Kawasaki Disease

00:00 Moving on to medium-sized vessel disease arteritis, there’s 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’s not present in other places, but much of the work on this disease has been done in Japan. So, here’s a typical case. You can see this is a 12-month-old Japanese male who was admitted with a fever greater than 38 degrees centigrade, lasted for 5 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 mucosa membranes in the mouth are also red. He has a strawberry tongue that is a 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 on the middle of the body but there’s no blisters. And you can see on 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’s a rare condition 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 markedly 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 1-1/2 year to 2 years and then tapered. And actually there’s a fairly significant mortality, 50% to 60% of children survived but a significant number are going to die often because of involvement of the coronary arteries. By the way, these children may also end up in later life with coronary aneurysms and require angioplasty. So, this is a disease that damages the coronary arteries and continues to cause problems in later life. Here’s another example of a child with a number of pictures, again just to point out the disease is 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 can also be present, involvement can be present. There is a high persistent fever usually greater than 102 Fahrenheit or 38-39 centigrade. It 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’s not clear what causes this. It occurs most frequently in Japan, it is sometimes seen after congenital heart defect repair. It’s more often in boys than in girls. Most of the patients are younger than age 5. It’s thought that it’s 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, and as I’ve 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-dosed aspirin and an even more powerful immunosuppressives, and usually with these, patients improve particularly with IV gammaglobulin, again suggesting this is an autoimmune disease because that’s a therapy very effective against autoimmune disease. There is no specific test to diagnose Kawasaki, it’s usually diagnosed on the bases of symptoms and often if there is any doubt, a biopsy is made to show the inflammation in the arteries.