Division of Rheumatology, Department of Internal Medicine, Eskişehir Osmangazi University, Eskişehir, Turkey.
Department of Rheumatology, Faculty of Medicine, Eskişehir Osmangazi University, 26480, Eskisehir, Turkey.
Rheumatol Int. 2024 Mar;44(3):557-572. doi: 10.1007/s00296-023-05419-x. Epub 2023 Aug 27.
IgG4-related disease (IgG4-RD) is a fibroinflammatory condition that is characterized by storiform fibrosis, infiltration of IgG4-positive lymphocytes, obliterative phlebitis, and high IgG4 levels. Since IgG4-RD affects a wide variety of organs, a differential diagnosis must include multiple conditions. IgG4-RD is also believed to coexist with certain diseases. In recent years, case reports and case series describing the co-occurrence of IgG4-RD and ANCA-associated vasculitis (AAV) have been published. We intended to evaluate patients with IgG4-RD and AAV overlap in the literature using a case similar to one that was diagnosed and monitored in our department. We searched the databases of Web of Science, Scopus, and Google Scholar as well as PubMed with the keywords ANCA, IgG4, IgG4-RD, granulomatosis with polyangiitis, Wegener's granulomatosis, microscopic polyangiitis, Eosinophilic granulomatosis with polyangiitis, and Churg-Strauss syndrome. Cases and Case series addressing the coexistence of IgG4-RD and AAV have been selected. Comprehensive diagnostic criteria are used to diagnose IgG4-RD. The Chapel Hill Consensus Conference nomenclature criteria were used for the inclusion of AAV. Out of a total of 910 publications, 20 articles, including 65 cases, were found to be eligible. Forty-seven cases with IgG4-RD were evaluated as definitive (71.2%), 10 cases as probable (15.1%), and 9 cases as possible IgG4-RD (13.6%). 26 patients were diagnosed with GPA, 1 patient with localized GPA, 23 patients with MPA, and 4 patients with EGPA. The aorta, lacrimal tissue, pancreas, and retroperitoneum are the sites of IgG4-RD rather than AAV. AAV and IgG4-RD might coexist in the same patient. IgG4-RD is mainly associated with GPA.
IgG4 相关疾病（IgG4-RD）是一种纤维炎症性疾病，其特征为席纹状纤维化、IgG4 阳性淋巴细胞浸润、闭塞性静脉炎和高 IgG4 水平。由于 IgG4-RD 影响多种器官，因此必须包括多种疾病的鉴别诊断。IgG4-RD 也被认为与某些疾病共存。近年来，描述 IgG4-RD 与 ANCA 相关性血管炎（AAV）共存的病例报告和病例系列已经发表。我们旨在使用与我们科室诊断和监测的病例相似的病例，通过文献评估 IgG4-RD 和 AAV 重叠患者。我们使用关键词 ANCA、IgG4、IgG4-RD、肉芽肿性多血管炎、韦格纳肉芽肿、显微镜下多血管炎、嗜酸性肉芽肿性多血管炎和变应性肉芽肿性血管炎，在 Web of Science、Scopus 和 Google Scholar 数据库以及 PubMed 中进行了搜索。选择了涉及 IgG4-RD 和 AAV 共存的病例和病例系列。综合诊断标准用于诊断 IgG4-RD。采用 Chapel Hill 共识会议命名标准纳入 AAV。在总共 910 篇出版物中，发现了 20 篇文章，包括 65 例符合条件。47 例 IgG4-RD 被评估为明确（71.2%），10 例为可能（15.1%），9 例为可能 IgG4-RD（13.6%）。26 例患者诊断为 GPA，1 例为局限性 GPA，23 例为 MPA，4 例为 EGPA。IgG4-RD 的部位是主动脉、泪腺组织、胰腺和腹膜后，而不是 AAV。AAV 和 IgG4-RD 可能存在于同一患者中。IgG4-RD 主要与 GPA 相关。
