目的:分析腹膜后纤维化(retroperitoneal fibrosis,RPF)的临床特点,重点探讨通过药物联合外科干预治疗RPF及其所致肾盂积水的疗效及转归。方法:对北京大学国际医院2016年5月至2019年12月诊治的17例RPF合并肾盂积水患者的临床资料和转归进行回顾性分析。结果:17例患者中男12例,女5例,年龄38~71岁,中位年龄56(53,65)岁,中位病程4.00(0.83,8.00)个月。常见的临床表现为腰痛(9例)、腹痛(6例)、少尿(2例)以及下肢水肿(3例)。入组患者中,合并左侧肾盂积水8例、右侧1例、双侧8例。17例患者的血液样本检查发现,C反应蛋白(C-reactive protein,CRP)升高13例(76.5%), 红细胞沉降率(erythrocyte sedimentation rate,ESR)升高13例(76.5%),免疫球蛋白G亚型4(immunoglobin G4 subtype, IgG4)升高5例(29.4%);进行血免疫球蛋白(immunoglobin, Ig)亚型分析的13例患者中,IgG升高4例(30.8%), IgE升高4例(30.8%), IgA升高1例(7.7%)。有12例患者接受了病理活检,病理组织中的IgG4表达量高低不一,6例表达量<10/高倍视野(high power field,HPF)或不表达(50.0%), 2例为10~30/HPF(16.7%), 4例>30/HPF(33.3%),3例患者可诊断为IgG4相关性RPF。17例患者中,13例在接受药物治疗前放置输尿管支架,其中4例因药物治疗时间尚短,未到评估拔管时机,仍需继续随访;余9例患者平均置管时间(6.7±3.0)个月,包括6例在随访期间因药物治疗梗阻好转拔除支架,3例患者则因梗阻难以解除,拟行药物治疗前行输尿管松解术,术后顺利拔除支架。4例患者虽然存在单侧肾盂积水,但由于病变较小,梗阻轻,未放置输尿管支架,其中2例仅进行药物治疗后输尿管梗阻便得以解除,另外2例患者在接受药物治疗后失访。10例具有完整随访资料的患者中位随访时间为5(3,13)个月,ESR、CRP、IgG4、IgG、IgE、IgA治疗前分别为54.0(36.3,98.5) mm/h、26.8(8.7,53.0) mg/L、1.34(0.55,3.36) g/L、16.3(13.0,21.1) g/L、40.5(31.4,203.0) IU/mL、2.51(1.82,3.25) g/L,治疗后分别下降了38.5(23.5,54.3) mm/h(P<0.01)、23.0(5.5,52.0) mg/L(P<0.05)、0.92(0.40,2.85) g/L(P<0.01)、6.5(1.7,9.1) g/L(P<0.05)、23.7(4.8,162.0) IU/mL(P<0.05)、0.77(0.32,1.26) g/L(P<0.05)。对比治疗前后影像学资料发现RPF肿物较治疗前显著缩小,以上患者在治疗后输尿管梗阻均得以解除。结论:RPF起病隐匿,早期无特异性临床表现。以激素治疗为主并根据病情选择联用免疫抑制剂,同时积极通过外科手段解除输尿管梗阻的综合治疗方案可改善患者临床症状,取得确切疗效,改善预后。

Objective: To investigate the clinical features and outcome of hydronephrosis induced by retroperitoneal fibrosis (RPF), and to evaluate the effect of corticosteroid based therapy combined with surgical intervention of ureteral obstruction. Methods: A total of 17 RPF patients with hydronephrosis hospitalized in Peking University International Hospital from May 2016 to December 2019 were analyzed retrospectively. Results: The median age was 56 (53, 65) years, the male to female ratio was 2.4:1, and the disease duration was 4.00 (0.83, 8.00) months. The initial symptoms included back pain (9 cases), abdominal pain (6 cases), oliguria (2 cases) and lower limb edema (3 cases). Eight patients presented left hydronephrosis, 1 right hydronephrosis and 8 bilateral hydronephrosis. C-reactive protein (CRP) and erythrocyte sedimentation rate (ESR) were both elevated in 13 patients (76.5%, n=17). Immunoglobin (Ig) G4 increased in 5 cases (29.4%, n=17). IgG, IgE and IgA increased in 4 cases (30.8%, n=13), 4 cases (30.8%, n=13) and 1 case (7.7%, n=13), respectively. Among 12 patients who underwent biopsy, 3 patients were diagnosed with IgG4-relate disease. The level of IgG4 in the tissues varied, 6 cases expressed less than 10 per high power field (HPF) or no expression (50.0%). Only 2 cases expressed 10-30/HPF (16.7%), and 4 cases revealed more than 30/HPF (33.3%). Among the 17 patients with ureteral obstruction, no urinary drainage procedure was needed in 4 patients who had mild ureteral obstruction, whereas, ureteral stenting was carried out in the other 13 cases before drug treatment. Time was too short to evaluate the effect of urinary drainage procedures in 4 patients. For the rest, ureterolysis had to be performed in 3 cases after failed ureteral stent insertion. Successful drain removal was accomplished in all of these 9 patients and the mean time to drain removal was (6.7±3.0) months. In addition, 10 patients had complete medical records after an average follow-up time of 5 (3-13) months. Levels of ESR, CRP, IgG4, IgG, IgE, IgA were 54.0 (36.3, 98.5) mm/h, 26.8 (8.7, 53.0) mg/L, 1.34 (0.55, 3.36) g/L, 16.3 (13.0, 21.1) g/L, 40.5 (31.4, 203.0) IU/mL, 2.51 (1.82, 3.25) g/L at baseline, which all decreased predominantly after treatment. ESR, CRP, IgG4, IgG, IgE and IgA dropped by 38.5 (23.5, 54.3) mm/h (P<0.01), 23.0 (5.5, 52.0) mg/L (P<0.05), 0.92 (0.40, 2.85) g/L (P<0.01), 6.5 (1.7, 9.1) g/L (P<0.05), 23.7 (4.8, 162.0) IU/mL (P<0.05) and 0.77 (0.32, 1.26) g/L (P<0.05), respectively. Size of mass measured by CT/MRI imaging became smaller significantly and hydronephrosis relieved. Conclusion: Onset of RPF is insidious and lack of specific initial symptoms. Corticosteroid based therapy combined with surgical intervention of relieving obstruction is effective.