Rheumatic fever: pathogenesis, diagnosis, and clinical management

Rheumatic Fever

The Etiology, Incidence and Treatment of Rheumatic Fever Today

Like many diseases such as smallpox and polio, Zamula (1987) reports that until fairly recently, rheumatic fever was described by most public health officials as being a vanishing disease. "After World War II," Patlak (1991) reports, "the number of cases of rheumatic fever dramatically declined until, during the 20 years between 1965 and 1985 alone, the yearly number of cases of rheumatic fever among school-age children dropped by more than 90%" (p. 24). At the time, clinicians assumed that less crowded living conditions and the use of antibiotics were controlling the disease and some physicians called rheumatic fever a "vanishing disease in suburbia" (Patlak, p. 24). As a result, the children's hospitals that were previously dedicated to the care of rheumatic fever sufferers closed because of a lack of patients and disease registries that had been rigorously maintained by some states to track outbreaks of the disease were not updated. Not surprisingly, rheumatic fever, together with its concomitant risk of serious heart complications, reappeared in some parts of the country in a particularly virulent form during the last years of the 20th century (Zamula, 1987). To help determine the threat represented by this disease today, this paper provides a recapitulation of the signs and symptoms of the disease, how it is diagnosed, treatment options, the population affected, and long-term outcomes.

Review and Discussion

Signs and symptoms.

The name "rheumatic fever" is derived from two of the disease's most common symptoms: 1) joint pains and 2) fever. According to Zamula, "When rheumatic fever develops, it appears a few weeks after a strep throat, usually when the patient seems to be fully recovered. In children, the illness often begins with a fever -- sometimes as high as 104 degrees Fahrenheit in the first few days -- that may last as long as two weeks. Rheumatism often follows" (p. 26).

Diagnosis.

In her essay, "Strep' Demands Immediate Care," Patlak (1991) notes that diagnosis of rheumatic fever is based on its symptoms together with a history of a recent strep infection, confirmed by tests for strep antibodies in the blood. According to Arocha and Patel (1995), streptococcal infections precede the appearance of rheumatic fever; however, the disease presents a number of confounding elements that make diagnose problematic (Zamula, 1987). For instance, Zamula reports that a mild, untreated strep throat may result in rheumatic fever, while a severe case will not. "More than a third of patients with acute rheumatic fever don't remember having had a previous sore throat at all," she advises, and in the well-known Utah epidemic, fully 66% of the victims had no clear-cut history of a sore throat in the three months prior to the appearance of the disease (Zamula, p. 27). While some episodes of rheumatic fever can result in fatal heart damage (in fact, almost 13,000 deaths from rheumatic heart disease were reported in 1975), other cases result in no adverse effects at all (Zamula, 1987). In addition, one episode of rheumatic fever does not provide lifelong immunity and the disease can recur with subsequent cases of strep throat (Zamula, 1987).

Diagnosis is further complicated by the fact that an individual can experience an acute attack that results in heart damage and not even be aware of it. According to Zamula, "The damage is discovered much later, sometimes by accident. In fact, many adults found to have rheumatic heart damage have no memory of a rheumatic fever attack. The disease wasn't detected in the first place because they didn't feel sick enough to go to the doctor" (p. 27). In some cases, children have experienced neither fever nor rheumatism, making diagnosis even more difficult; in addition, there was no specific lab test for the disease at the time of this author's report (1987) (Zamula, 1987). In response, clinicians rely on the diagnostic criteria developed by Harvard's Dr. T. Duckett Jones during the 1940s (and later revised by the American Heart Association) to differentiate between rheumatic fever and childhood rheumatoid arthritis, gout, acute appendicitis, sickle-cell anemia, and other diseases with similar symptoms (Zamula, 1987). This author reports that for diagnostic purposes, patients are more likely to have rheumatic fever when two major symptoms, or one major and two minor, are present; the major symptoms are: 1) a painful form of arthritis (known as migrating polyarthritis) that travels from joint to joint (knee, ankle, elbow, wrist), and 2) inflammation of the heart, which develops in about 50% of those who experience the arthritis (Zamula, 1987).

An unusual symptom called St. Vitus' dance, or Sydenham's chorea, may also occur in about 10% of children up to age six months following an initial attack; this condition primarily affects prepubescent girls. Some of the symptoms of this condition include facial grimaces, uncontrolled twitching of the arms and legs, clumsiness, and changes in personality (Zamula, 1987). Another major symptom of rheumatic fever that is fairly uncommon is a fleeting, nonitchy, "chicken wire" rash that occurs on the chest and abdomen; this rash is exacerbated by heat (Zamula, 1987). Firm nodules can also develop under the skin over large joints in later stages of the illness; while these nodules are painless, they typically occur in conjunction with heart inflammation. Finally, the minor manifestations of rheumatic fever are less specific, but have been shown to include fever, joint pains, and a history of previous attack of rheumatic fever; symptoms such as evidence of prior strep infection (as evinced by the strep antibody level in the blood, nosebleeds, and abdominal pain that resembles acute appendicitis) are also useful in diagnosing rheumatic fever (Zamula, 1987).

Treatment.