rheumatic fever

rheumatic fever, inflammatory disease of the heart, joints, central nervous system, and subcutaneous tissues that develops after a throat infection with group A beta-hemolytic Streptococcus bacteria, including untreated scarlet fever or strep throat. Prevention is possible with penicillin, but specific treatment is not available. Rheumatic fever is particularly important because of the heart disease, which includes vascular damage, that may ensue. The illness occurs chiefly in children and young adults, with a peak incidence between ages 5 and 15.

Symptoms

When a streptococcal throat infection goes untreated, most patients recover without complications. Approximately 1 percent, however, develop rheumatic fever. The onset of the disease is most often characterized by the sudden occurrence of fever and joint pain and inflammation several days to six weeks after the streptococcal infection. Signs of cardiac involvement include heart murmurs, increased pulse (heart rate), and heart enlargement. Inflammation of the heart muscle and supporting structures may lead to a permanent scarring and contracture of the heart valves and a marked decrease in life expectancy.

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Other symptoms of rheumatic fever include nodules beneath the skin and skin rashes, the most typical of which is erythema marginatum; Sydenham chorea, a nervous system manifestation marked by emotional instability and purposeless, involuntary movements of the arms and legs; abdominal pain; nosebleeds; weakness; and loss of appetite and body weight. Generally, the clinical symptoms, severity, and aftereffects of an attack of rheumatic fever are highly variable, ranging from a condition so mild as to go unnoticed to a severe acute attack associated with cardiac failure and death.

Diagnosis and treatment

During the course of rheumatic fever, the streptococcal organism may no longer be demonstrable in cultures of the throat or other infected body areas, but blood titres of antibodies against the streptococcus, such as antistreptolysin O, are high. All the numerous types of group A beta-hemolytic Streptococcus appear capable of inducing rheumatic fever in susceptible individuals, infection with one type confers no immunity against the others, and individuals who have experienced one attack of rheumatic fever are especially prone to subsequent attacks. Both the initial and recurrent attacks can be effectively prevented with penicillin.

Symptomatic treatment of the condition includes the use of salicylates such as aspirin or one of the steroid hormones. Surgery may be advocated to relieve the narrowing of the openings of the heart valves. Rheumatic fever patients must receive antibiotics on a regular basis for the remainder of their lives because their damaged heart valves predispose them to the development of bacterial endocarditis.

Suspected role of autoimmunity and risk factors

The underlying physiological factors that are responsible for rheumatic fever are not clear. Most authorities favour the theory that the disease results from an autoimmune reaction, involving the production of antibodies that attack the body’s own tissues. The autoimmune reaction is thought to be triggered by certain protein components of the streptococci, known as antigens, the structure of which resembles that of molecules found in human tissue (“self antigens”). Because of this resemblance, the antibodies that recognize streptococcal antigens may mistakenly react with similarly shaped antigens of certain cells of the body—such as those of the heart, joints, and central nervous system. By binding to these self antigens, the antibodies cause the tissue damage characteristic of rheumatic fever.

Various risk factors have been identified for rheumatic fever, among them family history of the disease and socioeconomic factors. Persons with a family history of rheumatic fever may have a genetic predisposition that increases their chances of developing the disease. In terms of socioeconomic factors, rheumatic fever is sometimes referred to as a disease of poverty, because of the notably high risk for developing the disease in persons living in overcrowded unsanitary conditions. Such conditions provide the foundation for the rapid transmission of Streptococcus. The strain of Streptococcus can also be a factor in determining whether a person will develop rheumatic fever.

Historical developments and epidemiological considerations

Since the mid-20th century the incidence and severity of rheumatic fever and other streptococcal infections, such as scarlet fever, have declined precipitously in the developed countries. This decline has occurred independently of the use of penicillin and other drugs. Research suggests that the historical decline in rheumatic fever likely was due to vast improvements in living conditions that occurred in the mid-20th century in more-developed countries. This idea is supported by the fact that rheumatic fever in the 21st century persists mainly in less-developed parts of the world, where poor nutrition, poor living conditions, and limited access to health care are common. Rheumatic fever also remains a serious concern for certain indigenous populations, particularly those of Australia and New Zealand.