Ishiwatari Ayumi, Endo Mariko, Wakai Sachiko
Department of Nephrology, Okubo Hospital, 2-44-1 Kabuki-cho, Shinjuku-ku, Tokyo, 160-8488, Japan.
CEN Case Rep. 2018 Nov;7(2):301-306. doi: 10.1007/s13730-018-0347-9. Epub 2018 Jun 27.
Medium-vessel hemorrhage is a rare occurrence in ANCA-associated vasculitis, and has been previously described in only a few patients with microscopic polyangiitis. We report a case of renal hemorrhage in a patient with microscopic polyangiitis that was successfully managed by transcatheter arterial embolization of the active bleeding sites. The early clinical findings included necrotizing arteritis, as indicated by skin biopsy; rapidly progressive glomerulonephritis; mononeuritis multiplex; positive screening for myeloperoxidase-specific antineutrophil cytoplasmic antibody. Corticosteroid therapy was initiated. The patient's health deteriorated at 1 week, with rapidly progressing anemia. Computerized tomography identified a large, right-sided, perirenal hematoma, with active bleeding. Bleeding was successfully managed via segmental embolization of the renal artery. The patient was treated with steroid therapy and MZR, and subsequently underwent maintenance hemodialysis treatment for end-stage renal disease. Spontaneous renal hemorrhage is a rare but fatal clinical condition. A ruptured renal artery should be considered in a patient with microscopic polyangiitis, even in the absence of previous trauma and renal biopsy, when unexplained anemia or signs of shock occur.
中等血管出血在抗中性粒细胞胞浆抗体相关性血管炎中较为罕见,此前仅在少数显微镜下多血管炎患者中有所描述。我们报告了一例显微镜下多血管炎患者发生肾出血的病例,通过对活动性出血部位进行经导管动脉栓塞成功治疗。早期临床发现包括皮肤活检提示的坏死性动脉炎;快速进展性肾小球肾炎;多发性单神经炎;髓过氧化物酶特异性抗中性粒细胞胞浆抗体筛查呈阳性。开始使用糖皮质激素治疗。患者在第1周时健康状况恶化,贫血迅速进展。计算机断层扫描发现右侧肾周有一个大血肿,伴有活动性出血。通过肾动脉节段性栓塞成功控制了出血。患者接受了类固醇治疗和霉酚酸酯治疗,随后因终末期肾病接受维持性血液透析治疗。自发性肾出血是一种罕见但致命的临床情况。对于显微镜下多血管炎患者,即使没有既往创伤和肾活检,当出现无法解释的贫血或休克迹象时,也应考虑肾动脉破裂。