Department of Internal Medicine #3, Bogomolets National Medical University, T. Shevchenko Boulevard, 13, Kiev, 01601, Ukraine.
The Therapy Department, Kyiv City Clinical Hospital #3, 26, P. Zaporozhtsia Street, Kiev, 02125, Ukraine.
Rheumatol Int. 2023 Nov;43(11):2141-2153. doi: 10.1007/s00296-023-05402-6. Epub 2023 Aug 5.
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.
IgG4 相关的腹膜后纤维化是肾功能障碍的一种罕见病因,通常表现为梗阻性肾病(有时伴有肾外表现)。由于疾病初期的非特异性症状和常为潜伏性病程,通常需要特殊的实验室和仪器检查方法来确立诊断。本文描述了一例 53 岁患者 IgG4 相关腹膜后纤维化复发的临床病例,该患者在停用糖皮质激素治疗后出现双侧输尿管梗阻性肾盂积水和肾后性急性肾损伤。患者接受了双侧经皮肾造瘘术,并以 30mg/天的剂量恢复使用糖皮质激素。解除梗阻后尿量为 22L。治疗导致肌酐水平完全正常化和短暂性低钾血症,通过补钾药物消除了低钾血症。在治疗的最后阶段,在 5 个月后通过 CT 对腹膜后间隙进行控制,将糖皮质激素逐渐减少至 5mg/天,并对双侧输尿管进行支架置入。该临床病例表明,IgG4 相关腹膜后纤维化患者中断糖皮质激素治疗可导致输尿管梗阻性肾盂积水和急性肾损伤。在这种情况下,输尿管支架置入术可能是一种最佳的治疗选择。