A 20yr old girl presents with malaise and abdominal pain to her GP, who on examination notes that her left hand is very cold, and her peripheral pulses are weak. He refers her to the vascular surgeons who request an angiographic phase CT. CT demonstrates wall thickening and enhancement of the aortic arch, with stenosis particularly affecting the proximal portion of the left subclavian artery. There is also narrowing of the SMA. What is the probable diagnosis?
Bechet Syndrome | |
Polyarteritis Nodosa | |
Microscopic Polyangitis | |
Takayasu Arteritis | |
Fibromuscular Dysplasia |
Takayasu’s generally affects the large and medium vessels such as the aorta and its major branches. It has a strong female predominance (9:1) and a young age of onset. On CT there is wall thickening and enhancement early on, which progresses to stenosis/occlusion. There can be aneurysmal dilatation, and in the late stages the distal aorta can appear narrowed.
Polyarteritis Nodosa is a medium vessel vasculitis, with a characteristic finding of multiple aneurysm formation. These form after segmental weakening of the arterial wall due to inflammation. The symptoms are usually due to ischaemia and infarction of the affected organ. The renal arteries are most commonly involved, classically with multiple microaneurysms identified the kidney on angiography.
Fibromuscular dysplasia is a non-inflammatory vascular disease which commonly affects the renal arteries. It causes intermittent arterial stenosis and aneurysmal dilatation which gives a ‘string of beads’ appearance. It responds very well to angioplasty. Microscopic polyangitis is a small vessel vasculitis, and on angiography the tiny microaneurysms are not typically seen. Bechet’s is a vasculitis affecting multiple organ systems, but classically with a triad of oral and genital ulceration and uveitis. When it affects the GI tract, it causes large ulcerations, particularly in the terminal ileum.
References
Radiologic Features of Vasculitis Involving the Gastrointestinal Tract: http://pubs.rsna.org/doi/full/10.1148/radiographics.20.3.g00mc02779
http://radiopaedia.org/articles/polyarteritis-nodosa-1