Intensive Care Unit of Cardiology, Louis Pradel Hospital, Hospices civils de Lyon, boulevard Pinel, 69 317 Lyon cedex 04, France.
Heart Lung Circ. 2012 Mar;21(3):178-81. doi: 10.1016/j.hlc.2011.09.002. Epub 2011 Oct 1.
Churg-Strauss syndrome (CSS) is a multisystem disorder characterised by asthma, prominent peripheral blood eosinophilia, and vasculitis signs. We report the case of a 22 year-old man admitted to the intensive care unit for acute myocarditis complicated with cardiogenic shock. Eosinophilia, history of asthma, lung infiltrates, paranasal sinusitis, glomerulonephritis, and abdominal pain suggested the diagnosis of CSS. Cardiac MRI confirmed cardiac involvement with a diffuse subendocardial delayed enhancement of the left ventricular wall, and a left ventricular ejection fraction (LVEF) of 30%. Acute myocarditis was confirmed with myocardial biopsy. The patient was successfully treated with systemic corticosteroids, intravenous cyclophosphamide, vasopressor inotropes, intra-aortic balloon pump and mechanical ventilation, and was discharged 21 days later. One year after diagnosis, the patient was asymptomatic. The eosinophilic cell count was normal. Follow-up MRI at one year showed LVEF of 40% with persistent delayed enhancement. Cardiac involvement by CSS requires immediate therapy with corticosteroids and cyclophosphamide, which may allow recovery of the cardiac function.
变应性肉芽肿性血管炎（CSS）是一种多系统疾病，其特征为哮喘、外周血嗜酸性粒细胞显著增多和血管炎表现。我们报告了 1 例 22 岁男性患者，因急性心肌炎合并心源性休克而入住重症监护病房。嗜酸性粒细胞增多、哮喘史、肺部浸润、副鼻窦炎、肾小球肾炎和腹痛提示 CSS 的诊断。心脏 MRI 证实心脏受累，表现为左心室壁弥漫性心内膜下延迟强化，左心室射血分数（LVEF）为 30%。心肌活检证实急性心肌炎。患者接受全身皮质类固醇、静脉环磷酰胺、血管加压正性肌力药物、主动脉内球囊泵和机械通气治疗，21 天后出院。诊断 1 年后，患者无症状，嗜酸性粒细胞计数正常。1 年时的随访 MRI 显示 LVEF 为 40%，持续存在延迟强化。CSS 的心脏受累需要立即进行皮质类固醇和环磷酰胺治疗，这可能使心脏功能恢复。
