摘要:
背景:1951年Churg 和 Strauss首先描述了名為嗜酸性肉芽腫性多動脈炎(EGPA) 的臨床情況,表現為哮喘、鼻息肉、鼻竇炎、高嗜酸細胞及其器官浸潤和壞死性血管炎。這類患者分類為ANCA相關性血管炎,但其ANCA 常為陰性,患者常表現為心肌受累。使用糖皮質激素治療後患者的長期預後顯著改善,然而其心肌受累仍然是EGPA患者死亡的首要原因。
病例報告:一名53歲的男性既往有哮喘和鼻息肉病史,此次表現為急性胸痛伴有肌鈣蛋白升高;患者進行了經皮冠脈幹預治療。發現患者左心室肥厚。20天後患者左右心室心肌厚度均明顯增加。 進一步的評估顯示患者存在高嗜酸細胞血症及鼻粘膜嗜酸細胞增加,證實了患者EGPA的診斷。他存在急性血管炎跡象,包括體重下降、勞累、顱內出血和血清肌酐增加。經過6天糖皮質激素治療後,患者心肌恢複到最初厚度。
結論:EGPA患者快速和顯著的心肌增厚並不常見,但是確實是可以發生的。EGPA的心肌增厚可以使用糖皮質激素快速逆轉,其很可能是由水腫所致。
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.