Infection: Rheumatic Fever
Infection: Rheumatic Fever
Rheumatic fever is caused by an inflammatory reaction that occurs after an infection caused by certain Group A streptococci, sphere-shaped bacteria that grow in long chains. Strep throat and scarlet fever are the illnesses most likely to lead to rheumatic fever if they are not properly treated.
Rheumatic fever has been known for centuries; its symptoms were described in the seventeenth century by Thomas Sydenham (1624–1689), an English doctor, although the connection between rheumatic fever and bacterial infections was not yet known. In 1880 doctors discovered that a sore throat could lead to rheumatic fever; in the early 1900s they found that there was also a link between scarlet fever and rheumatic fever. The diagnostic criteria for rheumatic fever, still used in the early 2000s, were first identified by a doctor named T. Duckett Jones in 1944. There were no effective preventative treatments for rheumatic fever, however, until the late 1940s, when antibiotics proved to be effective treatments for both strep throat and scarlet fever.
Although fever is one of the symptoms of rheumatic fever, the disease is better understood as an inflammatory disorder that affects several body systems, particularly the skin, the tissues under the skin, the central nervous system, the joints and muscles, and the circulatory system. Multiple episodes of rheumatic fever can cause long-term damage to the heart and its valves that gets worse over time. This complication is known as rheumatic heart disease. Rheumatic fever can recur even after antibiotic treatment for the acute phase of the disease.
Rheumatic fever develops in about 3 percent of people who have either untreated strep throat or scarlet fever about twenty days after the strep infection. In about 30 percent of cases, the patient was not treated for the strep infection because he or she had no noticeable symptoms. A common pattern for rheumatic fever is pain and swelling in the joints between two and six weeks after the strep infection, along with fever and a generally unwell feeling. The patient may also develop lumps of tissue
known as nodules beneath the skin of the wrists, elbows, and knees, and a rash on the chest and upper arms. Another symptom is chorea, a movement disorder that affects the muscles of the patient's face and upper arms. The symptoms of the initial attack of rheumatic fever go away in about six weeks for 75 percent of patients; the remainder may take as long as twelve weeks to recover.
People who have had an acute attack of rheumatic fever are more likely to have the disease flare up again if they get another strep infection. These recurrences can lead to severe heart valve damage and can predispose people to bacterial endocarditis, a condition that develops when bacteria form colonies on the tissues of the heart valves. Pieces of damaged tissue can break off from the valves and travel through the bloodstream, eventually blocking major blood vessels and causing stroke or heart failure.
Rheumatic fever used to be much more common before the introduction of antibiotics in the 1940s than it is in the twenty-first century. According to the American Heart Association, about 15,000 people in the United States died each year of rheumatic fever and its complications in the 1950s. In 2004 there were 3,248 deaths resulting from rheumatic fever, 2,226 in women and 1,022 in men. The disease is rare in the
United States and other developed countries as of 2008, but is still a major killer in less developed parts of the world. A recent report indicates that 95 percent of cases of rheumatic fever worldwide occur in the poorer nations, particularly those with tropical climates.
A Murder Mystery Solved
For over 200 years, music scholars and biographers wrestled with a conspiracy theory about the sudden death of Wolfgang Amadeus Mozart (1756–1791) just a few weeks before the famous composer's thirty-sixth birthday. Some thought that Mozart might have been poisoned by Antonio Salieri, a rival musician who was jealous of Mozart's talents. In 2000, however, a professor of medicine at the University of California, Davis, reopened the case by looking at the composer's symptoms as reported by his family and doctors at the time. She concluded that Mozart died of complications from rheumatic fever, not murder.
The professor noted that Mozart had experienced several episodes of rheumatic fever earlier in his life, which almost certainly damaged his heart. In late November 1791 he fell ill suddenly with fever, headaches, a skin rash, and painful swelling of the joints in his arms and legs. In the second week of his illness, his body swelled with fluid to the point where his clothes did not fit and he could not sit up in bed without help—indications of congestive heart failure. Mozart then lost consciousness and
died on December 5, 1791, after just fifteen days of illness.
People of any age can get rheumatic fever after a strep infection; however, most cases in North America are diagnosed in children between the ages of five and fifteen. As far as is known, rheumatic fever in the United States affects both sexes and all races equally, although people of any race living in crowded conditions are at increased risk. In terms of symptoms, girls are more likely than boys to develop chorea and heart valve problems. The disease is most likely to occur in the winter—the peak season for strep throat and scarlet fever.
As of 2008, rheumatic fever is thought to result from an abnormal response of the immune system to certain Group A streptococci. There is no evidence that infections involving other types of bacteria can lead to rheumatic fever. Some doctors also think that there may be genetic factors that make some people more susceptible to rheumatic fever.
