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

Special Article - Heart Failure

Austin J Clin Cardiolog. 2021; 7(1): 1074.

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

Nashawi M¹*, Ahmed MS¹, Abualfoul M³, Lee LK², Ghali A¹ and Chilton RJ¹

¹Department of Medicine-Cardiology, University of Texas Health Science Center at San Antonio, USA

²Department of Internal Medicine, The University of Texas Health Science Center, USA

³Department of Internal Medicine, Methodist Dallas Medical Center, USA

*Corresponding author: Mouhamed Nashawi, Department of Medicine-Cardiology, University of Texas Health Science Center at San Antonio, 7703 Floyd Curl Dr., San Antonio, TX, 78229-3900, USA

Received: March 08, 2021; Accepted: March 26, 2021; Published: April 02, 2021


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.

Keywords: Vasculitis; Coronary; Myocardial infarction; Multi-vessel disease; Polyangiitis; Granulomatosis; Pr3


A significant proportion of triple-vessel disease cases can be attributed to Coronary Artery Disease (CAD) [1]. Underlying severe CAD is coronary atherosclerosis and a complex, multifactorial pathologic orchestra marked initially by the accumulation of Low- Density Lipoprotein (LDL) within the subendothelial space of affected vessels, which subsequently undergoes oxidation [2,3]. This Oxidized Low-Density Lipoprotein (oxLDL) stimulates macrophage infiltration, and the uptake of oxLDL results in the formation of foam cells, or the so called infamous “fatty streak” observable under microscopy. The degradation of foam cells results in the egress of their contents, which induce a cascade of smooth cell proliferation and an inflammatory cascade culminating in a fibroinflammatory lipid plaque, called an atheroma [4,5]. This focal thickening of the arterial tunica intima hampers vessel compliance in addition to promoting a stenotic phenotype [6]. Coronary vessel occlusion may lead to inadequate myocardial perfusion and subsequent angina if anatomical restriction is significant enough to warrant a physiologically significant dearth in myocardial oxygenation [7]. One notable consequence of this resultant ischemia is cellular death of cardiomyocytes in the setting of increased oxygen demand. In the context of symptomatic CAD, compensatory physiologic processes are insufficient to provide adequate perfusion to the myocardium and may invoke a Myocardial Infarction (MI). Moreover, the lesions accrued at the commencement of vascular insult results in vessel stiffness, which promotes a state of hypertension. The mechanical forces associated with pernicious blood flow dynamics may cause plaque disruption and subsequent thrombosis in this setting. Moreover, expansion of thrombi without complete dislodgement may in itself additionally obstruction myocardial perfusion and induce MI.

Systemic inflammatory syndromes, such as the family of vasculitides, have received increased credence regarding their role as a nidus or provocateur of coronary artery disease due to aberrant signaling pathways or repeated degranulation of cytotoxic products from the leukocytes associated with these syndromes [8]. While multifarious in their pathophysiology, these conditions share a central theme of triggering bouts of inflammation that overlap with the same mediators that participate in pathologic plaque formation [9]. Substantively, every stage of atherosclerosis is characterized by an inflammatory process. Even more interesting is that through inflammation of the vasculature, vasculopathies such as Granulomatosis with Polyangiitis (GPA) have the potential to disrupt the structural and physiologic architecture of the vascular endothelium the most preliminary event in pathologic cholesterol deposition. Within the literature, Kawasaki Disease and Takayasu’s arteritis are the classic vasculopathies associated with CAD [10-13]. However, there has been recent evidence that show that vasculitides not typically associated with CAD may also exhibit coronary artery complications ranging from mild fatty deposition to fulminant multivessel disease [14,15]. There is evidence that supports GPA as a trigger for these pathologic events through autoimmune provocation. In the case of GPA, it is also believed that Antineutrophil Cytoplasmic Antibodies (c-ANCA) promote neutrophil adherence to vascular endothelium with subsequent degranulation of cytotoxic contents serving as the focus for vasculitis, vessel insult, and subsequent cholesterol accumulation [16].

Although extremely rare per current reviews of the literature, mindfulness of the association between vasculitides such as GPA and heart disease are can be critical in delineating the clinical manifestations of patients with MI, CAD, and recent history of vasculopathy [17]. We report the case of NSTEMI and triple-vessel CAD in a middle-aged, adult male with a recent history of lower limb claudication, later worked up for GPA. Given the rarity of GPA to present with coronary involvement in the literature, reports of these encounters are useful in informing clinicians while holding value in guiding management of vasculitis with concomitant Cardiovascular Disease (CVD). Moreover, a commentary outlining the mutual pathways in GPA and CAD offers an opportunity to shape perspectives regarding autoimmune conditions and the cardiovascular disease risk associated with them by placing inflammation in the spotlight.

Case Presentation

A 55-year-old male with no significant past medical history arrived at the emergency department due to a 1 week history of sharp pain in his groin and buttocks region that worsened at night and when laying down, consistent with a picture of claudication. These episodes of sharp pain were substantial enough to cause the patient to awaken the patient from sleep. Of note, he works as a skilled laborer in the automotive industry and has reported intermittent cramping also in his groin and buttocks with radiation from his right thigh down to his calf that he attributed to work-related overuse injuries. The patient reported that cramping episodes were more frequent and severe in nature with physical exertion, prompting temporary cessation of activity until the pain subsided. The remainder of a review of systems was null for significant clinical findings. Initially, the patient used over-the-counter Nonsteroidal Anti-Inflammatory (NSAIDs) agents as needed to alleviate his pain, but they no longer were effective per the patient. Upon arrival, the patient was afebrile and hemodynamically stable with the only pertinent physical exam findings elicited being tenderness to palpation of the right lower quadrant of the abdomen. A query of past medical and surgical history was nonsignificant, as the patient has not sought medical counsel of any kind in roughly 20 years. Socially, the patient affirmed intermittent tobacco and cannabis use (quit tobacco roughly 12 years ago), and informed providers of a remote history of alcohol use disorder with complete sobriety reached over 20 years ago. His family history was unremarkable.

Computed Tomography Angiogram (CTA) with contrast of the abdominal aorta in addition to a CT study of the abdomen and pelvis without contrast were ordered to appraise the anatomy of the inciting limb. Appraisal of this image was significant for illdefined non-enhancing attenuation of the soft tissue surrounding the proximal right iliac artery just distal to the aortic bifurcation, without extravasation or extraluminal contrast deposition. Such findings suggest inflammatory changes about the proximal right iliac artery with prevailing diagnoses being focal vasculitis versus retroperitoneal fibrosis (Figure 1). Given clinical stability of and chronicity of symptoms without emergent aggravation, it was deemed that there was no indication for acute surgical intervention. It was decided that it would be appropriate for the patient to follow-up as an outpatient with vascular surgery pending primary care clearance.