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I was talking to a rheumatologist on the phone. "In Colorado I found two cases of adult rheumatic fever and then my partner found another one."
"Adult rheumatic fever?" said the rheumatologist, "You? I've never seen adult rheumatic fever. I don't believe it."
"Um," I said, "All three were confirmed by a rheumatologist." A different one.
Awkward silence. Family Practice is a specialty but sometimes other specialists lose track of that.

So, what is rheumatic fever and why all the fuss?

Rheumatic fever is a serious complication of streptoccal infection, and it's one of the reasons doctors get excited about strep throat. We do want to treat you for strep throat and yes you should take the penicillin until the tablets are gone. Acute rheumatic fever is a leading cause of acquired heart disease in children and teens. That is, it can damage the heart.

There is more than one sort of strep. This is group A B-hemolytic streptococcus. Other streps do not seem to cause rheumatic fever.

My first patient presented with joint pain, the sudden onset of arthritis. Her joints were not red and not hot and she had a normal range of motion. We had just had an outbreak of parvovirus B19 and I had gotten that with painful arthritis that lasted for two months. But when I looked at her chart (ALWAYS look at the last note), she had been seen within the last few weeks with a fever. She had had a sore throat, too.

I sent her for labs. She had an elevated ASO -- antistreptolysin O antibody and an elevated sed rate.

Rheumatic fever is an odd diagnosis. A patient must have two major criteria and evidence of streptococcal infection or one major and two minor criteria and evidence of streptococcal infection.

Major criteria are:
1. carditis - an inflamed heart
2. polyarthritis - joint pain in multiple joints
3. chorea - an abnormal involuntary movement disorder
4. subcutaneous nodules
5. erythema marginatum - a particular rash

Minor criteria are:
1. previous rheumatic fever or evidence of rheumatic heart disease
2. arthralgias - muscle aches
3. fever
4. elevated sed rate, positive C-reactive protein or leukocytosis
5. prolonged PR interval on an electrocardiogram

Evidence of strep
1. increased antistreptolysin antibody or other strep-related antibodies
2. recent positive strep A throat culture
3. recent scarlet fever

She had the polyarthralgia, had a documented fever, an elevated sed rate and a positive ASO. I read about rheumatic fever and promptly sent her to a rheumatologist to confirm: the treatment was monthly penicillin shots for five years to keep it from recurring.

Yes, said the rheumatologist, she has it and she does need monthly penicillin shots for five years.

The second patient was similar, except that she also had a rash. The rash, however, looked like parvovirus. More labs: she had BOTH parvovirus and rheumatic fever. (So much for Occam's razor.) She was a school teacher. The rheumatologist did not prescribe five years of penicillin shots. He said that she should have monthly penicillin shots until she retired.

Oh, my.

My partner soon picked up a third case. All three had arthritis and luckily none of them had the heart damage.

I did have a young patient, around age 10, who had had rheumatic fever and was coming in for monthly penicillin shots. He had damage to his aortic valve and saw the pediatric cardiologist every year.

His mother brought him in, distraught, after the yearly visit. "They want to do heart surgery. But they said that he could die. What should I do?"

I read the notes and explained. "The heart valve leaks. It has leaked more and more. Now his heart can't keep up when he is active. He could die in the surgery, but if he does not have the surgery, his heart will get worse and worse and he will die."

"I am frightened. Do you think he will be ok?"

"I hope that he will be ok. I know it is very frightening, but he will grow and be healthy if the surgery succeeds."

He had the aortic valve replacement and did beautifully. Now he could do sports and felt better; and still came in for the monthly penicillin shots.

for Science Quest 2012

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