IgG4相关性眼病的诊疗研究进展

免疫球蛋白G4(IgG4)相关性疾病(IgG4-RD)是近年来受关注度较高的一类慢性、系统性疾病。IgG4-RD可累及全身各个组织,主要表现为累及器官的肿胀及占位改变,当病变侵犯至眼部周围时称为IgG4相关性眼病(IgG4-ROD),主要侵犯泪腺、眼眶脂肪、眶下神经、眼外肌和眼睑等。目前,针对IgG4-ROD的主要治疗方式有药物治疗、手术、放射治疗等。随着近年对该病认识的不断提升,治疗有效率不断提高,本文就IgG4-ROD的流行病学特点、临床表现、影像特点、诊断和治疗的最新进展进行综述。     

以眼球运动障碍为首要表现的IgG4相关疾病的临床特征

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...     

IgG4相关性眼病研究进展

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。     

IgG4相关眼病三例

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,...     

国人甲状腺相关眼病患者血清免疫球蛋白4水平的研究

目的通过检测甲状腺相关眼病（TAO）患者血清IgG4和IgG的水平,探究IgG4与TAO之间的关系。方法前瞻性病例对照研究。分析和比较在第二军医大学附属长征医院眼科2014年9月至2015年7月期间收治的88例TAO患者和37例对照组的血清IgG4和IgG检测结果,研究IgG4和TAO临床指标之间的关联性。结果TAO活动期组的IgG4水平显著高于TAO静止期组（P=0．046）以及对照组（P=0．021）;TAO静止期组和对照组的IgG4水平差异无统计学意义（P=0．963）。TAO活动期组血清IgG4升高（IgG4≥135mg／dL）的比例显著高于静止期组（P=0．003）和对照组（P=0．007）;TAO静止期组和对照组之间差异无统计学意义（P=0．896）。TAO活动期组IgG4／IgG增加（IgG4／IgG8．0％）的比例显著高于静止期组（P=0．023）以及对照组（P=0.017）,但TAO静止期组和对照组之间差异无统计学意义（P=0．756）。CAS为血清IgG4水平的独立影响因素（P=0.021）。IgG4与性别、年龄、TAO病程、突眼度、CAS和严重程度之间均无线性相关性（P值分别为0．767、0.469、0．335、0．385、0．281和0．527）。结论部分TAO活动期患者血清IgG4水平、IgG4／IgG显著升高,并与CAS有关,提示TAO发病机制的异质性可能。     

IgG4相关性眼病的病理诊断研究进展

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。     

IgG4相关眼病的机制及诊疗研究进展

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。     

IgG4相关性眼病和眼附属器黏膜相关淋巴组织淋巴瘤的关系研究进展

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。     

眼眶IgG4相关疾病一种仍在演变中眼眶综合征

眼眶IgG4相关疾病具有一系列独特病理生理改变,主要表现为眼附属器组织有IgG4阳性淋巴浆细胞浸润,常伴血清IgG4升高,多双侧发病,累及多个系统,常有过敏性疾病史,并且有发展为眼眶淋巴瘤的可能.文中就其临床表现、病理特征与其他疾病的关系及治疗等方面展开综述.     

IgG4相关性眼病与眼附属器淋巴瘤的关系及鉴别

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...     

A clinicopathological study on IgG4-related ophthalmic disease

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.     

IgG4-related ophthalmic disease

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.     

IgG4-related dacryoadenitis

A 44-year-old female presented with bilateral eyelid swelling and painless, palpable mass over the lacrimal gland area for more than 1 year. A bilateral enlarged lacrimal gland with mild contrast enhancement was noted on computed tomography. Biopsy of the lacrimal gland showed lymphoplasmacytic infiltrate with focal hyaline and sclerotic change, and immunohistochemical staining revealed numerous IgG4-positive plasma cells, which was characteristic of IgG4 dacryoadentitis. Serum IgG and IgG4 levels were within normal range. The patient was treated with surgical excision instead of systemic steroid owing to poor tolerance. No tumor recurrence or systemic involvement was noted during the follow-up period. Our case highlights the importance of IgG4 dacryoadenitis in differentiating bilateral lacrimal gland inflammatory conditions. A normal serum IgG4 level does not rule out the diagnosis and surgical excision may be an alternative for those who are considered ineligible for corticosteroid treatment.     

IgG4相关性疾病眼部病变的临床及影像学特征分析

目的 探讨IgG4相关性疾病眼部病变的临床及影像学特征。设计 回顾性病例系列。研究对象 25例在2010年10月-2014年5月期间首诊于眼科并经实验室和（或）活组织检查证实的IgG4相关性疾病患者。方法 回顾性分析25例患者眼部病变的临床及影像学表现。主要指标 临床表现,眼眶MRI,生长抑素受体SPECT/CT显像（SRS）。结果 25例患者双眼睑肿胀、眼球突出为主要临床表现,其中23例为双眼受累,22例患者有≥2个器官受累,眼外受累器官多见于鼻部（19例）和涎腺（13例）。眼眶MRI主要表现为弥漫性泪腺增大（22例）、眼外肌增粗（14例）、眶周软组织浸润（10例）等。SRS显示治疗前眼眶摄取示踪剂显著增高（UR=1.87±0.43）;经免疫抑制治疗后活动期眼眶示踪剂摄取减少（UR=1.51±0.24）。结论 IgG4相关性疾病的眼部病变具有较典型的临床特征,泪腺弥漫性增大为眼眶MRI最常见表现,SRS能客观反映受累眼眶的免疫活动程度,对临床疗效评价有较好的价值。 （眼科,2015, 24： 309-312）       

Adjunctive Orbital Radiotherapy for Ocular Adnexal IgG4-related Disease: Preliminary Experience in Patients Refractory or Intolerant to Corticosteroid Therapy

Abstract

Purpose: To present the clinical outcomes of combined orbital radiotherapy and systemic corticosteroid for patients with refractory ocular adnexal IgG4-related disease.

Methods: We retrospectively reviewed 3 patients with histopathologically confirmed ocular adnexal IgG4-related disease who had been refractory or intolerant to corticosteroid therapy and treated with adjunctive orbital radiotherapy (2000 cGy; 10 fractions). Clinical improvement was assessed by monitoring the patient's ability to taper corticosteroid to discontinuation and by follow-up radiologic examination.

Results: All 3 patients had a favorable response to adjunctive radiotherapy with improvement of the clinical symptoms and radiologic abnormalities. Systemic corticosteroid was tapered and discontinued in all patients successfully. There were no adverse effects of treatment or recurrence after a mean follow-up of 19 months.

Conclusion: Adjunctive radiotherapy can help to achieve stable disease and cessation of systemic corticosteroid in patients with refractory ocular adnexal IgG4-related disease.     

免疫球蛋白G4相关性眼眶病的研究进展

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。     

A Case of Orbital Involvement in IgG4-Related Disease

A 46-year-old male was referred to the Ophthalmology Service for a 7-year history of bilateral proptosis and a presumptive diagnosis of thyroid eye disease. Past medical history was only significant for autoimmune pancreatitis. All laboratory testing including tests of thyroid function were within normal limits. The patient underwent orbital biopsy and was found to have plasma cells containing mainly IgG4 immunoglobulin that was consistent with IgG4-related disease. The patient was treated with oral prednisone and the proptosis resolved within 3 weeks.