Eosinophilic Coronary Periarteritis: A New Type of Coronary Arteritis as a Cause of Vasospastic Angina and Sudden Death

Abstract

Eosinophilic coronary periarteritis (ECPA) has been recently established as a new pathological entity and clinically showed a vasospastic angina and sudden cardiac death (SCD). The patients were relatively young (mainly 30 to 50 years old) and predominantly male. The characteristic clinical findings of this disease include a) vasospastic angina (Prinzmetal's variant angina) appearing usually from evening to early in the morning, b) all patients experienced SCD early in the morning, and c) allergy or history of allergy was hard to identify in the patients with this disease. Histological findings include a) eosinophilic inflammatory infiltration limited to the adventitia and periadventitial soft issue is recognized in the epicardial large coronary arteries, b) all 3 main coronary artery branches are affected, with the left anterior descending artery most frequently affected, c) medial smooth muscle cells of the affected coronary artery and both internal and elastic laminae are well preserved, d) fibrinoid necrosis or granuloma as seen in polyarteritis nodosa or allergic granulomatous angina are not found in or around the inflammatory area, and e) no findings of any type of vasculitis in any other tissues or organs (i.e., localized or non-systemic periarteritis). Spontaneous coronary artery dissection (SCAD) is frequently accompanied by eosinophilic inflammatory infiltration limited to the adventitia and periadventitial soft issue in the dissected portion of the epicardial coronary arteries, i.e., same as the findings of ECPA, and the patients usually die suddenly.A considerable number of patients with this disease will be included among patients suffering from vasospastic angina. Therefore, it is very important for cardiologists to make a precise diagnosis of and provide adequate treatment for this disease.