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北京大学学报（医学版） ›› 2020, Vol. 52 ›› Issue (6): 1069-1074. doi: 10.19723/j.issn.1671-167X.2020.06.013

• 论著 • 上一篇    下一篇

腹膜后纤维化致肾盂积水的临床分析:附17例报道

刘世博1,高辉2,冯元春3,李静4,张彤5,万利6,刘燕鹰7,李胜光2,罗成华1,△(),张学武7,△()   

  1. 1. 北京大学国际医院 腹膜后肿瘤外科
    2. 北京大学国际医院 风湿免疫科
    3. 北京大学国际医院 放射科
    4. 北京大学国际医院 检验科
    5. 北京大学国际医院 病理科
    6. 北京大学国际医院 肾内科,北京 102206
    7. 北京大学人民医院风湿免疫科,北京 100044
  • 收稿日期:2020-07-31 出版日期:2020-12-18 发布日期:2020-12-13
  • 通讯作者: 罗成华,张学武 E-mail:luochenghua@pkuih.edu.cn;xuewulore@163.com
  • 基金资助:
    北京大学国际医院院内科研基金(YN2019QN02)

Clinical features of hydronephrosis induced by retroperitoneal fibrosis: 17 cases reports

Shi-bo LIU1,Hui GAO2,Yuan-chun FENG3,Jing LI4,Tong ZHANG5,Li WAN6,Yan-ying LIU7,Sheng-guang LI2,Cheng-hua LUO1,△(),Xue-wu ZHANG7,△()   

  1. 1. Department of Retroperitoneal Tumor Surgery
    2.Department of Rheumatology and Immunology
    3. Department of Radiology
    4. Department of Laboratory Medicine
    5. Department of Pathology
    6. Department of Nephrology, Peking University International Hospital, Beijing 102206, China
    7. Department of Rheumatology and Immunology, Peking University People’s Hospital, Beijing 100044, China
  • Received:2020-07-31 Online:2020-12-18 Published:2020-12-13
  • Contact: Cheng-hua LUO,Xue-wu ZHANG E-mail:luochenghua@pkuih.edu.cn;xuewulore@163.com
  • Supported by:
    Peking University International Hospital Research Grant(YN2019QN02)

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摘要：

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。

关键词: 腹膜后纤维化, 肾盂积水, 糖皮质激素类, 外科手术, 预后

Abstract:

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.

Key words: Retroperitoneal fibrosis, Hydronephrosis, Glucocorticoids, Surgical procedures, operative, Prognosis

中图分类号: 

  • R656.5

表1

17例RPF患者临床特点"

Clinical Features n (%)
Gender
Male 12 (70.6)
Female 5 (29.4)
Symptoms
Back pain 9 (52.9)
Abdominal pain 6 (35.3)
Oliguria 2 (11.8)
Lower limb edema 3 (17.6)
Diarrhea 1 (5.9)
Constipation 1 (5.9)
Laboratory studies
Increased CRP 13 (76.5)
Increased ESR 13 (76.5)
Increased IgG4 5 (29.4)
Increased IgG (n=13) 4 (30.8)
Increased IgM (n=13) 0
Increased IgE (n=13) 4 (30.8)
Increased IgA (n=13) 1 (7.7)
Decreased C3 (n=13) 1 (7.7)
Decreased C4 (n=13) 1 (7.7)
Hydronephrosis
Left hydronephrosis 8 (47.1)
Right hydronephrosis 1 (5.9)
Bilateral hydronephrosis 8 (47.1)
Radiographic class
Ⅰ+Ⅱ+Ⅲ 14 (82.4)
Ⅰ+Ⅱ+Ⅲ+Ⅳ 3 (17.6)
Pathological features (n=12)
<10 IgG4+ plasma cells/HPF 6 (50.0)
10-30 IgG4+ plasma cells/HPF 2 (16.7)
>30 IgG4+ plasma cells/HPF 4 (33.3)
IgG4+/IgG+ plasma cell ratio >40% 4 (33.3)
Storiform-type fibrosis 3 (25.0)
Obliterative phlebitis 4 (33.3)

图1

ESR(A)、CRP(B)、IgG4(C)、IgG(D)、IgE(E)、IgA(F)指标治疗前后变化"

图2

腹膜后肿物上下径线(A)、左右径线(B)、前后径线(C)治疗前后变化"

图3

腹膜后肿物治疗前后CT图像变化(A,治疗前;B,治疗后)"

