Raghunathan Vikram, Pelcovits Aryeh, Gutman Daniel, Carino Gerardo
Department of Medicine, Brown University Warren Alpert Medical School, Providence, Rhode Island, USA.
BMJ Case Rep. 2017 Jun 29;2017:bcr-2017-220233. doi: 10.1136/bcr-2017-220233.
Granulomatosis with polyangiitis (GPA) is a systemic vasculitis characterised by necrotising inflammatory changes in small-sized and medium-sized vessels and granuloma formation. It most commonly involves the kidneys and respiratory tract, but it can present with widespread manifestations involving any organ system. Rarely, it causes coronary vasculitis which can precipitate a severe cardiomyopathy. Here, we report a patient who presented in cardiogenic shock requiring vasopressors and was found to have extensive myocardial ischaemia secondary to coronary vasculitis. Further investigation led to a diagnosis of GPA, and he responded to treatment with corticosteroids, cyclophosphamide and plasmapheresis.
肉芽肿性多血管炎(GPA)是一种系统性血管炎,其特征为中小血管的坏死性炎症改变和肉芽肿形成。它最常累及肾脏和呼吸道,但也可表现出累及任何器官系统的广泛症状。罕见情况下,它会导致冠状动脉血管炎,进而引发严重的心肌病。在此,我们报告一名出现心源性休克需要血管加压药治疗的患者,该患者被发现因冠状动脉血管炎继发广泛心肌缺血。进一步检查后诊断为GPA,他对皮质类固醇、环磷酰胺和血浆置换治疗有反应。