What is Rheumatic fever?

Rheumatic fever is a disease characterized by the inflammation of many connective tissues throughout the body, particularly in the heart, joints and central nervous system (the brain and spinal cord). Rheumatic fever is usually caused by an untreated infection with streptococcus bacteria, such as in strep throat or scarlet fever.

Valvular stenosis is the narrowing, stiffening, thickening, fusion or blockage of a heart valve.Rheumatic fever usually develops roughly 20 days after the streptococcus infection and can damage a wide variety of tissues in the body, especially the heart valves. It is one of the major causes of heart valve disease in the world. It can also damage the chambers and other vessels of the heart. Untreated, the damage to the heart will become permanent, at which point rheumatic fever is called rheumatic heart disease. Rheumatic heart disease may involve one or more of the following:

• Valvular insufficiency. Heart valves that leak or "regurgitate" blood.
• Valvular stenosis. Narrowed or constricted valves.
• Atrial fibrillation. A rapid, abnormal heart rhythm (arrhythmia) caused by abnormal electrical signals from the upper chambers of the heart (atria).

Rheumatic fever is more common in children aged from 6 to 15. Before the widespread availability of antibiotics in the United States, rheumatic fever was the most common cause of valve damage. However, cases of rheumatic fever are now relatively rare. According to the National Institutes of Health (NIH) roughly three percent of untreated streptococcus infections develop into rheumatic fever.

The mortality rate for rheumatic heart disease varies greatly from country to country. In more developed countries, mortality is 0.5 per 100,000. In less developed countries, mortality reaches 8.2 per 100,000, accounting for millions of deaths globally every year. Much of these are due to the lack of antibiotics in developing countries and difficulties in diagnosing the disease.

How is it diagnosed?

There are no specific tests to diagnose rheumatic fever. Instead, physicians rely on observing any symptoms that may appear following an infection with streptococcus bacteria. Over time, a model has been developed to help guide physicians to diagnose rheumatic fever. A diagnosis can be made if a patient has two major manifestations, or one major and two minor manifestations.

The major manifestations include:

• Carditis. Carditis may be detected during a physical examination if the physician hears a valvular murmur while listening to the heart through a stethoscope. Heart damage may also be identified with an echocardiogram (a form of ultrasound imaging that takes pictures of the heart’s structures and functions) or with an electrocardiogram (a test that measures the heart’s electrical activity).
• Arthritis in the joints.
• Chorea. This describes inflammation of the central nervous system, resulting in involuntary dance-like movements.
• Rash.
• Lumps under the skin (subcutaneous nodules).

The minor manifestations include:

• Fever
• Joint pain
• Previous rheumatic fever or rheumatic heart disease

There is some evidence that a test for C-reactive protein (CRP) may also help physicians diagnose rheumatic fever. Because rheumatic fever is an inflammatory disease, blood levels of C-reactive protein are abnormal during the rheumatic process.

How is Rheumatic fever treated?

Medical intervention is critical to rid the body of the streptococcal infection. Penicillin therapy is the most commonly used and successful method for killing the infection, though other antibiotics may be used if a patient has an allergic reaction to penicillin. Penicillin is usually taken for at least ten days to treat the condition.

While the primary treatment is focused on killing the original infection, physicians will also attempt to relieve the symptoms of rheumatic fever. To relieve joint pain, the first step is plenty of bed rest to alleviate some of the pressure on the joints. As patients begin to heal, strenuous activity should be limited until they have fully recovered.

Anti-inflammatory medications are also commonly prescribed. These might include the following:

• Salicylates. Pain relievers, of which the most commonly known is aspirin, are used to relieve joint pain and reduce inflammation.
• corticosteroids. Anti-inflammatory medications that may be prescribed for patients with pericarditis or heart failure.

Heart replacement valve surgery may be necessary if serious degeneration of the valve structures occurs.

Once rheumatic fever has occurred, there is a strong likelihood that the event will recur. Preventive measures must then be taken to stop further damage. Continual antibiotic treatment is usually required. A common preventive program is long-acting penicillin administered every three to four weeks. Many researchers recommend this therapy in young patients until they are at least 18 years old.

Medications

Activity

The patient should stay in bed until studies show the disease has subsided. Bed rest for 2 to 5 weeks is usually required, but some cases require months. Provide a bed pan or bedside commode so the patient won’t have to get up to use the bathroom.

Diet

• A liquid or soft diet in the early stages, progressing to a normal diet high in protein, calories and vitamins.
• A low-salt diet may be recommended if patient has carditis (inflammation of the heart).

What might complicate it?

More than 50% of those who suffer acute rheumatic fever with carditis will later (after ten to twenty years) develop chronic rheumatic heart disease, predominantly affecting the mitral and aortic valves. Other complications include irregular heart rhythm (arrhythmias), inflammation of the sac enclosing the heart (pericarditis), chronic lung inflammation (rheumatic pneumonitis), and congestive heart failure.

Alternatives

Conditions with similar symptoms include rheumatoid arthritis, pyogenic arthritis, bacterial endocarditis, systemic lupus erythematosus, atrial myxoma, sickle cell crisis, viral myocarditis, dermatomyositis, influenza, Reiter's disease, osteomyelitis, and Lyme disease.

Appropriate specialists

Cardiologist, internist, infectious disease specialist, rheumatologist, and neurologist.

Last updated 30 June 2015