Autopsy features of sudden death due to isolated eosinophilic coronary arteritis

Abstract

Isolated eosinophilic coronary arteritis (IECA) has been reported as a cause of sudden unexpected death and has recently been recognized as a newly emerging vasculitic disease. We identified eight case reports and two case series of sudden death due to IECA in the medical literature and we present two new cases of sudden death due to IECA. Our cases further support the proposition that IECA may be a newly emerging distinct vasculitis, which can go undiagnosed and present with sudden death. At autopsy IECA presents with isolated non-necrotizing predominantly eosinophilic inflammation of the coronary arteries without vasculitis in any other organ or blood vessel. The mean age of death of our two cases and the previously reported cases of IECA is 47 years, comprising 13 females and 3 males with a range of 34-64 years. All cases died suddenly and unexpectedly. Past medical history of recurrent chest pain was documented in 63% of cases. The patho-etiology of IECA may involve an aberrant immune response or hypersensitivity reaction. Elucidation of the pathology of IECA may be translated into definitive diagnostic, interventional, and preventive modalities, which will further reduce the person years of life lost to heart disease.