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一例伴有坏死性新月体肾小球肾炎的 Churg-Strauss 综合征,伴有因血管痉挛引起的急性冠状动脉综合征。

A case of Churg-Strauss syndrome with necrotizing crescentic glomerulonephritis accompanied by acute coronary syndrome due to vasospasm.

机构信息

Division of Nephrology, Department of Medicine, Mitsui Memorial Hospital, Tokyo, Japan.

出版信息

Am J Kidney Dis. 2010 Aug;56(2):e5-9. doi: 10.1053/j.ajkd.2010.03.011.

DOI:10.1053/j.ajkd.2010.03.011
PMID:20537776
Abstract

We report a case of Churg-Strauss syndrome coexistent with coronary vasospasm and pauci-immune necrotizing crescentic glomerulonephritis. A 54-year-old man with bronchial asthma and allergic rhinitis was admitted to our hospital because of acute coronary syndrome. Angiography showed diffuse coronary artery spasm without anatomic stenosis. Acute coronary syndrome due to vasospasm was diagnosed. However, subsequent administration of vasodilators did not suppress angina symptoms. In addition, marked eosinophilia, eosinophilic pneumonitis, chronic sinusitis, pericardial effusion, and slight hematuria with red blood cell casts were detected. Although kidney function was normal, a kidney biopsy showed necrotizing crescentic glomerulonephritis with eosinophilic infiltration in both glomeruli and interstitium. With the diagnosis of Churg-Strauss syndrome, oral prednisolone at a dose of 60 mg/d was administered. Cardiac symptoms, pulmonary and sinonasal lesions, pericardial effusion, and urine sediment resolved rapidly. Six months later, a repeated kidney biopsy showed remarkable improvement and no eosinophilic infiltration. Coronary vasospasm with eosinophilia might be refractory to vasodilators and sensitive to corticosteroid therapy and often has been related to Churg-Strauss syndrome. Slight abnormalities in urine sediment can be the clue to the diagnosis of severe kidney involvement of Churg-Strauss syndrome.

摘要

我们报告了一例 Churg-Strauss 综合征合并冠状动脉痉挛和寡免疫坏死性新月体肾小球肾炎的病例。一名 54 岁男性,患有支气管哮喘和过敏性鼻炎,因急性冠状动脉综合征入住我院。血管造影显示弥漫性冠状动脉痉挛,无解剖学狭窄。诊断为血管痉挛性急性冠状动脉综合征。然而,随后使用血管扩张剂并未抑制心绞痛症状。此外,还检测到明显的嗜酸性粒细胞增多、嗜酸性肺炎、慢性鼻窦炎、心包积液和轻度血尿伴红细胞管型。尽管肾功能正常,但肾脏活检显示坏死性新月体肾小球肾炎,肾小球和间质均有嗜酸性粒细胞浸润。诊断为 Churg-Strauss 综合征后,给予 60mg/d 的口服泼尼松治疗。心脏症状、肺部和鼻旁窦病变、心包积液和尿沉渣迅速得到缓解。6 个月后,重复肾活检显示显著改善,无嗜酸性粒细胞浸润。伴有嗜酸性粒细胞增多的冠状动脉痉挛可能对血管扩张剂耐药,对皮质类固醇治疗敏感,常与 Churg-Strauss 综合征有关。轻微的尿沉渣异常可能是 Churg-Strauss 综合征严重肾脏受累的诊断线索。

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Immunosuppressive therapy ameliorates refractory vasospastic angina, severe pulmonary hypertension, and bronchiolitis in a patient with eosinophilic granulomatosis with polyangiitis: a case report.
免疫抑制疗法改善了一名嗜酸性肉芽肿性多血管炎患者的难治性血管痉挛性心绞痛、重度肺动脉高压和细支气管炎:一例报告
Eur Heart J Case Rep. 2018 Apr 25;2(2):yty050. doi: 10.1093/ehjcr/yty050. eCollection 2018 Jun.