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1.
黄韵洁  陈晓隆 《眼科新进展》2019,(11):1091-1095
IgG4相关性疾病是一种近几年才被认识的疾病,病因尚不明确。以同时或逐步出现的一种或多种组织和器官中大量IgG4阳性淋巴浆细胞浸润、席纹状纤维化、闭塞性静脉炎为特征,可伴有血清IgG4水平升高。当累及眼部时称为IgG4相关性眼病。任何眼附属器均可受累,多见于泪腺、眼眶脂肪、眶下神经、眼外肌和眼睑。本文将对近年来IgG4相关性眼病的流行病学特点、病理特点、临床表现、影像特点、诊断和治疗方面的最新进展作一综述。  相似文献   

2.
彭晓林  何为民 《国际眼科杂志》2019,19(11):1896-1900

IgG4相关疾病(IgG4-RD)是一种新近被认识的与IgG4淋巴细胞密切相关的慢性、系统性疾病。累及眼部时,命名为IgG4眼部相关疾病(IgG4-ROD)。IgG4-ROD临床表现受侵犯部位影响,与其他累及器官相比具有独特的病理特征。随着近年对该疾病临床认识的深入,诊疗率不断提高。本文对IgG4-ROD的概念、流行病学、病因及发病机制、临床表现、组织病理学、影像学检查、实验室检查、诊断与鉴别诊断,以及治疗方面的研究进展进行综述。  相似文献   


3.
IgG4相关性眼病属于发生在眼部的IgG4相关性疾病,泪腺是最常见的眼部受累部位。近年来,IgG4 相关性眼病的眼内受累及巩膜炎一直是研究的热点。现针对IgG4 相关性眼病进行综述,分析其病因、 临床特征、实验室检查、诊断及鉴别诊断。  相似文献   

4.
目的 对一组首诊于眼科的IgG4相关疾病患者进行总结分析.方法 回顾性病例分析.选取2011年1月~2021年4月解放军总医院第一医学中心神经眼科住院治疗的9例IgG4相关眼病(IgG4-ROD)纳入分析.总结患者临床表现、实验室检查、眼眶磁共振成像(MRI)、病理学检查和治疗情况.结果 9例患者,男性6例、女性3例,...  相似文献   

5.
近年来,免疫球蛋白G4(IgG4)相关性疾病作为一种新的临床疾病正逐渐受到医学界的广泛关注。尽管该病临床表现多种多样,但是血清IgG4质量浓度增高和组织中IgG4阳性浆细胞的大量浸润是其共有的特征。该病可累及全身多个器官,最常见的是胰腺,其次为腮腺、胆管、肝脏、肺脏和淋巴结等,累及眼眶者较为少见。目前研究初步显示,良性淋巴上皮病变、特发性眼眶炎性假瘤、Castleman病、黄色肉芽肿、Rosai—Dofman病等几种眼眶病具有IgG4相关性疾病的特征。就IgG4相关性眼眶病的研究进展进行综述。  相似文献   

6.
IgG4相关性眼病是近期被认识的一种良性淋巴增生性疾病。眼附属器黏膜相关淋巴组织(MALT)淋巴瘤是一种低度恶性淋巴细胞肿瘤。这2种疾病的临床特征有诸多相似性,为诊断和治疗带来一定困难,需要仔细鉴别。此外,近年来的研究发现这2种眼部疾病之间存在潜在联系,部分IgG4相关性眼病有可能进展为MALT淋巴瘤。因此,本文就2种疾病的对比以及相关性研究的最新进展做一综述。  相似文献   

7.
1例主诉为左眼视力下降1周;1例主诉为右眼视力减退2个月、结膜水肿10余天;1例主诉为双眼红肿1年,纳差、乏力4个月患者分别就诊眼科。3例患者均为男性,眼部检查均为双眼受累,2例表现为双眼泪腺肿大伴有球结膜水肿,1例为双眼眼睑肿胀,3例均伴有眶外组织受累,均诊断为IgG4相关眼病。经糖皮质激素冲击治疗后患者症状均好转,...  相似文献   

8.
IgG4相关性眼病(immunoglobulinG4-related ophthalmic disease,IgG4-ROD)是一种较为常见的眼部疾病,可以发展为淋巴瘤,但发生机制未明。眼附属器淋巴瘤是成年人最常见的发生于眼部的恶性肿瘤。IgG4-ROD和眼附属器淋巴瘤的临床表现和影像学特征具有相似性,故仅凭临床表现和...  相似文献   

9.
近年来,免疫球蛋白G4( IgG4)相关性疾病作为一种新的自身免疫性疾病,受到国内外医学界的的广泛关注。IgG4相关性疾病以IgG4+浆细胞浸润和血清IgG4浓度升高为特征,同时伴有因全身多器官的纤维化而发生肿大、结节性或增生性病变的自身免疫性疾病。当累及眼眶时称为IgG4相关性眼眶病。目前提出了IgG4相关性眼眶病的相对完善的诊断标准,然而治疗方面还没有公认有效的手段,目前首选激素治疗。本文就IgG4相关性眼眶病的临床特征、诊断和治疗的研究进展进行综述。  相似文献   

