【摘要】：目的分析原發(fā)性腹膜后纖維化(RPF)患者的臨床特點(diǎn)、治療方法和預(yù)后,以提高對該疾病的認(rèn)識(shí)。方法回顧性分析2011—2016年在上海交通大學(xué)醫(yī)學(xué)院附屬瑞金醫(yī)院腎臟科住院并被診斷為原發(fā)性RPF的7例患者首診時(shí)的臨床表現(xiàn)、實(shí)驗(yàn)室檢查、影像學(xué)檢查、治療方案和預(yù)后等資料。結(jié)果 7例患者中,男6例、女1例;中位發(fā)病年齡為55歲;起病時(shí)出現(xiàn)多飲、多尿癥狀1例,腰腹疼痛2例,無任何癥狀但在體格檢查時(shí)發(fā)現(xiàn)異常4例。實(shí)驗(yàn)室檢查結(jié)果示,6例出現(xiàn)不同程度的腎功能不全、CRP水平升高;7例紅細(xì)胞沉降率升高。7例患者均進(jìn)行泌尿系統(tǒng)CT或MRI檢查,可見軟組織影包繞腹主動(dòng)脈、髂動(dòng)脈、輸尿管,部分患者甚至包繞腎動(dòng)靜脈或腹膜后其他組織,均符合RPF的診斷;其中4例行腹膜后腫塊活組織病理學(xué)檢查,結(jié)果均提示纖維組織增生,伴有淋巴細(xì)胞和漿細(xì)胞浸潤。3例因泌尿系統(tǒng)梗阻行雙J管植入術(shù),1例行腎盂造瘺術(shù),余3例均未進(jìn)行外科干預(yù);4例因血肌酐水平升高明顯而行中心靜脈插管血液透析治療;1例采用單純糖皮質(zhì)激素治療,6例采用糖皮質(zhì)激素聯(lián)合免疫抑制劑治療(1例先后采用糖皮質(zhì)激素聯(lián)合嗎替麥考酚酯、環(huán)磷酰胺、帕夫林等治療,但因療效不佳且伴有激素依賴而加用利妥昔單抗治療;余5例均采用糖皮質(zhì)激素聯(lián)合環(huán)磷酰胺治療)。隨訪3~56個(gè)月,中位隨訪時(shí)間為23.0個(gè)月。1例因環(huán)磷酰胺治療后骨髓抑制導(dǎo)致感染而死亡,其余患者未再出現(xiàn)梗阻癥狀,無患者進(jìn)展至終末期腎功能不全;其中因激素依賴、聯(lián)合多種免疫抑制劑治療無效、疾病反復(fù)復(fù)發(fā)而予利妥昔單抗治療的1例患者,在隨訪過程中逐步減少潑尼松用量后,患者腎功能穩(wěn)定,且無過敏、感染等并發(fā)癥發(fā)生。結(jié)論 RPF的臨床表現(xiàn)無明顯特異性,影像學(xué)檢查對診斷的價(jià)值較高。外科與內(nèi)科結(jié)合治療、糖皮質(zhì)激素聯(lián)合免疫抑制劑治療是RPF的常用治療方案,對于激素依賴、聯(lián)合多種免疫抑制劑治療無效、疾病反復(fù)復(fù)發(fā)的患者可予利妥昔單抗治療,但其療效與安全性尚需進(jìn)一步研究予以證實(shí)。
[Abstract]:Objective to analyze the clinical characteristics, treatment and prognosis of patients with primary retroperitoneal fibrosis (RPF) in order to improve the understanding of the disease. Methods from 2011 to 2016, 7 patients who were hospitalized in Ruijin Hospital of Medical College of Shanghai Jiaotong University and diagnosed as primary RPF were analyzed retrospectively on their clinical manifestations, laboratory examination and imaging examination. Treatment plan and prognosis. Results among the 7 patients, 6 were male and 1 female, the median age of onset was 55 years, polyuria occurred in 1 case, abdominal pain in 2 cases, no symptoms were found in 4 cases but abnormal in physical examination. The results of laboratory examination showed that 6 cases had different degree of renal insufficiency and the level of CRP increased. Seven patients were examined by CT or MRI in the urinary system. Soft tissue enveloping abdominal aorta, iliac artery, ureter, and even renal arteriovenous or retroperitoneal tissue were found in 7 patients. All of them were in accordance with the diagnosis of RPF. Among them, 4 cases underwent pathological examination of retroperitoneal mass, the results showed that fibrous tissue hyperplasia was accompanied by infiltration of lymphocytes and plasmacytes, 3 cases underwent double J tube implantation due to urinary obstruction, 1 case underwent pyelostomy, 1 case underwent pyelostomy, and 1 case underwent pyelostomy. No surgical intervention was carried out in the other 3 cases. 4 cases were treated with hemodialysis by central venous catheterization because of the obvious increase of serum creatinine level. One case was treated with glucocorticoid alone, 6 cases were treated with glucocorticoid combined with immunosuppressive agents (1 case was treated with corticosteroid combined with mexicol ester, cyclophosphamide, Pavlin, etc.), and 1 case was treated with corticosteroids combined with immunosuppressive agents. However, Rituximab was used in addition to Rituximab because of poor curative effect and hormone dependence. The other 5 cases were treated with glucocorticoid combined with cyclophosphamide. The median follow-up time was 23.0 months. One patient died of infection caused by myelosuppression after cyclophosphamide treatment, and the other patients had no obstruction symptoms and no progression to end-stage renal failure. One patient was treated with rituximab because of hormone dependence, combined with multiple immunosuppressive agents and repeated recurrence of the disease. After gradually reducing the dosage of prednisone during the follow-up, the renal function of the patient was stable and there was no allergy. Complications such as infection occur. Conclusion the clinical manifestation of RPF has no obvious specificity, and imaging examination is of great value in diagnosis. Surgery combined with internal medicine, glucocorticoid combined with immunosuppressive therapy is a common treatment for RPF, for hormone dependence, combined with multiple immunosuppressive therapy, patients with recurrent disease can be treated with rituximab. But its curative effect and safety need further research to confirm.
【作者單位】： 上海交通大學(xué)醫(yī)學(xué)院附屬瑞金醫(yī)院腎臟科;上海交通大學(xué)醫(yī)學(xué)院附屬瑞金醫(yī)院放射科;
【分類號(hào)】：R572.2

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