McNab Alan A, McKelvie Penny
*Orbital Plastic and Lacrimal Clinic, Royal Victorian Eye and Ear Hospital, Melbourne, Australia; †Centre for Eye Research Australia, University of Melbourne, Melbourne, Australia; and ‡Department of Anatomical Pathology, St Vincent's Hospital, Melbourne, Australia.
Ophthalmic Plast Reconstr Surg. 2015 May-Jun;31(3):167-78. doi: 10.1097/IOP.0000000000000364.
To review the current state of knowledge of IgG4-related ophthalmic disease (IgG4-ROD).
A review of the literature and personal experience of the authors.
IgG4-related disease is a recently recognized fibroinflammatory disorder that may affect 1 or more organs. It is characterized by lymphoplasmacytic infiltrates with large numbers of IgG4 positive plasma cells, storiform fibrosis, obliterative phlebitis, and eosinophil infiltration as well as peripheral eosinophilia, and in some cases, elevated serum levels of IgG4. These features are not always seen, and the diagnosis should be made by integrating clinical, imaging, and histopathological data, with reference to recently defined diagnostic criteria. IgG4-ROD forms a significant proportion of what has previously been labeled "idiopathic orbital inflammation" or reactive lymphoid hyperplasia. Orbital disease may occur alone, at the same time as disease elsewhere, or metachronously with systemic disease. Although almost any ocular adnexal tissue may be affected, there are several commoner recognizable patterns of IgG4-ROD: (1) sclerosing dacryoadenitis; 2) enlargement of orbital nerves (most commonly the infraorbital nerve) associated with orbital myositis and lacrimal gland disease, often in combination with paranasal sinus disease, eosinophilia, and systemic involvement; and 3) sclerosing orbital inflammation. Patients with IgG4-ROD should be investigated and monitored for other organ involvement. Some patients with IgG4-related disease may develop lymphoma, usually marginal zone lymphoma of mucosa-associated lymphoid tissue type. Treatment of IgG4-ROD includes the use of corticosteroids and other immunosuppressants. Rituximab has been shown to be very effective. Longer term studies on the natural course and treatment of IgG4-ROD are needed.
Patients presenting with orbital inflammatory lesions should have biopsies obtained whenever possible. The examining pathologist should routinely look for features of IgG4-ROD, and if found, the patient should be investigated for other organ involvement. Early treatment may prevent destructive changes in affected tissues.
综述IgG4相关性眼病（IgG4-ROD）的当前知识状态。
文献回顾及作者个人经验总结。
IgG4相关性疾病是一种最近才被认识的纤维炎性疾病，可累及一个或多个器官。其特征为淋巴细胞和浆细胞浸润，伴有大量IgG4阳性浆细胞、席纹状纤维化、闭塞性静脉炎、嗜酸性粒细胞浸润以及外周嗜酸性粒细胞增多，部分病例血清IgG4水平升高。这些特征并非总能见到，诊断应综合临床、影像学和组织病理学数据，并参考最近制定的诊断标准。IgG4-ROD在既往被标记为“特发性眼眶炎症”或反应性淋巴样增生中占相当比例。眼眶疾病可单独出现，与其他部位疾病同时出现，或与全身性疾病异时出现。尽管几乎任何眼附属器组织都可能受累，但IgG4-ROD有几种较常见的可识别模式：（1）硬化性泪腺炎；（2）眶神经增粗（最常见的是眶下神经），伴有眶肌炎和泪腺疾病，常合并鼻窦疾病、嗜酸性粒细胞增多及全身受累；（3）硬化性眼眶炎症。IgG4-ROD患者应接受检查并监测其他器官受累情况。一些IgG4相关性疾病患者可能会发展为淋巴瘤，通常为黏膜相关淋巴组织型边缘区淋巴瘤。IgG4-ROD的治疗包括使用糖皮质激素和其他免疫抑制剂。利妥昔单抗已被证明非常有效。需要对IgG4-ROD的自然病程和治疗进行更长期的研究。
出现眼眶炎性病变的患者应尽可能进行活检。检查病理学家应常规寻找IgG4-ROD的特征，如发现，应对患者进行其他器官受累情况的检查。早期治疗可预防受累组织的破坏性改变。
