Abstract：
Immunoglobulin (Ig) G4-related diseases are very rare diseases and are difficult to diagnose and treat. Here, we report a rare condition, an IgG4-related disease involving the bilateral renal pelvises, which was successfully treated with steroid therapy. A 64-year-old female was admitted with left flank pain and hematuria. Computed tomographic images showed irregular enhancing masses around the bilateral renal pelvises and lymph nodes in the left para-aortic area. A histopathological examination of a renal needle biopsy revealed numerous cells that were positive for IgG and more than 10 IgG4-positive cells per high-powered field. The IgG4/IgG ratio was more than 40%. Her serum IgG4 level was elevated to 1440 mg/dL, and her IgG4/IgG ratio was 82.1%. After a diagnosis of IgG4 sclerosing disease, a double-J ureteral stent was inserted and steroid therapy was performed for 20 weeks. Her symptoms dramatically improved, and the follow-up images showed a resolved state. There was no relapse of the disease for more than 6 months after the removal of the stent.
摘要：
免疫球蛋白（Ig）G4相关疾病是非常罕见的疾病，难以诊断和治疗。这里,我们报告了一种罕见的情况，涉及双侧肾盂的IgG4相关疾病，用类固醇治疗成功。一名64岁女性因左侧腹疼痛和血尿入院。计算机断层扫描图像显示双侧肾盂周围的不规则增强肿块和左主动脉旁区域的淋巴结。肾针活检的组织病理学检查显示，每个高倍视野中有许多IgG阳性的细胞和超过10个IgG4阳性细胞。IgG4/IgG比率超过40%。她的血清IgG4水平升高到1440mg/dL,IgG4/IgG比值为82.1%。诊断为IgG4硬化性疾病后，我们置入输尿管双J支架,并进行类固醇治疗20周.她的症状明显改善,和后续图像显示一个解决的状态。在移除支架后超过6个月没有疾病复发。
