Rheumatic fever

Rheumatic fever
Other names	Acute rheumatic fever (ARF)
Rheumatic heart disease at autopsy with characteristic findings (thickened mitral valve, thickened chordae tendineae, hypertrophied left ventricular myocardium)
Specialty	Cardiology
Symptoms	Fever, multiple painful joints, involuntary muscle movements, erythema marginatum[1]
Complications	Rheumatic heart disease, heart failure, atrial fibrillation, infection of the valves[1]
Usual onset	2–4 weeks after a streptococcal throat infection, age 5–14 years[2]
Causes	Autoimmune disease triggered by pathogenic strains of Streptococcus[1]
Risk factors	Genetic factors, malnutrition and poverty.[1]
Diagnostic method	Based on symptoms and infection history[3]
Prevention	Prompt antibiotics for Streptococcus infections, improved sanitation[1][4]
Treatment	Prolonged periods of antibiotics, valve replacement surgery, valve repair[1]
Frequency	325,000 children a year[1]
Deaths	319,400 (2015)[5]

Rheumatic fever (RF) is an inflammatory disease that can involve the heart, joints, skin, and brain.^[1] The disease typically develops two to four weeks after a streptococcal throat infection.^[2] Signs and symptoms include fever, multiple painful joints, involuntary muscle movements, and occasionally a characteristic non-itchy rash known as erythema marginatum.^[1] The heart is involved in about half of the cases.^[1] Damage to the heart valves, known as rheumatic heart disease (RHD), usually occurs after repeated attacks but can sometimes occur after one.^[1] The damaged valves may result in heart failure, atrial fibrillation and infection of the valves.^[1]

Rheumatic fever may occur following an infection of the throat by the bacterium Streptococcus pyogenes.^[1] If the infection is left untreated, rheumatic fever occurs in up to three percent of people.^[6] The underlying mechanism is believed to involve the production of antibodies against a person's own tissues.^[1] Due to their genetics, some people are more likely to get the disease when exposed to the bacteria than others.^[1] Other risk factors include malnutrition and poverty.^[1] Diagnosis of RF is often based on the presence of signs and symptoms in combination with evidence of a recent streptococcal infection.^[3]

Treating people who have strep throat with antibiotics, such as penicillin, decreases the risk of developing rheumatic fever.^[4] In order to avoid antibiotic misuse this often involves testing people with sore throats for the infection; however, testing might not be available in the developing world.^[1] Other preventive measures include improved sanitation.^[1] In those with rheumatic fever and rheumatic heart disease, prolonged periods of antibiotics are sometimes recommended.^[1] Gradual return to normal activities may occur following an attack.^[1] Once RHD develops, treatment is more difficult.^[1] Occasionally valve replacement surgery or valve repair is required.^[1] Otherwise complications are treated as usual.^[1]

Rheumatic fever occurs in about 325,000 children each year and about 33.4 million people currently have rheumatic heart disease.^[1][7] Those who develop RF are most often between the ages of 5 and 14,^[1] with 20% of first-time attacks occurring in adults.^[8] The disease is most common in the developing world and among indigenous peoples in the developed world.^[1] In 2015 it resulted in 319,400 deaths down from 374,000 deaths in 1990.^[5][9] Most deaths occur in the developing world where as many as 12.5% of people affected may die each year.^[1] Descriptions of the condition are believed to date back to at least the 5th century BCE in the writings of Hippocrates.^[10] The disease is so named because its symptoms are similar to those of some rheumatic disorders.^[11]