Wang Shuo, Xu Xiangbo, Bai Zhaohui, Yi Fangfang, Wang Ran, Guo Xiaozhong, Qi Xingshun
Department of Gastroenterology, General Hospital of Northern Theater Command (formerly General Hospital of Shenyang Military Area), Shenyang, China.
Postgraduate College, China Medical University, Shenyang, China.
AME Case Rep. 2020 Oct 30;4:22. doi: 10.21037/acr-20-43. eCollection 2020.
Immunoglobulin G4 (IgG4) related disease is a rare autoimmune disease involving multiple organs and tissues. A diagnosis of IgG4-related disease (IgG4-RD) is mainly based on serum IgG4 concentration, imaging, pathology, and effective glucocorticoids therapy. In this paper, we report a 53-year-old male with typical signs and symptoms of IgG4-RD successfully treated with glucocorticoids. This patient had experienced bilateral mumps for more than 8 months and intermittent abdominal pain spreading to his lower back for 2 months before his admission. During his hospitalization, based on the characteristic appearance of magnetic resonance imaging (MRI), magnetic resonance cholangiopancreatography (MRCP), and computed tomography with positron emission tomography, a diagnosis of autoimmune pancreatitis (AIP), cholangitis with biliary obstruction, lachrymal adenitis, and submandibular adenitis was made. A high serum IgG4 concentration further supported a diagnosis of IgG4-RD. Then, the treatment was promptly initiated with corticosteroids. MRI, MRCP, and IgG4 concentration were re-examined during his follow up, suggesting that glucocorticoid treatment resulted in a resolution of his disease. The dosage of glucocorticoid had been gradually decreased. Now, he is stable with oral low-dose glucocorticoids. Certainly, long-term follow up of this patient with such a rare disease is very essential to observe the possibility of disease recurrence and glucocorticoids related complications.
免疫球蛋白G4（IgG4）相关疾病是一种累及多个器官和组织的罕见自身免疫性疾病。IgG4相关疾病（IgG4-RD）的诊断主要基于血清IgG4浓度、影像学、病理学以及糖皮质激素治疗有效。在本文中，我们报告了一名53岁男性，具有IgG4-RD的典型症状和体征，经糖皮质激素治疗成功。该患者入院前双侧腮腺炎发作超过8个月，间歇性腹痛蔓延至下背部2个月。住院期间，根据磁共振成像（MRI）、磁共振胰胆管造影（MRCP）以及正电子发射断层扫描计算机断层扫描的特征性表现，诊断为自身免疫性胰腺炎（AIP）、胆管炎伴胆道梗阻、泪腺炎和颌下腺炎。高血清IgG4浓度进一步支持IgG4-RD的诊断。随后，立即开始使用糖皮质激素进行治疗。随访期间复查MRI、MRCP和IgG4浓度，提示糖皮质激素治疗使疾病得到缓解。糖皮质激素剂量已逐渐减少。现在，他口服低剂量糖皮质激素病情稳定。当然，对这名患有如此罕见疾病的患者进行长期随访对于观察疾病复发的可能性以及糖皮质激素相关并发症非常重要。
