Andrade Joana, Freitas André, Costa Susana, Baptista Rui
Internal Medicine Department, Centro Hospitalar Tondela Viseu EPE, Viseu, Portugal
Cardiology Department, Centro Hospitalar e Universitario de Coimbra EPE, Coimbra, Portugal.
BMJ Case Rep. 2021 Apr 14;14(4):e239550. doi: 10.1136/bcr-2020-239550.
Eosinophilic granulomatosis with polyangiitis is an antineutrophil cytoplasmic antibody (ANCA)-associated small vessel vasculitis with cardiac involvement in more than 60% of cases. Authors describe the case of a 48-year-old woman who presented with progressively worsening asthenia, dyspnoea and macular, non-painful, non-itchy cutaneous lesions. She had signs of congestion on clinical examination and a history of asthma and nasal polyps. Blood tests showed eosinophilia (11.2%), positive troponin I (9698 μg/L), elevated B-type natriuretic peptide (2047 pg/mL) and positive C reactive protein (6.68 mg/dL). Echocardiogram displayed moderate left ventricular enlargement, left ventricular ejection fraction of 28% and mild pericardial effusion. Levosimendan relieved the congestion. Additional testing confirmed positive antinuclear antibodies with ANCA-negative autoimmune pattern. Cardiac magnetic resonance showed severely depressed systolic function due to diffuse hypokinesia. Cardiac biopsy had intercellular oedema and eosinophilic infiltrate. Treatment with prednisolone and cyclophosphamide was started. This is a case of a rare disease presenting with life-threatening cardiac involvement.
嗜酸性肉芽肿性多血管炎是一种抗中性粒细胞胞浆抗体（ANCA）相关性小血管炎，超过60%的病例有心脏受累。作者描述了一名48岁女性的病例，该患者表现为进行性加重的乏力、呼吸困难以及黄斑、无痛、无瘙痒的皮肤病变。临床检查有充血体征，并有哮喘和鼻息肉病史。血液检查显示嗜酸性粒细胞增多（11.2%）、肌钙蛋白I阳性（9698μg/L）、B型利钠肽升高（2047pg/mL）和C反应蛋白阳性（6.68mg/dL）。超声心动图显示左心室中度扩大，左心室射血分数为28%，并有轻度心包积液。左西孟旦缓解了充血症状。进一步检查证实抗核抗体阳性，ANCA阴性自身免疫模式。心脏磁共振显示由于弥漫性运动减弱导致严重的收缩功能降低。心脏活检有细胞间水肿和嗜酸性粒细胞浸润。开始使用泼尼松龙和环磷酰胺治疗。这是一例罕见疾病，伴有危及生命的心脏受累。
