A 34 y.o. woman comes in with R arm numbness
On exam she has carotid bruits
on CTA she has a markedly small aorta; what disease is this?
Our patient had adult takayasu’s large vessel arteritis. This is a systemic disease of unknown etiology that most commonly affects women of childbearing age although it can also occur in children. It presents with carotid bruits as in our patient, HT and blood pressure difference in the extremities. Often there is angiographic narrowing or occlusion of the entire aorta.
The first case of Takayasu’s was described in 1908 by Dr. Mikito Takayasu at the Meeting of the Japan Ophthalmologic Society. He described “wreathlike” blood vessels in the retina associated with absent wrist pulses. This was thought to represent new vessel growth in response to arterial narrowing in the carotids.
neovascularization associated with takayasu’s
Patients present generally with either a systemic phase with active inflammatory disease or an occlusive phase. The sed rate is often elevated in the inflammatory phase but not in the occlusive phase. Our patient was felt to be in occlusive phase. Her imaging did not show a stroke and she was referred to rheumatology who started immune suppression. The danger of surgery in these cases is that an aneurysm will develop at the site of the surgery.
TYPES OF VASCULITIS
Behcet’s disease- is characterized by a triad of mouth ulcer, genital ulcer, and eye inflammation.
Buerger’s disease- Mainly affects smokers, leading to decreased blood flow to the hands and feet.
Eosinophilic granulomatosis with polyangiitis( Churg Strauss)- is associated with asthma, nasal polyps, sinusitis and elevated eosinophil counts. It usually involves the lungs , peripheral nerves, kidneys and heart
Cryoglobulinemia-is characterized by recurrent purpura on the lower extremities and may be associated with Hep C or paraproteins.
cryoglobulinemia a small vessel vasculitis.
Henoch-Schoenlein Purpura- follows a URI and is often self limited but may be associated with kidney disease.
Polymyalgia Rheumatica- is a xyndrom of pain and stiffness to the shoulders and hips which often occurs in giant cell arteritis.
Rheumatoid Vasculitis- can occur in patients with severe RA
Takayasu’s Arteritis- affects young women < 50. Involving the aorta and its branches.
Granulomatosis with Polyangiitis (Wegener’s) – is a systemic vasculitis involving the lungs, kidneys, and upper respiratory tract. It is associated with ANCA.
narrowing of the R subclavian and absent common carotid on the L in Takayasu’s
Andres J, Nahhas A, Pennell, et al. Non-invasive imaging in the diagnosis and management of takayasu’s arteritis Annals of the Rheumatic Diseases 2004. 63:995-1000.
Miyata T, Sato O, Koyama H, et al. Long-term survival after surgical treatment of patients with Takayasu’s arteritis. Circulation 2003. 108:1474-1480.
Peter J, David S, Danda D, et al. Ocular manifestations of Takayasu arteritis: a cross-sectional study Retina 2011 Jun;31(6):1170-8.