Bilateral ureteral stenosis as a complication of Henoch–Schönlein vasculitis
Bilateral ureteral stenosis as a complication of Henoch–Schönlein vasculitis
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双侧输尿管狭窄是过敏性血管炎的并发症
DOI：
10.3109/s10165-008-0063-y
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发表时间：
2008
影响因子：
2.2
通讯作者：
N. Kashihara
中科院分区：
文献类型：
--
作者：
C. Ihoriya;Y. Morita;T. Tokura;Kengo Kidokoro;N. Komai;Tamaki Sasaki;N. Kashihara
我们先前曾报告一例年轻皮肌炎患者发生双侧输尿管狭窄[1]。虽然罕见，但输尿管受累可存在于各种形式的系统性小血管炎中[2]。然而，这种并发症可能被风湿病学家忽视。事实上，在风湿病学文献中只有少数关于这种并发症的报道。在此我们报告另一例过敏性血管炎患者双侧输尿管狭窄。一名66岁男性于2007年1月因肾衰竭加重转诊至我院。他于2006年12月因肉眼血尿而被另一家医院收治。他报告说，在过去几个月里，他没有任何异常的体征或症状。在该医院体检发现胸部和腹部正常，没有皮疹和紫癜。实验室检查结果包括白细胞计数为17，460/11，C反应蛋白（CRP）为10.1 mg/ dl，血清肌酐为1.95 mg/dl，血尿素氮为18 mg/dl。计算机断层扫描显示双侧肾积水。静脉肾盂造影显示左输尿管下段完全阻塞，右输尿管下段严重狭窄。膀胱内内窥镜检查显示膀胱粘膜无异常。由于明显的肾功能不全，将支架插入右侧狭窄的输尿管。经皮肾造瘘术治疗左肾积水（图1）。然而，血清肌酐进一步升高，他被转诊到我们的诊所接受进一步治疗。我们诊所的实验室检查显示白细胞计数为9，680/11，红细胞计数为282 9 10/11，血小板计数为27.5 9 10/11，血红蛋白为8.1 g/dl，CRP为7.30 mg/dl（正常值为0.30），血清肌酐为5.32 mg/dl，血尿素氮为28 mg/dl。血清学检查为阴性的cryocellulin，抗核抗体，抗神经细胞胞浆抗体。血清补体（C3和C4）浓度正常。IgG和IgM水平均正常，但伊加水平升高至998.3 mg/dl。凝血酶原时间（PT）和活化部分凝血活酶时间（APTT）正常。高倍视野下尿分析显示大量红细胞伴颗粒和白色细胞管型。尿蛋白呈阳性，为2.06 g/天。进行肾活检，组织学显示坏死性肾小球肾炎伴细胞新月体（图2）。发现5个肾小球伴全球硬化。在其余9个肾小球中的4个中发现了细胞新月体。免疫荧光组织化学显示肾小球系膜区伊加呈细颗粒状沉积。免疫复合物的存款也证实了电子显微镜。患者被诊断为Henoch-Schönlein血管炎。他接受了30 mg/天泼尼松龙治疗，导致CRP快速正常化和血清肌酐水平改善。然而，开始类固醇治疗两周后，患者出现大量黑便。红细胞计数和血红蛋白分别降低至173 9 10/11和5.5 g/dl。血小板计数为15.4 9 10/11。PT和APTT在10.0和21.0 s时无延长;伊堀屋Morita（&）T.德仓湾基多科罗湾驹井佐佐木Kashihara Division of Nephrology and Rheumatology，Department of Internal Medicine，川崎Medical School，577 Matsushima，Kurashiki，冈山701-0192，Japan电子邮件：morita@med.kawasaki-m.ac.jp
We reported previously on a case of a young adult with dermatomyositis who developed bilateral ureteral stenosis [1]. Although rare, ureteral involvement can be present in various forms of systemic small-vessel vasculitis [2]. However, this complication can be overlooked by rheumatologists. Indeed, there are only a few reports of this complication in the rheumatology literature. Here we report on another case of bilateral ureteral stenosis in a patient with Henoch–Schönlein vasculitis. A 66-year-old male was referred to our clinic in January 2007 for aggravation of renal failure. He was admitted to another hospital in December 2006 for macrohematuria. He reported that he hadn’t had any abnormal signs or symptoms during the last few months. Physical examination in that hospital revealed normal chest and abdomen and no skin rash or purpura. Laboratory findings included leukocyte count of 17,460/ll, C-reactive protein (CRP) 10.1 mg/ dl, serum creatinine 1.95 mg/dl, and blood urea nitrogen of 18 mg/dl. A computed tomographic scan demonstrated bilateral hydronephrosis. Intravenous pyelography revealed complete obstruction in the left lower ureter, and severe stenosis in the right lower ureter. Intracystic endoscopy showed no abnormalities in the bladder mucosa. Due to the evident renal dysfunction, a stent was inserted into the right stenotic ureter. Percutaneous nephrostomy was performed for hydronephrosis of the left kidney (Fig. 1). However, serum creatinine increased further, and he was referred to our clinic for further management. Laboratory tests at our clinic showed a leukocyte count of 9,680/ll, an erythrocyte count of 282 9 10/ll, a platelet count of 27.5 9 10/ll, hemoglobin 8.1 g/dl, CRP 7.30 mg/dl (normal \0.30), serum creatinine 5.32 mg/dl, and blood urea nitrogen 28 mg/dl. Serological tests were negative for cryoglobulin, antinuclear antibody, and antineutrophil cytoplasmic antibodies. Serum concentrations of complements (C3 and C4) were normal. The levels of both IgG and IgM were normal, but IgA levels were increased to 998.3 mg/dl. Prothrombin time (PT) and activated partial thromboplastin time (APTT) were normal. Urinalysis showed numerous red blood cells with granular and white cell casts under high-power field. Urine protein was positive at 2.06 g/day. Renal biopsy was performed and the histology showed necrotizing glomerulonephritis with cellular crescents (Fig. 2). Five glomeruli with global sclerosis were noted. Cellular crescents were found in four out of the remaining nine glomeruli. Immunofluorescence histochemistry showed fine granular deposition of IgA in the paramesangial areas. Immune-complex deposits were also confirmed by electron microscopy. The patient was diagnosed as having Henoch–Schönlein vasculitis. He was treated with 30 mg/day prednisolone, which resulted in a rapid normalization of CRP and improvement in serum creatinine levels. However, two weeks after starting the steroid therapy, the patient developed massive melena. Erythrocyte count and hemoglobin were decreased at 173 9 10/ll and 5.5 g/dl, respectively. Platelet count was 15.4 9 10/ll. PT and APTT were not prolonged at 10.0 and 21.0 s, C. Ihoriya Y. Morita (&) T. Tokura K. Kidokoro N. Komai T. Sasaki N. Kashihara Division of Nephrology and Rheumatology, Department of Internal Medicine, Kawasaki Medical School, 577 Matsushima, Kurashiki, Okayama 701-0192, Japan e-mail: morita@med.kawasaki-m.ac.jp