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.
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.
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.
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.
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.
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