首诊于眼科的IgG4相关疾病初步研究

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,...     

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。     

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。     

特发性眼眶炎性假瘤患者血清中IgG及其亚型水平的研究

目的通过检测特发性眼眶炎性假瘤(IOIP)患者血清中Ig G、Ig G1、Ig G2、Ig G3和Ig G4的水平,探究Ig G及其亚型与特发性眼眶炎性假瘤发病之间的关系。方法回顾性病例系列研究。回顾性分析首都医科大学附属北京同仁医院眼肿瘤专科2013年1月至2014年12月间经病理组织学确诊的44例IOIP患者的血清学检测结果,研究其Ig G、Ig G1、Ig G2、Ig G3和Ig G4的血清学水平。结果血清学分析Ig G水平为(1211.9±337.7)mg/d L,44例中3例Ig G升高,约占6.8%,1例Ig G下降,约占2.3%;Ig G1水平为(625.6±267.4)mg/d L,44例中4例Ig G1升高,占9.1%,4例Ig G1下降,占9.1%;Ig G2水平为(535.6±221.2)mg/d L,44例中5例Ig G2升高,2例下降,分别占11.4%和4.5%;Ig G3水平为(54.4±30.7)mg/d L,44例中4例Ig G3下降,约占9.1%;Ig G4水平为(107.8±247.5)mg/d L,44例中16例Ig G4升高,占36.4%,Ig G4/Ig G比值约为8.9%。结论超过三分之一的IOIP患者血清Ig G4升高,且Ig G4/Ig G比值高于正常水平,提示部分IOIP属于Ig G4相关性疾病。     

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）       

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.     

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.     

Precursor B-cell lymphoblastic lymphoma presenting as an orbital mass

A previously healthy 12-year-old boy presented with acute onset of proptosis of his left eye. CT scan demonstrated a mass involving the left orbit, left maxillary sinus, and left ethmoid sinus with extension through the cribriform plate into the anterior cranial fossa. Incisional biopsy of the mass revealed a precursor B-cell lymphoblastic lymphoma. Precursor B-cell lymphoblastic lymphoma is a rare type of non-Hodgkin's lymphoma seen exclusively in children and young adults. This is the first reported case of precursor B-cell lymphoblastic lymphoma presenting in the orbit. Treatment is primarily by systemic chemotherapy and is potentially curative. The principal role of the ophthalmologist is in diagnosis and monitoring of such patients. The clinical features and multidisciplinary diagnosis and management of childhood non-Hodgkin's lymphomas are reviewed.     

IgG4-Related Mikulicz’s Disease Associated with Thyroiditis: a Case Report and Review of the Literature

 Purpose: To report an unusual case of IgG4-related Mikulicz’s disease associated with thyroiditis. Case report: We describe a 25-year-old Chinese man who presented with bilateral, painless swellings of the lachrymal glands, parotid glands, and thyroid nodules. The patient underwent left-sided dacryoadenectomy and the diagnosis of IgG4-related Mikulicz’s disease was pathologically confirmed. The size of the right-sided lachrymal gland and parotid glands recovered fundamentally after one month of glucocorticoid therapy. Conclusion: IgG4-related Mikulicz’s disease associated with thyroiditis should be considered in the differential diagnosis of bilateral swellings of lachrymal glands, salivary glands, and thyroid nodules. Surgical excision is recommended in order to treat the tumor and to ensure the pathological diagnosis. Glucocorticoid therapy should be considered in association with surgery after removal.     

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 orbital disease masquerading as thyroid eye disease,vice versa,or both?

A 40 year-old male presented after one year of unilateral, progressive, steroid-responsive, orbital inflammatory disease causing proptosis, extraocular muscle (EOM) restriction, and compressive optic neuropathy. The development of anti-thyroidal antibodies prompted the diagnosis of thyroid eye disease (TED); however, the prolonged active phase, remarkable reversibility of ophthalmic features with high-dose corticosteroids, unilaterally of disease, uncharacteristic EOM involvement (including both obliques), and the absence of autoimmune thyroid disease provoked consideration of alternative diagnoses. Inferior oblique biopsy stained positive for IgG4 with histologic features atypical of TED. The patient received rituximab for presumed IgG4-related orbital disease (IgG4-ROD) with subsequent reversal of compressive optic neuropathy, near complete resolution of EOM restriction, and improved proptosis, the latter two of which are not routinely anticipated in advanced TED. The possible role for B-cell depletion in both TED and IgG4-ROD suggests a degree of overlap in the underlying immune-related pathophysiology that is yet to be defined.     

Orbital Manifestations of Immunoglobulin G4–Related Disease in Bilateral Lacrimal Glands,Optic Nerves,Trigeminal Nerves,and Maxillary Sinuses

Immunoglobulin G4 (IgG4)-related disease is characterised by numerous aggregates of IgG4-positive plasma cells in multiple organs. We report two patients who had bilateral proptosis associated with extensive inflammation bilaterally in lacrimal glands, optic nerves, trigeminal nerves, and maxillary sinuses. The patients were treated as idiopathic orbital inflammation syndrome with corticosteroid pulse therapy. As symptoms relapsed upon tapering, a reassessment of immunohistochemical stains of the lacrimal glands confirmed the diagnosis of IgG4-related disease. During 2 years of follow-up, the inflammation regressed spontaneously without any medical treatment in the first patient; however, inflammation in the other patient progressed, and he lost his vision. The extensive orbital involvement, characteristic pathological findings, and slowly progressive clinical course might help practitioners differentiate orbital IgG4-related disease from presumed idiopathic orbital inflammation syndrome.     

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.