Ljubicić Divo, Matković Zinka, Piskac-Zivković Nevenka, Tudorić Neven
University Department of Medicine, Dubrava University Hospital, Zagreb, Croatia.
Acta Clin Croat. 2010 Sep;49(3):353-8.
Churg-Strauss syndrome (CSS) is a small-vessel necrotizing vasculitis typically characterized by asthma, lung infiltrates, extravascular necrotizing granulomas and hypereosinophilia. Cardiac disease is a major contributor to disease-related death in CSS. We describe a 38-year-old man with late-onset asthma, allergic rhinosinusitis, and high extravascular and peripheral blood eosinophilia, who presented with migratory pulmonary infiltrates and acute myopericarditis. Antineutrophilic cytoplasmic antibodies (ANCA) were negative. Early therapy with medium-dose methylprednisolone led to resolution of the pericardial effusion and significant clinical improvement. In the present case report, the importance of early recognition of CSS in patients with asthma and peripheral eosinophilia is discussed. Cardiac magnetic resonance imaging, besides electro- and echocardiography, may be helpful in early detection of cardiac involvement in CSS, enabling appropriate treatment aimed to prevent further disease progression and potentially fatal consequences.
变应性肉芽肿性血管炎(CSS)是一种小血管坏死性血管炎,典型特征为哮喘、肺部浸润、血管外坏死性肉芽肿和嗜酸性粒细胞增多。心脏疾病是CSS患者疾病相关死亡的主要原因。我们描述了一名38岁男性,患有迟发性哮喘、变应性鼻-鼻窦炎,血管外和外周血嗜酸性粒细胞增多,表现为游走性肺部浸润和急性心肌心包炎。抗中性粒细胞胞浆抗体(ANCA)阴性。早期使用中等剂量甲泼尼龙治疗使心包积液消退,临床症状显著改善。在本病例报告中,讨论了早期识别哮喘和外周嗜酸性粒细胞增多患者中CSS的重要性。除了心电图和超声心动图外,心脏磁共振成像可能有助于早期检测CSS患者的心脏受累情况,从而进行适当治疗以防止疾病进一步进展和潜在的致命后果。