Rheumatic Fever

Synonyms

2

Overview

Rheumatic fever (also known as acute rheumatic fever, ARF), is an inflammatory disease that can involve the heart, joints, skin, and brain. The disease typically develops two to four weeks after a throat infection. Signs and symptoms include fever, multiple painful joints, involuntary muscle movements, and a characteristic but uncommon non itchy rash known as erythema marginatum. The heart is involved in about half of cases. Permanent damage to the heart valves, known as rheumatic heart disease (RHD), usually only occurs after multiple attacks but may occasionally occur after a single case of ARF. The damaged valves may result in heart failure. The abnormal valves also increase the risk of the person developing atrial fibrillation and infection of the valves.

Acute rheumatic fever may occur following an infection of the throat by the bacteria Streptococcus pyogenes. If it is untreated ARF occurs in up to three percent of people. The underlying mechanism is believed to involve the production of antibodies against a person's own tissues. Some people due to their genetics are more likely to get the disease when exposed to the bacteria than others. Other risk factors include malnutrition and poverty. Diagnosis of ARF is often based on the presence of signs and symptoms in combination with evidence of a recent streptococcal infection.

Treating people who have strep throat with antibiotics, such as penicillin, decreases their risk of getting ARF. This often involves testing people with sore throats for the infection, which may not be available in the developing world. Other preventative measures include improved sanitation. In those with ARF and RHD prolonged periods of antibiotics are sometimes recommended. Gradual return to normal activities may occur following an attack. Once RHD develops, treatment is more difficult. Occasionally valve replacement surgery or repair is required. Otherwise complications are treated as per normal.

Acute rheumatic fever occurs in about 325,000 children each year and about 18 million people currently have rheumatic heart disease. Those who get ARF are most often between the ages of 5 and 14, with 20% of first-time attacks occurring in adults. The disease is most common in the developing world and among indigenous peoples in the developed world. In 2013 it resulted in 275,000 deaths down from 374,000 deaths in 1990. Most deaths occur in the developing world where as many as 12.5% of people affected may die each year. Descriptions of the condition are believed to date back to at least the 5th century BCE in the writings of Hippocrates. The disease is so named because its symptoms are similar to those of some rheumatic disorders

Symptoms

The disease typically develops two to four weeks after a throat infection. Symptoms include: fever, multiple painful joints with the joints affected changing with time,involuntary muscle movements, and a characteristic but uncommon non itchy rash known as erythema marginatum. The heart is involved in about half of cases. Permanent damage to the heart valves usually only occurs after multiple attacks but may occasionally occur after a single case of ARF. The damaged valves may result in heart failure. The abnormal valves also increase the risk of the person developing atrial fibrillation and infection of the valves.

Other

  • Abdominal pain
  • Nose bleeds

Causes

Rheumatic fever can occur after an infection of the throat with the bacterium Streptococcus pyogenes, or group A streptococcus. Group A Streptococcus infections of the throat cause strep throat or, less commonly, scarlet fever. 

Prevention

Prevention of recurrence is achieved by eradicating the acute infection and prophylaxis with antibiotics. The American Heart Association suggests that dental health be maintained, and that people with a history of bacterial endocarditis, a heart transplant, artificial heart valves, or "some types of congenital heart defects" may wish to consider long-term antibiotic prophylaxis.

Diagnosis

Modified Jones criteria were first published in 1944 by T. Duckett Jones, MD. They have been periodically revised by the American Heart Association in collaboration with other groups. According to revised Jones criteria, the diagnosis of rheumatic fever can be made when two of the major criteria, or one major criterion plus two minor criteria, are present along with evidence of streptococcal infection: elevated or rising antistreptolysin O titre or DNAase. Exceptions are chorea and indolent carditis, each of which by itself can indicate rheumatic fever. An April 2013 review article in the Indian Journal of Medical Research stated that echocardiographic and Doppler (E & D) studies, despite some reservations about their utility, have identified a massive burden of rheumatic heart disease, which suggests the inadequacy of the 1992 Jones' criteria. E & D studies have identified subclinical carditis in patients with acute rheumatic fever, as well as in follow-ups of rheumatic heart disease patients who initially presented as having isolated cases of Sydenham's chorea. Signs of a preceding streptococcal infection include: recent scarlet fever, raised antistreptolysin O or other streptococcal antibody titre, or positive throat culture.

