· A: Rheumatic fever is a disease that can develop as a complication of strep throat. It is rare in the US (for reasons discussed below), but is relatively common in the developing world. Rheumatic heart disease is a long-term result of rheumatic fever (which sometimes you have to get multiple times) which can cause valve deficiencies, heart failure, and death.
Q: How do I know if I have it?
· A: You don’t. But symptoms occur several weeks after strep throat and can include fever, joint pain (without swelling), a heart murmur or heart failure, a rash, and involuntary movements. Kids make up 80% of the cases, and adults 20%. [For people who have taken medical boards and theoretically should remember this stuff: the major (Jones) criteria for acute rheumatic fever are: 1. Migratory polyarthritis, 2. Carditis, 3. Subcutaneous nodules, 4. Erythema marginatum, and 5. Syndenham’s Chorea*.]
Q: What causes it?
· A: I’d like to just say, “strep throat,” but that’s oversimplifying things. The most common cause of a throat infection (pharyngitis, if you will) is a virus, but the reason doctors always stabbing kids’ throats with long Q-tips is that they are worried about “strep throat,” which is infection of the throat caused by the streptococci bacteria. There are very very few strains of strep which can actually lead to Rheumatic Fever (and these are known as the rheumatogenic strains).
Q: So everyone who gets strep throat with one of these rheumatogenic strains of Strep develops Rheumatic Fever?
· A: Nope, that would be too simple. Rheumatic Fever comes about when someone’s body reacts (for unknown reasons) to the bacteria, causing the body to attack its own tissues (kind of like an auto-immune disease like lupus).
Q: What is Rheumatic Fever vs Rheumatic Heart Disease?
· A: Nope. Rheumatic heart disease is the long-term results of several bouts of (or a bad or long-term case of) rheumatic fever. If you catch and treat rheumatic fever (with literally years of penicillin), it is possible to avoid the heart disease. Rheumatic heart disease is a huge problem in the developing world. It is estimated that 15,000,000 children and young adults have rheumatic heart disease, and 230,000 die of it each year. It is especially bad because once you develop the heart disease, the only treatment is a valve replacement. As you can imagine, the majority of people who actually have rheumatic heart disease don’t have access to a cardiothoracic surgeon to pop in a new valve, as would be the case in the US. Also, dying from heart failure is a drawn-out and painful process.
Q: Why don’t we have rheumatic heart disease in the US?
· A: One hundred years ago, rheumatic heart disease was the #1 killer of children and young adults in the US, and now the incidence is nearly 0%. It would be nice to pat ourselves on the back about our great medical care and widespread use of penicillin in eradicating this deadly disease, but that’s not why it practically doesn’t exist here anymore. For reasons that are unclear, the rheumatogenic strains of Strep have practically disappeared in the US, except when they recur sporadically in specific pockets of the country (like Western PA). The incidence of rheumatic fever was already decreasing rapidly before we even started diagnosing it and treating it with penicillin.
Q: Does this mean we don’t have to test every single kid who has a sore throat for Strep?
· A: Probably, but who wants to open that can of worms? Also, why stop doing something you probably don’t have to be doing when it costs only a half a billion (that’s right, that’s a B) dollars per year??
*The word chorea describes a disorder of abnormal, involuntary movements. Chorea is a kind of ancient Greek dance (and also gives us the word choreography). Syndenham’s chorea is a term only used to describe the chorea of rheumatic fever (cause there are other kinds, like in patients with Huntington’s Disease). The other term for it (cause why only have one term in medicine when you can have two or three for us to memorize) is St. Vitus’ Dance, in reference to the “manic dancing that historically took place in front of [St. Vitus’] statue during the feast of Saint Vitus in Germanic and Latvian cultures.” (Thanks, Wikipedia!)