Clinical Immunology Unit, Department of Clinical and Experimental Medicine, University of Pisa, Pisa, Italy.
Otorhinolaryngology Unit, Department of Surgery, Azienda Ospedaliero-Universitaria di Cagliari, University of Cagliari, Cagliari, Italy.
J Immunol Res. 2018 Apr 1;2018:6936727. doi: 10.1155/2018/6936727. eCollection 2018.
IgG4-related disease (IgG4-RD) is a rare fibroinflammatory condition that can affect almost any organ, characterized by swollen lesions and often by eosinophilia and elevated serum IgG4 concentrations. The diagnosis of IgG4-RD is a challenging task: in fact, single or multiple organs can be affected and clinical, serological, and histological findings can be heterogeneous. In IgG4-RD, the involvement of salivary glands is observed in 27% to 53% of patients. Several organ-specific conditions, now recognized as different manifestations of IgG4-related sialadenitis (IgG4-RS), were viewed in the past as individual disease entities. The study of salivary glands may sometimes be complex, because of the number of pathological conditions that may affect them, often with overlapping clinical pictures. Integration of different imaging techniques is often required in the case of swelling of salivary glands, even though biopsy remains the gold standard for a definite diagnosis of IgG4-RS. Thus, in this review, we discuss new insights in the pathogenesis of IgG4-RD, focusing on its clinical aspects and the tools that are currently available for a correct differential diagnosis when the salivary glands are involved.
IgG4 相关疾病（IgG4-RD）是一种罕见的纤维炎症性疾病，几乎可以影响任何器官，其特征为肿胀性病变，常伴有嗜酸性粒细胞增多和血清 IgG4 浓度升高。IgG4-RD 的诊断具有挑战性：事实上，单个或多个器官可能受到影响，临床、血清学和组织学表现可能存在异质性。在 IgG4-RD 中，27%至 53%的患者存在唾液腺受累。过去被认为是独立疾病实体的几种器官特异性疾病，现在被认为是 IgG4 相关唾液腺炎（IgG4-RS）的不同表现。由于可能影响唾液腺的病理情况众多，且常伴有重叠的临床表现，因此对唾液腺的研究有时可能较为复杂。即使活检仍是 IgG4-RS 明确诊断的金标准，但对于唾液腺肿胀，通常需要整合不同的影像学技术。因此，在本文中，我们讨论了 IgG4-RD 发病机制的新见解，重点讨论了其临床方面以及目前用于正确鉴别诊断时涉及唾液腺的工具。
