Department of Pathology and Laboratory Medicine, Weill Cornell Medical College, New York, USA.
Curr Opin Rheumatol. 2011 Jan;23(1):95-101. doi: 10.1097/BOR.0b013e3283413011.
An enlarged salivary gland or lacrimal gland raises a wide differential diagnosis that includes both benign inflammatory conditions and malignant disorders. This review aims to address the numerous controversies that have arisen regarding inflammatory diseases of the salivary gland over the past two centuries and more specifically address the relevance of IgG4 in this setting.
A significant percentage of cases previously classified as Mikulicz disease, Küttner tumor, and orbital pseudotumor (idiopathic orbital inflammation) show elevated numbers of IgG4-positive plasma cells, and some of these cases also show elevated levels of serum IgG4. These data support the evolving concept of IgG4-associated sialadenitis/dacroadenitis. The disease presents with enlargement of one of more salivary gland(s) and/or lacrimal gland(s). Histologically this disease is characterized by a dense polyclonal lymphoplasmacytic infiltrate, and is frequently associated with germinal centers, fibrosis and obliterative phlebitis. IgG4-bearing plasma cells are virtually always present, as is an elevated ratio of IgG4 to IgG containing plasma cells.
IgG4-related sialadenitis belongs to the IgG4-related systemic disease spectrum and shows a swift response to immunosuppression.
唾液腺或泪腺肿大引起广泛的鉴别诊断，包括良性炎症性疾病和恶性疾病。本篇综述旨在解决过去两个多世纪以来唾液腺炎症性疾病所产生的众多争议，并特别针对 IgG4 在这种情况下的相关性进行讨论。
以前被归类为 Mikulicz 病、Küttner 肿瘤和眼眶假瘤（特发性眼眶炎症）的大量病例显示 IgG4 阳性浆细胞数量增加，其中一些病例还显示血清 IgG4 水平升高。这些数据支持 IgG4 相关的唾液腺/涎腺炎的不断发展的概念。该疾病表现为一个或多个唾液腺和/或泪腺肿大。组织学上，这种疾病的特征是密集的多克隆淋巴浆细胞浸润，常伴有生发中心、纤维化和闭塞性静脉炎。IgG4 阳性浆细胞几乎总是存在，IgG4 与 IgG 阳性浆细胞的比值升高。
IgG4 相关的唾液腺炎属于 IgG4 相关的系统性疾病谱，对免疫抑制有迅速的反应。
