Isolated Giant Right Coronary Aneurysm Associated With IgG4–Related Disease

IgG4-related disease (IgG4-RD) is a multisystem fibroinflammatory disease marked by a dense infiltration of plasma cells that are positive for IgG4 in the affected tissue(s), either with or without increased plasma levels of IgG4. Cardiac manifestations of IgG-RD include periaortitis, aneurysms, luminal thrombus or stenosis, multifocal fibrosclerosis, periarteritis, coronary artery disease, pseudotumours, phlebitis, pulmonary vasculopathy, valvulopathy, pericardial disease, myocardial disease, neurological/cerebrovascular disease and vasculitis. We report a case of a 60-year-old male who presented with atypical chest pain that led to the diagnosis of giant coronary aneurysm associated with IgG4-RD.