IgG4 related thoracic aortitis is a recent addition to the differential 
diagnosis for inflammatory aortic disease - a condition which is often 
underappreciated until complications arise such as aneurysmal formation or 
aortic dissection. Currently, IgG4 aortitis remains a post-surgical diagnosis 
reliant on positive immunohistochemistry findings. Management is guided by the 
extent of disease involvement, which can be gauged by serum IgG4 levels and 
radiological findings. Options include surgical resection, corticosteroid 
therapy and steroid-sparing agents to prevent relapses.