The most significant symptoms of rheumatic fever include:
- Chorea: Jerky involuntary movements of the arms and facial muscles
- Painful swollen joints
- Chest pain
- A faint pink rash on the upper body, which may have a ring-shaped or snake-like pattern
- Nodules on the wrists, elbows, and knees
Other symptoms may include shortness of breath, pain in the abdomen, and nosebleeds.
The diagnosis is based on a combination of the patient's history (particularly a strep infection within the previous two months), the patient's descriptions of his or her symptoms, and the results of laboratory tests used to evaluate the symptoms. There is no single test that can diagnose rheumatic fever. The present guidelines for diagnosis state that the patient must meet either two major diagnostic criteria or one major criterion and two minor ones. The major criteria for diagnosing rheumatic fever are:
- Evidence of carditis (inflammation of the heart). The signs of carditis include heart murmurs, congestive heart failure, or shortness of breath and chest pain.
- Arthritis in several joints.
- Nodules under the skin.
- Skin rash.
The minor criteria include fever; joint pain; abnormal findings on an electrocardiogram (ECG); blood test results that indicate a recent strep infection; and a history of previous episodes of rheumatic fever.
Acute rheumatic fever is treated with antibiotics to kill the bacteria and to prevent future flareups of rheumatic fever. Penicillin is the most common antibiotic used. Patients are typically maintained on daily (by mouth) or monthly (by injection) doses of antibiotics for three to five years after the first episode of rheumatic fever; in some cases, they may be given maintenance doses of penicillin for life, particularly if they have suffered damage to their heart valves or are at high risk of repeated exposure to streptococcal infections.
Patients are also given over-the counter pain relievers like aspirin or Tylenol for joint pain and fever. Those with severe carditis are given corticosteroid medications to reduce the inflammation in the heart tissue. Patients who have developed congestive heart failure are treated with diuretics and a heart drug called digitalis.
The prognosis of rheumatic fever depends on whether the patient developed carditis and if so, how severely the heart was damaged. Bacterial endocarditis can lead to eventual death from stroke or heart failure.
WORDS TO KNOW
Carditis : Inflammation of the heart.
Chorea : A movement disorder characterized by brief involuntary movements of the muscles.
Diuretics : A group of medications given to increase urine output.
Endocarditis : Inflammation of the lining of the heart and its valves.
Group A streptococcus : A sphere-shaped bacterium that grows in long chains and causes strep throat as well as scarlet fever.
Nodule : The medical term for a small rounded lump of tissue.
Rheumatic fever can be prevented by prompt diagnosis and treatment of scarlet fever or strep throat. The most effective form of prevention is giving antibiotics within nine days of the first symptoms of a Group A streptococcal infection.
At present the National Institutes of Health (NIH) is conducting clinical studies of the rates of rheumatic heart disease in Central America and parts of Asia, as well as using DNA typing to look for possible genetic factors in rheumatic fever.
SEE ALSO Heart failure; Scarlet fever; Sore throat; Strep throat
Margulies, Phillip. Everything You Need to Know about Rheumatic Fever. New York: Rosen Publishing Group, 2004.
Crawford, Franklin. “Medical Experts and Scholars Rule Out Foul Play in Death of Wolfgang Amadeus Mozart: Rheumatic Fever, Not Murder, Most Likely Killed the Young Musical Genius.” Cornell News, February 15, 2000. Available online at http://www.news.cornell.edu/releases/Feb00/Mozart.death.html (accessed June 26, 2008).
Zuger, Abigail. “Cases: Paying Homage to the Wisdom of Voices from Medicine's Past.” New York Times, June 22, 2004. Available online at http://query.nytimes.com/gst/fullpage.html?res=9B0DE6DE1339F931A15755C0A9629C8B63&sec=&spon=&pagewanted=all (accessed June 26, 2008).
American Heart Association (AHA) Fact Sheet. What about My Child and Rheumatic Fever? Available online in PDF format at http://www.americanheart.org/downloadable/heart/1196275912918ChildRhueFever.pdf (accessed June 26, 2008).
Mayo Clinic. Rheumatic Fever. Available online at http://www.mayoclinic.com/health/rheumatic-fever/DS00250 (accessed June 26, 2008).
Medline Plus. Rheumatic Fever. Available online at http://www.nlm.nih.gov/medlineplus/ency/article/003940.htm (accessed June 26, 2008).
National Heart, Lung, and Blood Institute (NHLBI). Heart Valve Disease. Available online at http://www.nhlbi.nih.gov/health/dci/Diseases/hvd/hvd_whatis.html accessed June 26, 2008).