Department of Oral Diagnosis, Piracicaba Dental School, University of Campinas, Piracicaba, Brazil.
Service of Surgery and Oral Pathology, João de Barros Barreto University Hospital, Universidade Federal do Pará, Belém, Brazil.
J Oral Pathol Med. 2022 May;51(5):493-500. doi: 10.1111/jop.13296. Epub 2022 Apr 11.
IgG4-related disease is a fibroinflammatory and immune-mediated condition, which has extremely variable clinical manifestations. In this study, we aim to investigate the clinicopathological features of IgG4-related disease involving the oral and maxillofacial region.
Cases of IgG4-related disease manifesting in the oral and maxillofacial region were retrieved from three Brazilian institutions. Clinical and serological data were obtained from the patients' medical charts, while microscopic and immunohistochemical findings were revised by oral pathologists. Diagnosis followed the American College of Rheumatology/European League against Rheumatism criteria.
Seven patients diagnosed with IgG4-related disease were included in this study. Women were affected in all analysed cases, with a mean age of 55.4 years. Two patients presented with the clinical involvement of more than one oral and maxillofacial anatomic site. Therefore, our sample comprised nine oral and maxillofacial anatomic sites affected by IgG4-related disease. The submandibular gland was affected in four cases, the tongue and the parotid gland in two cases each, and the palate in one case. In a few cases, exploratory lower lip biopsy was used as a diagnostic approach. A moderate-to-severe lymphoid infiltrate containing plasma cells and lymphocytes, with an increased IgG4/IgG ratio, was common. Treatment varied and steroids were the most frequently used (57.4%). Six patients remained alive, while one died from unknown causes.
Although major salivary glands are commonly affected by IgG4-related disease, the oral cavity can also be involved, and lower lip biopsy may be an auxiliary diagnostic tool.
IgG4 相关疾病是一种纤维炎症性和免疫介导性疾病，其临床表现极其多样。本研究旨在探讨累及口腔颌面部的 IgG4 相关疾病的临床病理特征。
从巴西的三家机构中检索出表现为口腔颌面部的 IgG4 相关疾病的病例。临床和血清学数据来自患者的病历，而口腔病理学家则对显微镜和免疫组织化学检查结果进行了复查。诊断依据美国风湿病学会/欧洲抗风湿病联盟的标准。
本研究共纳入 7 例 IgG4 相关疾病患者。所有分析病例均为女性，平均年龄为 55.4 岁。有 2 例患者的临床受累部位超过 1 个口腔颌面部解剖部位。因此，我们的样本包括 9 个受累于 IgG4 相关疾病的口腔颌面部解剖部位。4 例患者的颌下腺受累，2 例患者的舌和腮腺受累，1 例患者的腭受累。在少数情况下，采用下唇活检作为诊断方法。常见的表现为中等至重度淋巴样浸润，其中含有浆细胞和淋巴细胞，且 IgG4/IgG 比值升高。治疗方法多种多样，皮质类固醇是最常用的治疗方法（57.4%）。6 例患者存活，1 例患者因不明原因死亡。
尽管 IgG4 相关疾病常累及大唾液腺，但口腔也可受累，下唇活检可能是一种辅助诊断工具。
