Acute Rheumatic Fever

Acute Rheumatic Fever (ARF) is an inflammatory disorder of the connective tissue that occurs as a delayed consequence of a group A streptococcal infection, most commonly that of streptococcal pharyngitis. It is most common in children aged 5 to 15. Symptoms usually start two to four weeks after streptococcal infection.

ARF typically involves the joints, heart, skin and brain. Joint symptoms include painful, swollen joints (migratory arthritis), inflammation of the ankles with resulting limping and pain or stiffness in the wrists, elbows, shoulders, hips and knees.

Skin changes may include tiny lumps on the skin's surface (called "erythema marginatum") and a reddish, sometimes slightly painful rash (called "subcutaneous nodules"), and abnormal dermal pigmentation around the wrists and the elbow (also known as the "shirt-sleeve sign").

Heart problems associated with ARF include scarring of heart valves (called "valvular heart disease"), inflammation of heart muscle causing rhythm disturbances (called "carditis") and, rarely, inflammation of the sac containing the heart (called "pericarditis").

ARF can also cause inflammation of the brain (called "sydenham's chorea"), which can lead to emotional and behavioural changes. In severe cases, ARF can cause death.

Risk Factors and Prevention

The risk of developing ARF is primarily influenced by the presence or absence of certain genetic markers called "HLA-DR epitopes." Individuals with these markers are at an increased risk of developing ARF after a streptococcal infection. Other risk factors include inadequate treatment of strep throat, overcrowding, poverty and certain environmental factors.

To prevent the development of ARF, it is important to identify and treat streptococcal infections promptly. Vaccination against streptococcal illnesses is also recommended.

Diagnosis and Treatment

ARF is diagnosed based on the presence of certain symptoms and a positive streptococcal test. The diagnosis is further supported by testing for HLA-DR epitopes. Once ARF is diagnosed, treatment typically involves antibiotics to treat active strep infections, anti-inflammatory drugs and antibiotics for long-term prevention of recurrence.

Treatment also includes observation for signs and symptoms of heart and joint disease, and lifestyle modifications to manage the condition. If valvular heart disease is present, surgery may be indicated.