Giant Cell Arteritis

00:01 A third form of medium-sized vessel vasculitis we have talked about before is Buerger’s disease, thromboangiitis obliterans. Remember this is a disease that segmental. It can be acute as well as chronic. It involves medium and smaller arteries, particularly in the hands and the feet. Interestingly enough, it can also involve the veins of the leg and lead to deep venous thrombosis and pulmonary embolism. It can lead to peripheral vascular disease with intermittent claudication and it is almost always seen in heavy smokers.

00:36 And in almost always will not improve until these people discontinue smoking. It's usually seen in men, 25 to 40 years of age, and here you see a very dramatic picture of gangrene in finger tips related Buerger’s disease.

00:50 And now finally we come large-vessel vasculitis. There are two very common forms of large-vessel vasculitis. Giant cell often called temporal arteritis because the temporal artery is often favorably involved and Takayasu’s arteritis. And again an arteritis more commonly seen in Japan but also seen here in the United States and in Europe. Here we see something, a slide that refers to Giant cell arteritis. It involves medium-size and large vessels.

01:26 There is granulomatous inflammation of the aorta, major branches usually with focal inflammation, and a predilection for extracranial vessels. The temporal artery is involved and that is often how the diagnosis is made. One biopsies, the temporal artery and sees the inflammation and you see a biopsy right there with a high degree of inflammation and narrowing of the temporal artery lumen or channel. It is often seen in people over age 50 and in fact considerably older than age 50 and it can be associated with the syndrome of polymyalgia rheumatica with two to one female to male ratio. In polymyalgia rheumatica, the patient’s often report muscles aches and pains diffusely throughout the body associated with severe fatigue and a very high erythrocyte sedimentation rate often close to 100 cm per hour. Also, these patient’s report very impressive symptoms. They may have sudden headache, partly related to occlusion of the temporal artery. There can be tenderness over the temporal artery. There can be blurred vision, diplopia and if not treated can lead to blindness. Again the diagnosis is the elevated erythrocyte sedimentation rate. There is an elevated CRP protein which is an inflammatory protein and biopsy is usually diagnostic.

02:58 Therapy is high dose corticosteroids otherwise 80% of these going to go on to blindness, so it is really important to make the diagnosis. And it is a large vessel vasculitis.

03:10 The final large vessel vasculitis, Takayasu again seen more commonly in Japan but also reported here in United States and in Western Europe. It is a granulomatous inflammation of the aorta and it's major branches. Again usually seen in younger people, less than 50 years of age, can affect the coronary arteries and result in a heart attack, can affect the carotid arteries and result in a stroke and again treated with high dose corticosteroids or anti-immunological agents. More common in Japan but definitely seen here in the United States.