摘要:

        背景:1951年Churg 和 Strauss首先描述了名为嗜酸性肉芽肿性多动脉炎(EGPA) 的临床情况，表现为哮喘、鼻息肉、鼻窦炎、高嗜酸细胞及其器官浸润和坏死性血管炎。这类患者分类为ANCA相关性血管炎，但其ANCA 常为阴性，患者常表现为心肌受累。使用糖皮质激素治疗后患者的长期预后显著改善，然而其心肌受累仍然是EGPA患者死亡的首要原因。

        病例报告:一名53岁的男性既往有哮喘和鼻息肉病史，此次表现为急性胸痛伴有肌钙蛋白升高;患者进行了经皮冠脉干预治疗。发现患者左心室肥厚。20天后患者左右心室心肌厚度均明显增加。 进一步的评估显示患者存在高嗜酸细胞血症及鼻粘膜嗜酸细胞增加，证实了患者EGPA的诊断。他存在急性血管炎迹象，包括体重下降、劳累、颅内出血和血清肌酐增加。经过6天糖皮质激素治疗后，患者心肌恢复到最初厚度。

        结论:EGPA患者快速和显著的心肌增厚并不常见，但是确实是可以发生的。EGPA的心肌增厚可以使用糖皮质激素快速逆转，其很可能是由水肿所致。

        附原文：

        Abstract BACKGROUND:In 1951 Churg andStrauss first described the clinical condition now known as eosinophilic granulomatosiswith polyangiitis (EGPA), characterized by asthma, nasal polyposis,rhinosinusitis, hypereosinophilia with organ infiltration, andnecrotizingvasculitis. It is classified as an antineutrophil cytoplasmicantibody (ANCA) associatedvasculitis, but ANCA negativity is common andmore frequently encountered in EGPA with myocardial involvement. Long-termsurvival has substantially improved with corticosteroid treatment butmyocardial involvement is still the leading cause of death in EGPA.CASE PRESENTATION:A53-year old man with a history of asthma and nasal polyposis presented withacute chest pain and elevated troponin; a percutaneous coronary interventionwas performed. The left ventricle was described as hypertrophic. After20days the myocardium had markedly increased in thickness of both theright and left ventricle. Evaluation revealed hypereosinophilia in the bloodand nasal mucosal tissue, which confirmed the diagnosis of EGPA. He presentedwith signs of activevasculitisincluding weight loss, tiredness,intracerebral hemorrhage, and increasing serum creatinine. After 6 days ofcorticosteroid treatment, the myocardium returned to its initial thickness.CONCLUSION:Rapidand marked thickening of the myocardium is not frequently reported but mayoccur in EGPA. Myocardial thickening in EGPA can be quickly reversed bycorticosteroids, and is most likely caused by edema.

        引自：Mattsson G,Magnusson P.Eosinophilic granulomatosis with polyangiitis: myocardial thickeningreversed by corticosteroids.BMC Cardiovasc Disord.2017 Dec 20;17(1):299. doi:10.1186/s12872-017-0734-8.