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首页> 外文期刊>Auris, nasus, larynx >A novel concept of Mikulicz's disease as IgG4-related disease.
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A novel concept of Mikulicz's disease as IgG4-related disease.

机译：Mikulicz病是IgG4相关疾病的新概念。

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Since Morgan's report in 1953, Mikulicz's disease (MD) has been considered part of primary Sjogren's syndrome (SS). However, MD has a unique presentation, including persistent swelling of the lacrimal and salivary glands, and is characterized by good responsiveness to glucocorticoids, leading to recovery of gland function. Recently, it has been revealed that MD patients show elevated serum immunoglobulin G4 (IgG4) levels and prominent infiltration of IgG4-positive plasmacytes. The complications of MD include autoimmune pancreatitis, retroperitoneal fibrosis, tubulointerstitial nephritis, autoimmune hypophysitis, and Riedel's thyroiditis, all of which show IgG4 involvement in their pathogenesis. Thus, MD is a systemic "IgG4-related disease." In addition, recent analyses have revealed that Kuttner's tumor (KT), a chronic sclerosing sialadenitis that presents with asymmetrical firm swelling of the submandibular glands, is also associated with prominent infiltration of IgG4-positive plasmacytes. MD and KT differ from SS and are thought to be singular systemic IgG4-related plasmacytic diseases. Here we discuss the results of recent studies and provide an overview of MD as an IgG4-related disease.
机译：自1953年Morgan的报告以来，Mikulicz病（MD）被认为是原发性干燥综合征（SS）的一部分。然而，MD具有独特的表现，包括泪腺和唾液腺的持续肿胀，并且其特征在于对糖皮质激素的良好响应性，从而导致腺体功能的恢复。最近，已经发现MD患者显示出升高的血清免疫球蛋白G4（IgG4）水平和IgG4阳性浆细胞的显着浸润。 MD的并发症包括自身免疫性胰腺炎，腹膜后纤维化，肾小管间质性肾炎，自身免疫性垂体炎和Riedel甲状腺炎，所有这些均表明IgG4参与了其发病机理。因此，MD是全身性的“ IgG4相关疾病”。此外，最近的分析表明，库特纳氏肿瘤（KT）是一种慢性硬化性蛛网膜腺炎，表现为下颌下腺的不对称牢固肿胀，也与IgG4阳性浆细胞的显着浸润有关。 MD和KT与SS不同，被认为是单一的全身性IgG4相关浆细胞性疾病。在这里，我们讨论最近的研究结果，并提供MD作为IgG4相关疾病的概述。

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