“…In most cases, CAE is found to coexist with CAD, whereas in 10-20 of cases, it is associated with cocaine abuse, toxins, infl ammation, infections, infl ammatory diseases, cardiac lymphoma and connective tissue disorders such as Kawasaki disease, systemic lupus erythematosus, Marfan syndrome, and Ehlers-Danlos syndrome [6][7][8][9][10]. In histopathological appearance of the disease, marked destruction and reduction of the medial elastic fi bers with disruption of the internal and external elastic lamina, smooth muscle hyalinization of the coronary fi bro-muscular media, excessive nitric oxide (NO) production which leads to hyalinization by indirect acetylcholine production have been found [6][7][8][9][10]. Severe coronary wall infl ammation may play a role in CAE pathogenesis [10,11].…”