Acute Rheumatic Fever

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Acute rheumatic fever is an illness caused by an immunological reaction to group A streptococcal infection of the throat. The onset of symptoms occurs 1-3 weeks after an untreated or inadequately treated throat infection. The disease occurs mainly in children and adolescents with a peak age of 5-15 years. Acute rheumatic fever often results in lasting damage to heart valves leading to the chronic form, which is known as rheumatic heart disease. 

Individuals who have had acute rheumatic fever previously are more likely to have subsequent episodes. These recurrences may cause further heart valve damage. Following treatment of an acute episode, secondary prophylaxis should be continued for a minimum of 10 years after the recent episode of acute rheumatic fever or until age 21 years (whichever is longer). For those with severe rheumatic heart disease secondary prophylaxis should be continued indefinitely.

Rheumatic heart disease is an important cause of heart failure and premature death. In Ghana, acute rheumatic fever may mimic malaria, typhoid fever and other febrile conditions, while the joint symptoms may mimic sickle cell disease. 

Jones criteria for diagnosis of Acute Rheumatic Fever

For diagnosis of initial acute rheumatic fever:

• Evidence of preceding group A streptococcal infection,

And

• 2 major criteria

Or

• 1 major criterion and 2 minor criteria

For diagnosis of recurrent acute rheumatic fever:

• Evidence of preceding group A streptococcal infection,

And

• 2 major criteria

Or

• 1 major criterion and 2 or 3 minor criteria

(See symptoms and Signs below for major and minor criteria)

Causes

• Group A streptococcal infection 

Symptoms

• Fever 
• Malaise 
• Joint pain which moves from one joint to another (knees, ankles, wrists, elbows)
• Palpitations
• Easy fatigability
• Chest pain 
• Skin rash
• Abnormal body movements (chorea)

Signs

• Fever > 38°C (minor criteria)
• Single joint tenderness and or swelling (minor criteria)
• Rapid heart rate (>100/minute), murmur, heart failure, pericardial rub (suggests carditis) (major criteria)
• Skin rash
• Subcutaneous nodules over bony prominences

Investigations

• FBC 
• ESR > 30 or C-reactive protein > 3 mg/dL (minor criteria)
• Sickling status
• Chest X-ray 
• Throat swab for culture
• Anti-streptolysin O titre 
• 12-lead ECG (prolonged PR interval for age - minor criteria)
• Echocardiogram evidence of carditis (major criteria)

TreatmentTreatment Objectives

• To eradicate streptococcal throat infection
• To suppress inflammatory response
• To prevent recurrent episodes of rheumatic fever and further heart valve damage
• To treat heart failure if co-existent

Non-pharmacological treatment

• Bed rest

Pharmacological treatment 

See sections below

Referral Criteria

• Refer all patients to a paediatrician, physician specialist or cardiologist as necessary for further management.