Abstract Body (Do not enter title and authors here): Description of Case
A 19-year-old female with history of granulomatosis with polyangiitis (GPA) presented to the emergency department after a syncopal episode at a concert. She reported a prodrome of feeling hot and dizzy prior to the event, followed by epigastric discomfort, nausea, and vomiting after regaining consciousness. She had been diagnosed with GPA one year prior to presentation based on serology and biopsy results. Because her disease was felt to be well-controlled on azathioprine, she had been tapered off of prednisone one month prior to presentation.
Initial vitals included blood pressure of 90/60 mmHg, and heart rate of 94 bpm. A 12-lead EKG revealed ST segment elevation in leads I, II and aVF with T wave inversions in the lateral leads (Figure 1). Initial bloodwork was notable for troponin I of 0.11ng/mL (upper limit of normal 0.034 ng/mL).
A diagnosis of inferior ST-elevation myocardial infarction was made. She was treated with oral aspirin and prasugrel load as well as intravenous heparin and taken urgently to the catheterization laboratory. Diagnostic coronary angiography was significant for single vessel coronary artery disease (CAD) of the right coronary artery (RCA), with acute complete occlusion of the mid-RCA (Figure 2A-B). She was treated successfully with primary percutaneous coronary intervention and implant of two overlapping drug eluting stents in the mid-RCA (Figure 2C).
Discussion
GPA is a systemic necrotizing vasculitis associated with antineutrophil cytoplasmic antibodies (ANCA) that predominantly affects small to medium sized vessels in the respiratory tract and kidneys. Cardiovascular manifestations in GPA are uncommon, but include pericarditis, myocarditis, and coronary arteritis. GPA can affect the coronary arteries either primarily through inflammatory changes in the arterial wall or via systemic inflammation leading to accelerated atherosclerosis. No standardized criteria exist to establish the diagnosis of coronary arteritis and to differentiate it from accelerated atherosclerosis. Long-term treatment of vasculitis patients with coronary involvement is also poorly supported by evidence. Optimization of cardiovascular risk factors is advocated as per standard of care, and vasculitis control may slow disease progression. This case not only raises awareness of cardiac complications in GPA, but highlights the current lack of evidence surrounding secondary prevention in young patients with vasculitis and cardiac complications.