Department of Radiology, Tongji Hospital, Tongji Medical College, Huazhong University of Science and Technology, Wuhan, 430030, China.
Department of Nuclear Medicine, Tongji Hospital, Tongji Medical College, Huazhong University of Science and Technology, Wuhan, 430030, China.
Curr Med Sci. 2021 Apr;41(2):390-397. doi: 10.1007/s11596-021-2359-5. Epub 2021 Apr 20.
The features and treatment of 98 Chinese patients with immunoglobulin G4 (IgG4)-related disease (IgG4-RD) referred to a single tertiary referring centre were reviewed. Patients diagnosed with IgG4-RD according to the comprehensive diagnostic criteria (CDC) were included in the retrospective study from May 2012 to March 2019. We collected data on clinical, laboratory, imaging, histological features and treatment. Totally, 98 patients with IgG4-RD were enrolled. The common clinical manifestations included abdominal pain, salivary gland swelling and lymphadenopathy. 51% of the patients had multiple organs involvement. Lymph nodes, pancreas and salivary glands were most commonly involved. Four rare sites including ulna, cerebellum, scalp, and mammary gland were found. The serum IgG4 level was increased by 85.7%. The serum IgG4 level was positively correlated with the number of involved organs, IgG and IgG4/IgG. Low C3 and C4 levels were observed in 37.5% and 12.2% patients respectively, and all patients with kidney involvement had hypocomplementemia. A total of 54 patients underwent tissue biopsies, and 55.6%, 31.5% and 11.1% cases were diagnosed as definite, probable and possible IgG4-RD, respectively. Eighty-eight patients received glucocorticoids (GCs) therapy. Five patients underwent radical surgery to remove the lesion. 73% of them presented a complete or partial remission. IgG4-RD is a systemic fibroinflammatory disease with involvement of multiple organs throughout the body including some rare sites. Most IgG4-RD patients had increased serum IgG4 levels and patients with kidney involvement showed hypocomplementemia. GCs therapy is effective. More research is needed to provide a more reliable basis for the diagnosis and treatment of patients.
对 98 例就诊于单一三级转诊中心的 IgG4 相关疾病（IgG4-RD）中国患者的特征和治疗进行了回顾性研究。根据综合诊断标准（CDC）诊断为 IgG4-RD 的患者纳入本回顾性研究，纳入时间为 2012 年 5 月至 2019 年 3 月。我们收集了临床、实验室、影像学、组织学特征和治疗的数据。共纳入 98 例 IgG4-RD 患者。常见的临床表现包括腹痛、唾液腺肿胀和淋巴结病。51%的患者存在多器官受累。淋巴结、胰腺和唾液腺最常受累。还发现了 4 个罕见部位，包括尺骨、小脑、头皮和乳腺。85.7%的患者血清 IgG4 水平升高。血清 IgG4 水平与受累器官数量、IgG 和 IgG4/IgG 呈正相关。分别有 37.5%和 12.2%的患者出现低 C3 和 C4 水平，所有肾脏受累患者均存在低补体血症。共有 54 例患者接受了组织活检，分别有 55.6%、31.5%和 11.1%的病例被诊断为明确、可能和可能 IgG4-RD。88 例患者接受了糖皮质激素（GCs）治疗。5 例患者接受了根治性手术切除病变。其中 73%的患者完全或部分缓解。IgG4-RD 是一种全身性纤维炎症性疾病，全身多器官受累，包括一些罕见部位。大多数 IgG4-RD 患者的血清 IgG4 水平升高，肾脏受累患者存在低补体血症。GCs 治疗有效。需要进一步研究为患者的诊断和治疗提供更可靠的依据。