10.
IgG4相关性眼病是一种与IgG4密切相关的慢性系统性疾病,是以血清IgG4水平升高及IgG4阳性浆细胞浸润泪腺、眼外肌、眶下神经等眼部附属器为特征,而且经常是全身系统性病变的首发症状.糖皮质激素对该病治疗有效.本文就近年来IgG4相关性眼病的病因发病机制,临床表现,诊断及治疗方面的最新进展进行综述.  相似文献   

11.
AIM: To investigate clinicopathological features of immunoglobulin G4-related ophthalmic disease (IgG4-ROD), and analyze the recurrence rates following systemic corticosteroid administration. METHODS: We retrospectively searched clinical features, laboratory and histological findings based on the medical records of 21 patients with IgG4-ROD. All the patients examined in this study underwent surgical resection in the ocular adnexal lesions and underwent histological evaluation. This study further investigated clinical and histopathological features of 15 patients who received systemic corticosteroid after the resection. RESULTS: The mean age of the patients consisting of 7 males (33%) and 14 females (67%) was 61y. Fourteen patients were diagnosed as definitive, and 2 and 5 patients were probable and possible IgG4-ROD, respectively. Eyelid swelling was an initial symptom in 11 patients (52%) who did not show systemic involvements at a diagnosis. Fifteen patients received systemic corticosteroid administration, and all showed remission of inflammation. Among them, 10 patients did not recur, whereas 5 patients (33%) recurred during tapering. There were no significant difference between patients with or without recurrence in clinicopathological features. CONCLUSION: In this study, female patients are more predominant in IgG4-ROD. While inflammation recurs in one-third of patients, this study do not identify factors associated with recurrence after systemic corticosteroid administration.  相似文献   

12.
目的 总结以眼球运动障碍为首要表现的IgG4相关疾病(IgG4-RD)的临床特征。设计 回顾性病例系列。研究对象 2020年12月至2021年12月北京同仁医院以眼球运动障碍为首要表现的IgG4-RD患者8例。方法 回顾患者的病历资料,包括眼位、眼球运动障碍方向、血清IgG4水平、眼眶磁共振成像(MRI)眼外肌表现、病理检查结果 、治疗方案等。主要指标 临床表现、眼眶MRI及病理结果 。结果 8例(100%)患者均表现为眼球运动障碍,其中外展受限87.5%、上转受限75.0%、下转受限37.5%、内收受限37.5%;视力下降5例(62.5%),头痛/眼痛3例(37.5%)。眼眶MRI显示局限性眼上肌群和外直肌增粗伴强化2例(25.0%),局限性眼上肌群和外直肌受压2例(25.0%),弥漫性眼外肌群增粗伴强化3例(37.5%),眼外肌正常但颅脑MRI示脑膜增厚伴强化1例(12.5%)。患者均合并神经系统外部位受累,表现为泪腺增大5例(62.5%)、鼻窦黏膜增生肥厚4例(50.0%)、淋巴结反应性增生4例(50.0%)。眼眶肿物、鼻窦黏膜、胸腺组织病理中均可见大量IgG4阳性浆细胞,伴组织...  相似文献   

13.
IgG4-related disease is a fibro-inflammatory condition with tendency to form tumors with inflammatory infiltrate with IgG4 rich plasma cells and elevation of IgG4 level in serum, which may affect virtually every organ and tissue in the organism. IgG4-related ophthalmic disease may present as dacryoadenitis, myositis, other orbital tissues, hypophysitis or pachymeningitis causing cranial neuropathies. The diagnosis of IgG4-related disease is based on a typical clinical scenario, supportive laboratory data, expected radiological characteristics and distinct histopathological and immunohistochemical features. Corticosteroid followed by the use of long-term immunosuppressive therapy is the most commonly attempted treatment.  相似文献   

14.
AIM: To summarize the application of deep learning in detecting ophthalmic disease with ultrawide-field fundus images and analyze the advantages, limitations, and possible solutions common to all tasks. METHODS: We searched three academic databases, including PubMed, Web of Science, and Ovid, with the date of August 2022. We matched and screened according to the target keywords and publication year and retrieved a total of 4358 research papers according to the keywords, of which 23 studies were retrieved on applying deep learning in diagnosing ophthalmic disease with ultrawide-field images. RESULTS: Deep learning in ultrawide-field images can detect various ophthalmic diseases and achieve great performance, including diabetic retinopathy, glaucoma, age-related macular degeneration, retinal vein occlusions, retinal detachment, and other peripheral retinal diseases. Compared to fundus images, the ultrawide-field fundus scanning laser ophthalmoscopy enables the capture of the ocular fundus up to 200° in a single exposure, which can observe more areas of the retina. CONCLUSION: The combination of ultrawide-field fundus images and artificial intelligence will achieve great performance in diagnosing multiple ophthalmic diseases in the future.  相似文献   

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