Eosinophilic coronary periarteritis (vasospastic angina and sudden death), a new type of coronary arteritis: report of seven autopsy cases and a review of the literature

Abstract

A previously reported autopsy case of eosinophilic coronary periarteritis (ECPA, or isolated eosinophilic coronary periarteritis, IECPA), and an additional six autopsy cases of ECPA are reported. In addition, another four autopsy cases of ECPA reported in the literature are discussed. Fifteen cases of ECPA with spontaneous coronary dissection (hematoma), which appeared in the literature from 1987 to 2011, are also reviewed. The characteristic clinico-pathological findings of ECPA are: (a) variant angina (Prinzmetal's vasospastic angina) appeared mainly from evening to early in the morning; (b) allergy or allergic history could be identified in only three of a total of 11 cases; (c) sudden unexpected death (sudden cardiac death) usually occurred early in the morning; (d) eosinophilic inflammation limited to the adventitia and periadventitial soft issue appeared in the epicardial large coronary arteries, chiefly in the left coronary anterior descending artery; (e) fibrinoid necrosis or granuloma could not be found in or around the inflammatory area; (f) no type of vasculitis could be found in any other tissues or organs (i.e., localized and non-systemic periarteritis); (g) ECPA was frequently accompanied by spontaneous coronary arterial dissection (SCAD) in the affected wall; and (h) ECPA without SCAD appeared mainly in men (male/female ratio was 8:3), while EPCA with SCAD appeared in almost all female cases (male/female ratio was 1:14). Although the etiology and pathogenesis are still unknown, we believe that ECPA (with or without SCAD) might be a distinct new type of coronary arteritis.

Eosinophilic cardiac disease: Molecular, clinical and imaging aspects

“…At autopsy, epicardial coronary arteries (from the main stem to their large branches) are grayish-white in colour and elastically hard. ECPA is reported to frequently be accompanied by spontaneous coronary arterial dissection in the affected wall, especially in women [64]. Histological examination shows a severe inflammatory infiltration, mainly eosinophils, of the adventitia and periadventitial soft tissue.…”

“…Almost all patients with ECPA have anginal pain for several weeks or years before sudden death [64]. Vasospastic angina mainly appears from the evening to the early morning (Prinzmetal's vasospastic angina), and patients usually die in the early morning.…”

“…Vasospastic angina mainly appears from the evening to the early morning (Prinzmetal's vasospastic angina), and patients usually die in the early morning. Despite the fact that bronchial asthma or drug allergy is generally not found, ECPA is believed to be a distinct coronary disease due to a localized inflammatory reaction rather than a limited form of a primary vasculitis [64,65]. At autopsy, epicardial coronary arteries (from the main stem to their large branches) are grayish-white in colour and elastically hard.…”

Response by Alexander et al to Letters Regarding Article, “A Shocking Development in a Young Male Athlete With Chest Pain”

“…The most distinguishing features of eosinophilic coronary periarteritis are appreciated by histology, which reveals eosinophilic infiltration isolated to the adventitia and vasa vasorum of the epicardial coronary arteries. 2 Thus, this diagnosis is difficult to confirm outside of autopsy.…”

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

“…However, the primary vasculitis is usually systemic, and almost all-coronary vasculitis appears as a manifestation of systemic vasculitis [1]. Recently, a special type of coronary arteritis, eosinophilic coronary periarteritis (ECPA), clinically showing vasospastic angina (Prinzmetal's variant angina [2]) and SCD was established as a new pathological entity by Kajihara et al, who found that ECPA is not a systemic vasculitis and appears predominantly in males [3]. Relatively younger patients are affected, mainly 30-to 50-year-olds, and the mortality rate from SCD is high.…”