Department of Urology, Institute of Wonkwang Medical Science, Wonkwang University School of Medicine and Hospital, Iksan, South Korea.
Department of Radiology, Institute of Wonkwang Medical Science, Wonkwang University School of Medicine and Hospital, Iksan, South Korea.
Saudi J Kidney Dis Transpl. 2023 Sep 1;34(5):449-454. doi: 10.4103/1319-2442.397207. Epub 2024 Mar 11.
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.
免疫球蛋白（Ig）G4 相关疾病是非常罕见的疾病，其诊断和治疗都具有一定难度。在这里，我们报告了一例罕见的 IgG4 相关疾病，涉及双侧肾盂，经类固醇治疗后成功治愈。一名 64 岁女性因左侧腰痛和血尿入院。计算机断层扫描图像显示双侧肾盂周围和左肾旁区的不规则增强肿块以及淋巴结。肾活检的组织病理学检查显示大量细胞 IgG 阳性，高倍视野中 IgG4 阳性细胞超过 10 个。IgG4/IgG 比值大于 40%。她的血清 IgG4 水平升高至 1440mg/dL，IgG4/IgG 比值为 82.1%。在诊断为 IgG4 硬化性疾病后，插入双 J 输尿管支架，并进行了 20 周的类固醇治疗。她的症状显著改善，随访图像显示已缓解。支架取出后 6 个月以上无疾病复发。
