy with clinical and histopathologic correlations.
    UNASSIGNED: A 62-year-old female with a history of stage IV small cell carcinoma of the lung (SCC, status post chemotherapy and maintenance immunotherapy) presented with hand motions vision and vitreous hemorrhage, status post prior vitrectomy and biopsy that was non-diagnostic. She was found to have unilateral retinal metastatic tumor and underwent a repeat vitrector-assisted biopsy which confirmed the diagnosis. The eye became blind and painful due to recurrent non-clearing vitreous hemorrhage and ghost cell glaucoma and was enucleated. Detailed histopathologic analysis of the globe confirmed small cell carcinoma metastatic to the retina and vitreous cavity and sparing the choroid.
    UNASSIGNED: This case demonstrates the importance of maintaining a high index of suspicion for metastasis in patients with a known history of malignancy who present with new vitreoretinal lesions.
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  • 文章类型: Journal Article
    X连锁视网膜劈裂(XLRS)在葡萄膜炎患者中也显示出特征,被认为是葡萄膜炎伪装综合征。这项回顾性研究旨在描述最初诊断为葡萄膜炎的XLRS患者的特征,并将其与最初诊断为XLRS的患者进行对比。患者转诊到葡萄膜炎诊所,结果证明有XLRS(n=4),包括转诊到遗传性视网膜疾病诊所的患者(n=18)。所有患者都接受了全面的眼科检查,包括眼底照相的视网膜成像,超宽视野眼底成像,和光学相干断层扫描(OCT)。在最初诊断为葡萄膜炎的患者中,黄斑囊样分裂通常被解释为炎性黄斑水肿;玻璃体出血通常被解释为眼内炎症.最初诊断为XLRS的患者很少(2/18;p=0.02)出现玻璃体出血。没有额外的人口统计,记忆,和解剖学上的差异被发现。对XLRS作为葡萄膜炎伪装综合征的认识增加可能有助于早期诊断,并可能防止不必要的治疗。
    X-linked retinoschisis (XLRS) shows features also seen in patients with uveitis and is recognized as an uveitis masquerade syndrome. This retrospective study aimed to describe characteristics of XLRS patients with an initial uveitis diagnosis and to contrast these to patients with an initial XLRS diagnosis. Patients referred to a uveitis clinic, which turned out to have XLRS (n = 4), and patients referred to a clinic for inherited retinal diseases (n = 18) were included. All patients underwent comprehensive ophthalmic examinations, including retinal imaging with fundus photography, ultra-widefield fundus imaging, and optical coherence tomography (OCT). In patients with an initial diagnosis of uveitis, a macular cystoid schisis was always interpreted as an inflammatory macular edema; vitreous hemorrhages were commonly interpreted as intraocular inflammation. Patients with an initial diagnosis of XLRS rarely (2/18; p = 0.02) showed vitreous hemorrhages. No additional demographic, anamnestic, and anatomical differences were found. An increased awareness of XLRS as a uveitis masquerade syndrome may facilitate early diagnosis and may prevent unnecessary therapies.
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  • 文章类型: Journal Article
    目的:确定诊断性玻璃体切除术后不明原因的全葡萄膜炎(PUO)的特征和结局。
    方法:回顾性分析了2013年至2020年为诊断/治疗目的进行玻璃体切除术的所有患者,其玻璃体活检结果为阴性,最终诊断没有得到临床支持。
    结果:在122只手术的眼睛中,36眼(29.5%)定义为PUO(67.8±14.9年)。目前的临床表现显示主要是双侧疾病(70%的眼睛),并有明显的后段受累:3.1±0.6玻璃体炎,61.1%的眼睛患有视网膜血管炎,44.4%伴有黄斑水肿,30.6%伴渗出性视网膜脱离。视力为1.2±0.7logMAR,在约3.5年的观察期内,高达90%的视力保持稳定或改善。所有呈现的临床特征都不能预测最终的视觉结果或生存。
    结论:在诊断/治疗性玻璃体切除术后,高达30%的病例存在PUO。这主要是双边情况显示慢性和总体稳定的长期结果,通常保持稳定的视觉功能。
    OBJECTIVE: 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.
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  • 文章类型: Case Reports
    本系列的目的是报告三例对常规治疗无反应的复发性或非典型葡萄膜炎的诊断所面临的挑战。高度怀疑,在新技术的帮助下,如细胞学,免疫组织化学,眼液流式细胞术,和对比增强磁共振成像,对于及时诊断葡萄膜炎伪装综合征可能是必要的。
    The aim of this series is to report challenges faced in diagnosis of three cases of recurrent or atypical uveitis not responding to conventional treatment. A high index of suspicion, aided by newer techniques, such as cytology, immunohistochemistry, flow cytometry of ocular fluids, and contrast-enhanced magnetic resonance imaging, may be necessary for a prompt diagnosis of uveitis masquerade syndromes.
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  • 文章类型: Case Reports
    未经授权:描述一例血管内大B细胞淋巴瘤的眼科发现和诊断。
    UNASSIGNED:临床病例观察报告。
    未经证实:一名西班牙51岁男子转诊到我们医院,诊断为泛葡萄膜炎。病人视力模糊,畏光,发烧,和减肥。眼部检查显示前葡萄膜炎,玻璃体炎,后杆上有多个圆形和椭圆形的乳脂状斑点。荧光素血管造影术,光学相干断层扫描(OCT),和血管OCT用于眼部检查。淋巴瘤的诊断是在随机正常皮肤活检的基础上制定的,显示血管内显着的CD20细胞性和广泛的CD3表达。
    未经证实:血管内淋巴瘤是一种罕见的结外弥漫性大B细胞淋巴瘤,通常由于非特异性症状而延迟诊断。因此,随机皮肤活检可能有助于诊断。
    UNASSIGNED: To describe the ophthalmic findings and diagnosis of a case of intravascular large B-cell lymphoma.
    UNASSIGNED: Clinical case observational report.
    UNASSIGNED: A Spanish 51-year-old man referred to our hospital with a diagnosis of panuveitis. The patient presented with blurred vision, photophobia, fever, and weight loss. Ocular examination revealed anterior uveitis, vitritis, and multiple round and oval creamy spots on the posterior pole. Fluorescein angiography, optical coherence tomography (OCT), and angio-OCT were used for the ocular examination. The diagnosis of lymphoma was formulated on the basis of a random normal skin biopsy, which showed significant CD20 cellularity within the vessels and extensive CD3 expression.
    UNASSIGNED: Intravascular lymphoma is a rare form of extranodal diffuse large B-cell lymphoma, often with delayed diagnosis because of the nonspecific symptoms. Hence, random skin biopsy could be useful in the diagnosis.
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