Department of Internal Medicine/Dutch National Center of Expertise Retroperitoneal Fibrosis, Albert Schweitzer Hospital, PO Box 444, NL-3300 AK, Dordrecht, The Netherlands.
Department of Radiology, Albert Schweitzer Hospital, Dordrecht, The Netherlands.
Int Urol Nephrol. 2022 Oct;54(10):2663-2671. doi: 10.1007/s11255-022-03196-3. Epub 2022 Mar 31.
Idiopathic retroperitoneal fibrosis (iRPF) is a rare chronic fibro-inflammatory disorder of unknown etiology. Activated T-helper cells, which shed soluble interleukin-2 receptor (sIL-2R) into the circulation, may play a pathogenetic role. Hence, measuring sIL-2R may be of value in monitoring disease activity and treatment response in iRPF patients.
We performed a prospective inception cohort study of 82 patients with untreated (re)active iRPF stratified by elevated (> 623 U/mL) or normal sIL-2R level at baseline and compared disease characteristics among these groups. Baseline and changes in sIL-2R levels following treatment with tamoxifen (TMX) or prednisone (PDN) were analyzed and related to treatment response.
Median sIL-2R level was 668 U/mL (IQR 502.8-827.5); 48 patients (59%) had elevated baseline sIL-2R levels. Patients with elevated sIL-2R presented with higher CRP (P = 0.049) and serum creatinine (sCr) levels (P < 0.001) and more often had hydroureteronephrosis (P = 0.01). There was an age and sex adjusted linear association between baseline sIL-2R and both CRP (P = 0.02) and sCr (P < 0.001). Baseline and serial levels of sIL-2R were predictive and concordant, respectively, with clinical response in patients treated with PDN. ROC curve analyses of sIL-2R on a continuous scale and PDN treatment success showed an AUC of 0.84. A serum sIL-2R cut-off value for PDN treatment success of ≤ 703 U/mL was found with a sensitivity of 100% and specificity of 72%.
Serial measurement of sIL-2R may be of value in monitoring disease activity and PDN treatment response in iRPF patients.
特发性腹膜后纤维化（iRPF）是一种罕见的病因不明的慢性纤维炎症性疾病。活化的辅助性 T 细胞（Th 细胞）会将可溶性白细胞介素 2 受体（sIL-2R）释放入血液循环，其可能在发病机制中发挥作用。因此，测量 sIL-2R 可能有助于监测 iRPF 患者的疾病活动度和治疗反应。
我们对 82 例未经治疗的（复发性）iRPF 患者进行了一项前瞻性起始队列研究，这些患者在基线时根据 sIL-2R 水平升高（>623 U/mL）或正常进行分层，并比较了这些组之间的疾病特征。分析了他莫昔芬（TMX）或泼尼松（PDN）治疗前后 sIL-2R 水平的变化，并将其与治疗反应相关联。
中位 sIL-2R 水平为 668 U/mL（IQR 502.8-827.5）；48 例（59%）患者基线 sIL-2R 水平升高。sIL-2R 水平升高的患者 CRP（P=0.049）和血清肌酐（sCr）水平（P<0.001）更高，且更常出现肾盂积水（P=0.01）。年龄和性别调整后，基线 sIL-2R 与 CRP（P=0.02）和 sCr（P<0.001）均呈线性相关。sIL-2R 基线和连续水平与接受 PDN 治疗的患者的临床反应均具有预测性和一致性。sIL-2R 连续尺度和 PDN 治疗成功的 ROC 曲线分析显示 AUC 为 0.84。发现 sIL-2R 血清截断值≤703 U/mL 可预测 PDN 治疗成功，其灵敏度为 100%，特异性为 72%。
连续测量 sIL-2R 可能有助于监测 iRPF 患者的疾病活动度和 PDN 治疗反应。
