Harada Tamaki, Uzu Takashi, Namba Tomoko, Yamamoto Ryohei, Takahara Ken, Yamauchi Atsushi
Division of Nephrology, Department of Medicine, Osaka Rosai Hospital, 1179-3 Nagosone-cho, Sakai, Osaka 591-8025, Japan.
Clin Exp Nephrol. 2005 Jun;9(2):174-8. doi: 10.1007/s10157-005-0344-0.
On April 25, 2003, a 62-year-old Japanese man had been admitted to a hospital because of heavy proteinuria and elevated serum creatinine level, and purpura on the lower extremities. On May 15, 2003, he was referred to our hospital for evaluation and treatment. Serum immunoglobulin and complements were within normal ranges. Immune serology was negative for antinuclear antibody, antiglomerular basement membrane antibody, and antineutrophil cytoplasmic antibodies. Histological examination of a percutaneous renal biopsy specimen revealed that all of the glomeruli had severe crescent formation without deposits of immunoreactants. A diagnosis of antineutrophil cytoplasmic antibody-negative pauci-immune crescentic glomerulonephritis was made. The patient was treated with one cycle of steroid pulse therapy (1000 mg methylprednisolone daily, given on 3 consecutive days), and subsequently with prednisolone (60 mg/day). Despite this treatment, renal failure progressed rapidly and hemodialysis was started 1 month after the acute presentation. On May 30, 2003, he suddenly developed massive hematochezia. A technetium-targeted red-blood-cell scan suggested bleeding in the small intestine. On June 11, he presented with massive melena. A bleeding ulcer was found in the third part of the duodenum, and was treated successfully with endoscopy, using a heater probe. On June 19, he presented with massive hematochezia again. Mesenteric angiography revealed active bleeding from the iliac branch of the superior mesenteric artery. He was treated with continuous intraarterial vasopressin infusion by a catheter seated in the branch artery. The majority of patients with pauci-immune crescentic glomerulonephritis, one of the most common causes of rapidly progressive glomerulonephritis, have glomerular disease as part of a systemic vasculitis. Massive gastrointestinal bleeding, although rare, should be considered one of the serious complications in these patients.
2003年4月25日，一名62岁的日本男性因大量蛋白尿、血清肌酐水平升高以及下肢紫癜入住一家医院。2003年5月15日，他被转诊至我院进行评估和治疗。血清免疫球蛋白和补体在正常范围内。免疫血清学检查抗核抗体、抗肾小球基底膜抗体和抗中性粒细胞胞浆抗体均为阴性。经皮肾活检标本的组织学检查显示，所有肾小球均有严重的新月体形成，无免疫反应物沉积。诊断为抗中性粒细胞胞浆抗体阴性的寡免疫性新月体性肾小球肾炎。患者接受了一个周期的类固醇冲击治疗（每日1000毫克甲泼尼龙，连续3天给药），随后服用泼尼松龙（60毫克/天）。尽管进行了这种治疗，但肾衰竭仍迅速进展，急性发病1个月后开始进行血液透析。2003年5月30日，他突然出现大量便血。锝标记红细胞扫描提示小肠出血。6月11日，他出现大量黑便。在十二指肠第三部发现一个出血性溃疡，通过使用热探头的内镜检查成功进行了治疗。6月19日，他再次出现大量便血。肠系膜血管造影显示肠系膜上动脉髂支有活动性出血。通过置于分支动脉的导管持续动脉内输注血管加压素对他进行了治疗。寡免疫性新月体性肾小球肾炎是快速进展性肾小球肾炎最常见的病因之一，大多数患者的肾小球疾病是系统性血管炎的一部分。大量胃肠道出血虽然罕见，但应被视为这些患者的严重并发症之一。
