Myocardial Infarction and Three-Vessel Coronary Artery Disease as Presenting Features of Granulomatosis with Polyangiitis: A Case Report with Review of Literature

M. Nashawi, Ahmed Ms, M. Abualfoul, Lee Lk, A. Ghali, Chilton Rj
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Abstract

Granulomatosis with Polyangiitis (GPA) is a systemic, autoimmune disorder characterized by inflammatory insult and granulomatous processes in small and medium-sized vessels leading to various clinical presentations from underlying vasculitis. Underlying such inflammatory cascade is the overactivity of Antineutrophil Cytoplasmic Antibodies (c-ANCA) targeting serum Proteinase 3 (PR3), whose aberrant targeting classically modulates molecular signaling pathways leading to clinical manifestations of the Ear, Nose and Throat (ENT), in addition to renal impairment. Peripheral vessel involvement (i.e. limb vasculature) is not generally associated with GPA. With the exception of seldom reports in the literature, it is rare for GPA to present with coronary artery involvement. Moreover, reports of multi-vessel disease (e.g. triple-vessel disease) with GPA warranting Coronary Artery Bypass Graft (CABG) are lacking in such accounts. The latter with preceding iliac artery claudication makes such a presentation of GPA exceptionally novel and warrants contextual commentary regarding inflammation and Coronary Artery Disease (CAD). We report the case of a 55-year-old Caucasian male presenting with a 2 years history of right-sided groin cramping and an acute one-week history of claudication in the same area. After advised to follow up as an outpatient, this patient returned shortly thereafter to an acute care setting with hemoptysis and myocardial infarction worked up for GPA and triple-vessel disease. The patient was subsequently treated with immunosuppressive pharmacotherapy prior to CABG. We conducted a review of the literature underpinned in clinical and translational biology with a focus on the salient inflammatory pathways featured in both coronary artery disease and GPA.
心肌梗死和三支冠状动脉病变是肉芽肿病合并多血管炎的主要表现:1例报告并文献复习
肉芽肿病合并多血管炎(GPA)是一种系统性自身免疫性疾病,其特征是炎症性损伤和中小型血管肉芽肿过程,导致潜在血管炎的各种临床表现。这种炎症级联的基础是靶向血清蛋白酶3 (PR3)的抗中性粒细胞胞质抗体(c-ANCA)的过度活性,其异常靶向通常会调节分子信号通路,导致耳鼻喉(ENT)的临床表现,以及肾脏损害。周围血管受累(即肢体血管)通常与GPA无关。除了文献中很少报道外,GPA很少表现为冠状动脉受累。此外,有GPA的多血管疾病(如三血管疾病)需要冠状动脉旁路移植术(CABG)的报道在此类报道中缺乏。后者伴有髂动脉跛行,使得这种GPA的表现异常新颖,值得对炎症和冠状动脉疾病(CAD)进行背景评论。我们报告一个55岁的白人男性的情况下,提出了2年的历史,右侧腹股沟痉挛和急性一周的跛行史在同一区域。在建议作为门诊随访后,该患者因咯血和心肌梗死(GPA和三支血管疾病)返回急性护理室。患者随后在CABG前接受免疫抑制药物治疗。我们回顾了以临床和转化生物学为基础的文献,重点关注冠状动脉疾病和GPA的显著炎症途径。
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