Major criteria

  • Polyarthritis: A temporary migrating inflammation of the large joints, usually starting in the legs and migrating upwards.
  • Carditis: Inflammation of the heart muscle (myocarditis) which can manifest as congestive heart failure with shortness of breath, pericarditis with a rub, or a new heart murmur.
  • Subcutaneous nodules: Painless, firm collections of collagen fibers over bones or tendons. They commonly appear on the back of the wrist, the outside elbow, and the front of the knees.
  • Erythema marginatum: A long-lasting reddish rash that begins on the trunk or arms as macules, which spread outward and clear in the middle to form rings, which continue to spread and coalesce with other rings, ultimately taking on a snake-like appearance. This rash typically spares the face and is made worse with heat.
  • Sydenham's chorea (St. Vitus' dance): A characteristic series of rapid movements without purpose of the face and arms. This can occur very late in the disease for at least three months from onset of infection.

Minor criteria

  • Fever of 38.2–38.9 °C (100.8–102.0 °F)
  • Arthralgia: Joint pain without swelling (Cannot be included if polyarthritis is present as a major symptom)
  • Raised erythrocyte sedimentation rate or C reactive protein
  • Leukocytosis
  • ECG showing features of heart block, such as a prolonged PR interval (Cannot be included if carditis is present as a major symptom)
  • Previous episode of rheumatic fever or inactive heart disease

Prognosis

In some cases the inflammation caused by rheumatic fever may cause long term complications. 

Rheumatic heart disease is permanent damage to the heart caused by the inflammation incurred during rheumatic fever. Heart problems are most common with the mitral valve, but the other valves may be affected. Heart damage can result in valve stenosis, valve regurgitation, or damage to the heart muscle. Damage to the mitral valve, other valves, or other heart tissues can cause problems with the heart later in life such as: atrial fibrilation, or heart failure. 

Treatment

The management of acute rheumatic fever is geared toward the reduction of inflammation with anti-inflammatory medications such as aspirin or corticosteroids. Individuals with positive cultures for strep throat should also be treated with antibiotics. Aspirin is the drug of choice and should be given at high doses of 100 mg/kg/day. One should watch for side effects like gastritis and salicylate poisoning. In children and teenagers, the use of aspirin and aspirin-containing products can be associated with Reye's syndrome, a serious and potentially deadly condition. The risks, benefits and alternative treatments must always be considered when administering aspirin and aspirin-containing products in children and teenagers. Ibuprofen for pain and discomfort and corticosteroids for moderate to severe inflammatory reactions manifested by rheumatic fever should be considered in children and teenagers. Steroids are reserved for cases where there is evidence of involvement of heart. The use of steroids may prevent further scarring of tissue and may prevent development of sequelae such as mitral stenosis. Monthly injections of longacting penicillin must be given for a period of five years in patients having one attack of rheumatic fever. If there is evidence of carditis, the length of therapy may be up to 40 years. Another important cornerstone in treating rheumatic fever includes the continual use of low-dose antibiotics (such as penicillin, sulfadiazine, or erythromycin) to prevent recurrence.

Vaccine

No vaccines are currently available to protect against S. pyogenes infection, although there has been research into the development of one. Difficulties in developing a vaccine include the wide variety of strains of S. pyogenes present in the environment and the large amount of time and people that will be needed for appropriate trials for safety and efficacy of the vaccine.

Infection

People with positive cultures for Streptococcus pyogenes should be treated with penicillin as long as allergy is not present. This treatment will not alter the course of the acute disease.

The most appropriate treatment stated in the Oxford Handbook of Clinical Medicine for rheumatic fever is benzathine benzylpenicillin.

Inflammation

While corticosteroids are often used, evidence to support this is poor. Salicylates are useful for pain.

Heart failure

Some patients develop significant carditis which manifests as congestive heart failure. This requires the usual treatment for heart failure: ACE inhibitors, diuretics, beta blockers, and digoxin. Unlike normal heart failure, rheumatic heart failure responds well to corticosteroids.