Sudden cardiac death caused by coronary vasculitis

Coronary vasculitis is a rare and diagnostically challenging cause of sudden cardiac death (SCD). There are currently no large-scale series on this rare entity. A retrospective non-case–control observational study of SCD with coronary vasculitis referred to a tertiary cardiac pathology referral centre at the National Heart and Lung Institute at the Royal Brompton Hospital between 1996 and 2010 was completed. Ten cases of SCD with coronary artery vasculitis were retrieved from a database of 1,980 SCD cases (0.5%) with a 1:1 male/female ratio; median age was 39 years and range 15–71 years. Six deaths occurred in hospital following symptoms or cardiac arrest in the community; the remaining died at rest at home (n = 4). Appearances ranged from aneurysms of the coronary artery to occlusive lesions mimicking atheroma or masses imitating tumour. Types of vasculitis detected were: eosinophilic (n = 5), two associated with Churg–Strauss syndrome; lymphoplasmacytic vasculitis (n = 4); and idiopathic giant cell arteritis (n = 1). This study shows coronary vasculitis as a rare cause of SCD with a variable macroscopic and microscopic presentation that pathologists need to be aware of.