[1] Uibu T, Oksa P, Auvinen A, et al. Asbestos exposure as a risk factor for retroperitoneal fibrosis[J]. Lancet, 2004,363(9419):1422-1426.
pmid: 15121404
[2] Vaglio A, Palmisano A, Alberici F, et al. Prednisone versus tamoxifen in patients with idiopathic retroperitoneal fibrosis: An open-label randomised controlled trial[J]. Lancet, 2011,378(9788):338-346.
doi: 10.1016/S0140-6736(11)60934-3 pmid: 21733570
[3] Scheel PJ Jr, Feeley N. Retroperitoneal fibrosis: the clinical, laboratory, and radiographic presentation[J]. Medicine (Baltimore), 2009,88(4):202-207.
doi: 10.1097/MD.0b013e3181afc439
[4] Deshpande V, Zen Y, Chan JK, et al. Consensus statement on the pathology of IgG4-related disease[J]. Mod Pathol, 2012,25(9):1181-1192.
doi: 10.1038/modpathol.2012.72 pmid: 22596100
[5] Ormond JK. Bilateral ureteral obstruction due to envelopment and compression by an inflammatory retroperitoneal process[J]. J Urol, 1948,59(6):1072-1079.
doi: 10.1016/s0022-5347(17)69482-5 pmid: 18858051
[6] Zen Y, Onodera M, Inoue D, et al. Retroperitoneal fibrosis: A clinicopathologic study with respect to immunoglobulin G4[J]. Am J Surg Pathol, 2009,33(12):1833-1839.
doi: 10.1097/pas.0b013e3181b72882 pmid: 19950407
[7] Rossi GM, Rocco R, Accorsi Buttini E, et al. Idiopathic retroperitoneal fibrosis and its overlap with IgG4-related disease[J]. Intern Emerg Med, 2017,12(3):287-299.
doi: 10.1007/s11739-016-1599-z pmid: 28070877
[8] Yamashita K, Haga H, Mikami Y, et al. Degree of IgG4+ plasma cell infiltration in retroperitoneal fibrosis with or without multifocal fibrosclerosis[J]. 2010,52(3):404-409.
[9] Khosroshahi A, Carruthers MN, Stone JH, et al. Rethinking Ormond's disease: "idiopathic" retroperitoneal fibrosis in the era of IgG4-related disease[J]. Medicine (Baltimore), 2013,92(2):82-91.
doi: 10.1097/MD.0b013e318289610f
[10] Caiafa RO, Vinuesa AS, Izquierdo RS, et al. Retroperitoneal fibrosis: Role of imaging in diagnosis and follow-up[J]. Radiographics, 2013,33(2):535-552.
doi: 10.1148/rg.332125085 pmid: 23479712
[11] George V, Tammisetti VS, Surabhi VR, et al. Chronic fibrosing conditions in abdominal imaging[J]. Radiographics, 2013,33(4):1053-1080.
doi: 10.1148/rg.334125081 pmid: 23842972
[12] Pelkmans LG, Aarnoudse AJ, Hendriksz TR, et al. Value of acute-phase reactants in monitoring disease activity and treatment response in idiopathic retroperitoneal fibrosis[J]. Nephrol Dial Transplant, 2012,27(7):2819-2825.
doi: 10.1093/ndt/gfr779 pmid: 22273666
[13] van Bommel EF, Siemes C, Hak LE, et al. Long-term renal and patient outcome in idiopathic retroperitoneal fibrosis treated with prednisone[J]. Am J Kidney Dis, 2007,49(5):615-625.
doi: 10.1053/j.ajkd.2007.02.268 pmid: 17472843
[14] Fry AC, Singh S, Gunda SS, et al. Successful use of steroids and ureteric stents in 24 patients with idiopathic retroperitoneal fibrosis: A retrospective study[J]. Nephron Clin Pract, 2008,108(3):c213-220.
doi: 10.1159/000119715 pmid: 18332635
[15] Alberici F, Palmisano A, Urban ML, et al. Methotrexate plus prednisone in patients with relapsing idiopathic retroperitoneal fibrosis[J]. Ann Rheum Dis, 2013,72(9):1584-1586.
doi: 10.1136/annrheumdis-2013-203545 pmid: 23696631
[16] Scheel PJ Jr, Feeley N, Sozio SM. Combined prednisone and mycophenolate mofetil treatment for retroperitoneal fibrosis: a case series[J]. Ann Intern Med, 2011,154(1):31-36.
doi: 10.7326/0003-4819-154-1-201101040-00005 pmid: 21200036
[17] Binder M, Uhl M, Wiech T, et al. Cyclophosphamide is a highly effective and safe induction therapy in chronic periaortitis: a long-term follow-up of 35 patients with chronic periaortitis[J]. Ann Rheum Dis, 2012,71(2):311-312.
doi: 10.1136/annrheumdis-2011-200148 pmid: 21859695
[18] Li KP, Zhu J, Zhang JL, et al. Idiopathic retroperitoneal fibrosis (RPF): Clinical features of 61 cases and literature review[J]. Clin Rheumatol, 2011,30(5):601-605.
doi: 10.1007/s10067-010-1580-6 pmid: 20957401
[19] Vaglio A, Maritati F. Idiopathic retroperitoneal fibrosis[J]. J Am Soc Nephrol, 2016,27(7):1880-1889.
doi: 10.1681/ASN.2015101110 pmid: 26860343
[20] Mertens S, Zeegers AG, Wertheimer PA, et al. Efficacy and complications of urinary drainage procedures in idiopathic retroperitoneal fibrosis complicated by extrinsic ureteral obstruction[J]. Int J Urol, 2014,21(3):283-288.
doi: 10.1111/iju.12234 pmid: 24033464
[21] Cristian S, Cristian M, Cristian P, et al. Management of idiopathic retroperitoneal fibrosis from the urologist's perspective[J]. Ther Adv Urol, 2015,7(2):85-99.
doi: 10.1177/1756287214565637 pmid: 25829952
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CN 11-4691/R
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