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Rheumatic Fever

The etiology of acute rheumatic fever, ARF, is an immune response to group A beta hemolytic streptococcal infection rheumatic fever, rumatic fever. It only occurs with the throat infection. It does not occur following the skin infections with the same organism. In order to make the diagnosis you have to have the Jones criteria but you also have to have evidence of recent group A strep infection; either positive throat culture, rapid antigen test.

The natural history of this disease is that it causes cardiac involvement in 40-80% of cases, again depending on the outbreak and the region in which the outbreak occurs. There is a wide variation in that. Most common lesions are mitral insufficiency or stenosis.

The treatment for streptococcal infections is the same as the prophylaxis. You can also, in the acute phase, use aspirin or steroids for acute inflammation in rheumatic fever, although the evidence that that actually alters your outcome significantly is pretty soft. To treat kid’s strep infections - this is from Pediatrics 1995 - primary treatment is benzathine penicillin G 6,000 to 12,000 units, times one IM or penicillin V 250 mg t.i.d. p.o. for ten days, or erythromycin. As group A strep resistance to penicillin goes up they are now recommending.

Most people actually prefer to use amoxicillin, same dose as this, which is fine. Secondary treatment, which is for prophylaxis, benzathine penicillin IM every three to four weeks, or penicillin V 250 mg p.o. b.i.d. Most of the patients I follow with this diagnosis - which is a relatively rare diagnosis these days - alternate. They hate taking the pills but then after they’ve had a couple of shots they want to go back to the pills. They take them for awhile and they want to go back to the shots.

Whichever is longer. In patients with a history of carditis but no residual valve disease, prophylaxis should be continued for ten years or well into adulthood. Whatever that means. This is from that same 1995 Pediatrics. These are also the American Heart Association recommendations. In patients with persistent valve disease or recurrence of rheumatic fever, prophylaxis should be continued for at least ten years or until age 40.

Moving on to Kawasaki’s syndrome. This again is a very difficult disease to diagnose, even more difficult than rheumatic fever. And diagnosis early is very important. The strict diagnostic criteria is: fever for five days plus four of the following five symptoms: non-purulent bulbar conjunctivitis, a single lymph node greater than 1 cm in diameter unilateral, strawberry tongue with red cracked lips, rash - which is usually an erythematous rash - and it can be any kind of a rash almost.

You need to be very careful of that. And then edema or peeling of the hands or the feet. Unfortunately, the edema or peeling of hands or feet often occurs late in this disease so this one criteria is not likely to help you in the first ten days, which is the time during which treatment actually has the best prognosis. So if you suspect Kawasaki’s syndrome for any reason whatsoever, better to treat it and find out you are wrong than to not treat it.

The treatment is IVIG 1 gm/kg IV as a single dose, aspirin acutely for the inflammation, at high doses 10 mg/kg per day, and then chronically if they develop coronary artery disease, which we’ll talk about. There’s a lot of difference of opinion as to what to do for echo-surveillance. This is the regimen that is recommended by the American Heart Association.

They should be normal at diagnosis unless it’s recurrent Kawasaki’s. Six to eight weeks after diagnosis, and then if that’s normal, 6-12 months after diagnosis to make sure they’ve stayed normal.