School of Medicine, University of Glasgow, University Avenue.
Otol Neurotol. 2022 Sep 1;43(8):856-863. doi: 10.1097/MAO.0000000000003614. Epub 2022 Aug 9.
IgG4-related disease (IgG4-RD) involving the temporal bone is an uncommon and underrecognized pathology often mistaken for malignancy. This systematic review is the first that aims to thoroughly analyze IgG4-RD of the temporal bone.
Ovid MEDLINE, EMBASE, Cochrane Library, and Google Scholar.
We used the following search keywords: "lgG4-RD," "skull," "skull base," "cranial," "temporal bone," "inner ear." We additionally manually searched the bibliographies of relevant articles. The JBI Critical Appraisal Checklist for Case Reports and Case Series was used to assess the risk of bias; because of the scarcity of the reports, data were available through limited case series and reports; thus, data synthesis was not possible.
We identified 17 studies with 22 cases with temporal bone involvement. The most common presenting symptoms were hearing loss, otalgia, and headache. The mastoid and petrous bone were the most affected anatomical areas. Both computed tomography and magnetic resonance imaging were used. Biopsies showed the characteristic lymphoplasmacytic infiltrate in all cases, with histopathology being the diagnostic modality that set the diagnosis. Most patients were treated with corticosteroids ± surgery or a combination of corticosteroids and immunosuppressants with 95.5% symptomatic response and disease control.
IgG4-RD of the temporal bone radiologically manifests as space-occupying, lytic lesions; clinically, it presents with vague otological symptoms. Diagnosis involves a thorough workup, with histopathology being crucial in setting a definite diagnosis. IgG4-RD tends to respond well to systemic corticosteroids, whereas surgery is mostly required for diagnostic purposes.
累及颞骨的 IgG4 相关疾病（IgG4-RD）是一种不常见且认识不足的疾病，常被误诊为恶性肿瘤。本系统评价是首次旨在全面分析颞骨 IgG4-RD 的研究。
Ovid MEDLINE、EMBASE、Cochrane 图书馆和 Google Scholar。
我们使用了以下搜索关键词：“lgG4-RD”、“颅骨”、“颅底”、“颅”、“颞骨”、“内耳”。我们还手动搜索了相关文章的参考文献。使用 JBI 病例报告和病例系列的批判性评估清单来评估偏倚风险；由于报告的稀缺性，数据可通过有限的病例系列和报告获得；因此，无法进行数据综合。
我们确定了 17 项研究，其中有 22 例颞骨受累病例。最常见的表现症状是听力损失、耳痛和头痛。乳突和岩骨是最常受累的解剖区域。所有病例均行 CT 和 MRI 检查。活检显示所有病例均有特征性的淋巴浆细胞浸润，组织病理学是确定诊断的诊断模式。大多数患者接受了皮质类固醇±手术或皮质类固醇和免疫抑制剂的联合治疗，症状缓解率为 95.5%，疾病控制率为 95.5%。
颞骨 IgG4-RD 影像学表现为占位性、溶骨性病变；临床上表现为模糊的耳科症状。诊断需要全面的检查，组织病理学在明确诊断中至关重要。IgG4-RD 对全身皮质类固醇反应良好，而手术主要用于诊断目的。
