Abstract：
IgG4-related retroperitoneal fibrosis is a rare cause of renal dysfunction that usually manifests as obstructive nephropathy (sometimes with extrarenal manifestations). Due to the non-specific symptoms at the onset of the disease and often latent course, special laboratory and instrumental examination methods are usually needed to establish a diagnosis. The article describes a clinical case of a relapse of IgG4-related retroperitoneal fibrosis in a 53-year-old patient, who developed bilateral ureterohydronephrosis with postrenal acute kidney injury after the withdrawal of glucocorticoid therapy. The patient underwent bilateral percutaneous nephrostomy and resumed glucocorticoids at a dose of 30 mg/day. Postobstructive diuresis was 22 L. Treatment resulted in a complete normalization of the creatinine level and transient hypokalemia, which was eliminated with potassium medications. At the final stage of the treatment, bilateral stenting of both ureters was performed with a tapering of glucocorticoids to 5 mg per day with CT control of the retroperitoneal space after 5 months. A clinical case demonstrates that an interruption of glucocorticoid treatment in patients with IgG4-related retroperitoneal fibrosis can lead to ureterohydronephrosis with the development of acute kidney injury. In such cases, stenting of the ureters could be considered an optimal therapeutic option.
摘要：
IgG4相关的腹膜后纤维化是肾功能障碍的罕见原因，通常表现为阻塞性肾病（有时伴有肾外表现）。由于疾病发作时的非特异性症状和经常潜伏的病程，通常需要特殊的实验室和仪器检查方法来建立诊断。文章介绍了一例53岁患者IgG4相关腹膜后纤维化复发的临床病例,停用糖皮质激素治疗后出现双侧输尿管积水伴肾后急性肾损伤。患者接受了双侧经皮肾造口术，并以30mg/天的剂量恢复了糖皮质激素。梗阻性利尿为22L。治疗导致肌酐水平完全正常化和短暂性低钾血症，用钾药物消除了。在治疗的最后阶段，在5个月后,在CT控制腹膜后间隙的情况下,通过逐渐减少糖皮质激素至每天5mg的剂量对两个输尿管进行双侧支架置入.一个临床病例表明，在IgG4相关腹膜后纤维化患者中中断糖皮质激素治疗可导致输尿管肾积水，并发展为急性肾损伤。在这种情况下,输尿管支架置入术可视为最佳治疗选择.
