Moving on to medium-sized vessel disease or
arteritis. There is a very interesting disease,
Kawasaki Disease. You can hear from the name
of the person who described it. This is a
Japanese disease, for some reason rarely seen,
once in a while seen in North America and
Western Europe, but very common in Japanese
children. So clearly there has to be some
ethnic genetic factor or some environmental
factor in Japan that is not present in other
places. But much of the work on this disease
has been done in Japan. So here is a typical
case. You can see this is a 12-month-old Japanese
male who is admitted with fever greater than
38 degree centigrade, lasted for five days.
He didn’t respond to the usual doses of
acetaminophen, Tylenol, or ibuprofen. His
physical exam, he has extremely red eyes without
pus or drainage. He has bright red, chapped,
and cracked lips. The mucosal membranes in
the mouth are also red. He has a strawberry
tongue, that is tongue with lots of little
swollen pores and his bacterial cultures have
been negative. The palms and the soles of
the feet are also red and there are skin rashes
in the middle of the body but there are no
blisters. And you can see in the pictures
on the right hand side some of each of these
manifestations, the red eye, the skin rashes
and so forth. This disease was originally
called mucocutaneous lymph node syndrome because
there was involvement of the lymph nodes.
It was also called infantile polyarteritis.
It is a rare condition and that again involves
inflammation of the medium sized arteries
including the coronary arteries by the way
and it is usually called Kawasaki Disease
because Dr. Kawasaki was the first person
to describe this syndrome in Japanese children.
The laboratory studies show a remarkably elevated
erythrosedimentation rate, no surprise because
of the inflammation, a high white count, and
anemia. The diagnosis is made by biopsy when
you can see inflammatory involvement of the
arteries and the therapy is again immunosuppressive
drugs. High-dose corticosteroids and other
immunosuppressive drugs actually for as much
as for year and half to two years and then
tapered and actually there is a fairly significant
mortality, 50% to 60% of children survive
but a significant number go on to die often
because of involvement of the coronary arteries.
By the way, these children may also end up
in later life with coronary aneurysm and require
angioplasty. So this is a disease that damages
the coronary arteries and continues to cause
problems in later life.
Now here is another example of a child with
a number of pictures. Again just to point
out, that disease diagnosed because it affects
mucous membranes, lymph nodes, walls of the
blood vessels especially the coronaries and
usually medium arteries but small and large
arteries involvement can also be present.
There is high persistent fever usually greater
than 102 Fahrenheit or 38 to 39 centigrade.
It is isn’t relieved by the usual things
that relieve fever such as acetaminophen and
it can also involve the lymph nodes and again
usually we call it Kawasaki Disease. It is
not clear what causes this. It occurs most
frequently in Japan. It is sometimes seen
after congenital heart defect repair. It is
more often in boys than in girls. Most of
the patients are younger than age 5. It is
thought that it is probably an autoimmune
disease because it seems to respond to anti-immune
therapy. Some people have suggested that it's
a reaction to staph aureus in the body or
on the skin. As I have said before chronic
coronary disease with aneurysms can result
in 15% to 25% of untreated children and this
causes continuing problems in adult life.
Therapy we have already talked about. It's
IV gamma globulin, high-dose aspirin and even
more powerful immunosuppressives and usually
with this patients improve, particularly with
IV gamma globulin. Again suggesting this is
an auto-immune disease because that is a therapy
very effective against autoimmune disease.
There is no specific test to diagnose Kawasaki.
It is usually diagnosed on the basis of symptoms
and often if there is any doubt, a biopsy
is made to show the inflammation in the arteries.