Objectives Idiopathic retroperitoneal fibrosis (iRPF) can lead to irreversible kidney damage. This study aimed to investigate predictors of irreversible renal dysfunction in patients with iRPF. Methods Eighty-three patients with newly diagnosed iRPF were enrolled between January 2010 and Sep 2022 at Zhongshan Hospital of Fudan University, including 60 in the training set and 23 in the validation set. They were regularly contacted or followed up via outpatient examinations by specialist doctors, who documented their condition and treatment progress. Predictors of irreversible renal dysfunction were identified using univariate and multivariate regression, logistic model, and receiver operating curve analyses. Results In the training set, over a median follow-up of 29 months, 16.7% of patients had an estimated glomerular filtration rate (eGFR) of < 60 ml/min/1.73m2 at the last follow-up, and 25% had hydronephrosis or required prolonged double-J stents. A prognostic score was developed by assigning 1, 1, and 2 points for peripheral CD19+ B cells <9.3%, serum creatinine (sCr) ≥120 μmol/l, and no response at 6 months, respectively. A score of ≥ 2 for predicting irreversible renal dysfunction had sensitivity and specificity of 100% and 92%, respectively. In the validation set, 21.7% of patients suffered from irreversible renal dysfunction. The sensitivity and specificity for predicting irreversible renal dysfunction were 100% and 94.4%, respectively. Conclusions A prognostic score based on factors including CD19+ B cells <9.3% and sCr ≥120 μmol/l at baseline, and no response at 6 months, is suitable for predicting irreversible renal dysfunction in iRPF.

中文翻译：

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特发性腹膜后纤维化患者不可逆性肾功能不全的预测因子


目的 特发性腹膜后纤维化 （iRPF） 可导致不可逆的肾损伤。本研究旨在探讨 iRPF 患者不可逆肾功能不全的预测因子。方法 选取 2010年1月至 2022年9月在复旦大学附属中山医院收治 83 例新诊断 iRPF 患者，其中训练集 60 例，验证集 23 例。专科医生定期联系他们或通过门诊检查对他们进行随访，专科医生记录他们的病情和治疗进展。使用单变量和多变量回归、logistic 模型和受试者工作曲线分析确定不可逆性肾功能不全的预测因子。结果 在训练集中，在中位 29 个月的随访中，16.7% 的患者在最后一次随访时估计肾小球滤过率 （eGFR） 为 < 60 ml/min/1.73m2,25% 患有肾积水或需要延长双 J 支架。通过分别为外周 CD19+ B 细胞 <9.3% 、血清肌酐 （sCr） ≥120 μmol/l，和 6 个月无反应分配 1 、 1 和 2 分来制定预后评分。预测不可逆性肾功能不全的 ≥ 分的敏感性和特异性分别为 100% 和 92%。在验证集中，21.7% 的患者患有不可逆的肾功能不全。预测不可逆性肾功能不全的敏感性和特异性分别为 100% 和 94.4%。结论 基线时基于 CD19+ B 细胞 <9.3% 和 sCr ≥120 μmol/l 等因素的预后评分，6 个月无反应，适合预测 iRPF 不可逆性肾功能不全。