Summary

Acute rheumatic fever (ARF) is an inflammatory disease that can develop after a Group A streptococcal (GAS) throat infection, affecting the heart, joints, skin, and brain. Think of it as your immune system overreacting to a strep throat infection—instead of just fighting the bacteria, it mistakenly attacks your own tissues, especially the heart valves, joints, and brain. This condition is most common in children and young adults (5-15 years) and is a leading cause of acquired heart disease in young people worldwide, especially in developing countries. The key to management is recognizing the condition (using the Jones criteria—fever, arthritis, carditis, chorea, skin changes), confirming recent streptococcal infection (throat swab, ASO titers), providing anti-inflammatory treatment (aspirin, corticosteroids if severe carditis), treating the streptococcal infection (penicillin), and preventing recurrence (long-term penicillin prophylaxis). Most patients recover, but carditis can cause permanent heart valve damage (rheumatic heart disease), which is why prevention and early treatment are crucial.

Key Facts

• Definition: Inflammatory disease following Group A streptococcal infection, affecting heart, joints, skin, brain
• Incidence: ~1-3 per 100,000 in developed countries, much higher in developing countries
• Mortality: Low (<1%) unless severe carditis
• Peak age: Children and young adults (5-15 years)
• Critical feature: Follows strep throat, affects multiple systems (heart, joints, skin, brain)
• Key investigation: Jones criteria, ASO titers, throat swab, echocardiography
• First-line treatment: Anti-inflammatories (aspirin), antibiotics (penicillin), bed rest

Clinical Pearls

"Think of it after strep throat" — ARF typically develops 2-4 weeks after a Group A streptococcal throat infection. Always ask about recent sore throat, especially in children.

"Jones criteria guide diagnosis" — The Jones criteria (major: carditis, polyarthritis, chorea, erythema marginatum, subcutaneous nodules; minor: fever, arthralgia, elevated acute phase reactants, prolonged PR interval) help diagnose ARF. Need 2 major or 1 major + 2 minor + evidence of recent GAS infection.

"Carditis is the most serious" — Carditis (inflammation of the heart) is the most serious manifestation and can cause permanent heart valve damage (rheumatic heart disease). Always check for carditis with echocardiography.

"Prevention is key" — Long-term penicillin prophylaxis prevents recurrence and further heart damage. This is essential for patients who have had ARF.

Why This Matters Clinically

ARF is a preventable condition that can cause permanent heart valve damage if not recognized and treated early. Early recognition (especially after strep throat), proper treatment (anti-inflammatories, antibiotics), and long-term prophylaxis (to prevent recurrence) are essential to prevent rheumatic heart disease. This is a condition that pediatricians, cardiologists, and primary care clinicians need to recognize, especially in high-risk populations.

Structured Approach: ABCDE

A - Airway

• Assessment: Usually patent
• Action: Secure if compromised

B - Breathing

• Look: May have difficulty breathing (if heart failure)
• Listen: May have crackles (pulmonary edema)
• Measure: SpO2 (usually normal, may be low if heart failure)
• Action: Support if needed, oxygen if needed

C - Circulation

• Look: May have signs of heart failure (elevated JVP, peripheral edema)
• Feel: Pulse (may be fast), BP (usually normal)
• Listen: Heart sounds (new murmur, may have S3, pericardial rub)
• Measure: BP (usually normal), HR (may be high)
• Action: Monitor if carditis

D - Disability

• Assessment: May have chorea (involuntary movements)
• Action: Assess if chorea present

E - Exposure

• Look: Joint examination, skin examination
• Feel: Joints (swelling, tenderness), skin (rash, nodules)
• Action: Complete examination

Specific Examination Findings

Cardiovascular Examination:

• JVP: May be elevated (if heart failure)
• Heart sounds:
  • New murmur: Mitral or aortic regurgitation (carditis)
  • S3: Heart failure
  • Pericardial rub: Pericarditis
• Peripheral pulses: Usually normal

Musculoskeletal Examination:

• Joints: Swelling, tenderness (large joints: knees, ankles, elbows, wrists)
• Migratory: Moves from joint to joint
• Range of motion: Limited due to pain

Neurological Examination (If Chorea):

• Involuntary movements: Chorea (dance-like movements)
• Emotional lability: Mood changes
• Coordination: May be affected

Skin Examination:

• Erythema marginatum: Pink rings on trunk/limbs (if present)
• Subcutaneous nodules: Firm nodules over bony prominences (if present)

Special Tests

Natural History (Without Treatment)

Untreated ARF:

• Recovery: Most recover from acute episode
• Heart damage: Carditis can cause permanent damage
• Recurrence: High risk without prophylaxis
• Rheumatic heart disease: 30-50% if carditis

Outcomes with Treatment

Factors Affecting Outcomes:

Good Prognosis:

• Early treatment: Better outcomes
• No carditis: Usually no permanent damage
• Prophylaxis compliance: Prevents recurrence
• Mild cases: Usually recover completely

Poor Prognosis:

• Severe carditis: Higher risk of permanent damage
• No prophylaxis: High risk of recurrence
• Recurrent episodes: More heart damage
• Late diagnosis: May have more damage

Prognostic Factors

Key Guidelines

1. WHO Guidelines (2004) — Rheumatic fever and rheumatic heart disease. World Health Organization

Key Recommendations:

• Jones criteria for diagnosis
• Penicillin for treatment and prophylaxis
• Evidence Level: 1A

2. AHA Guidelines (2015) — Prevention of rheumatic fever. American Heart Association

Key Recommendations:

• Similar to WHO
• Evidence Level: 1A

Landmark Trials

Multiple studies on penicillin prophylaxis, treatment.

Evidence Strength

What is Acute Rheumatic Fever?

Acute rheumatic fever (ARF) is an inflammatory disease that can develop after a strep throat infection, affecting your heart, joints, skin, and brain. Think of it as your immune system overreacting to a strep throat infection—instead of just fighting the bacteria, it mistakenly attacks your own tissues, especially your heart valves, joints, and brain.

In simple terms: After a strep throat infection, your immune system can mistakenly attack your own body, especially your heart. This can cause permanent heart damage if not treated, but with proper treatment and prevention, most people do well.

Why does it matter?

ARF can cause permanent heart valve damage (rheumatic heart disease) if not recognized and treated early. Early recognition (especially after strep throat), proper treatment (anti-inflammatories, antibiotics), and long-term prophylaxis (to prevent recurrence) are essential to prevent heart damage. The good news? With proper treatment and prevention, most people do well.

Think of it like this: It's like your immune system getting confused after a strep throat and attacking your own body—with the right treatment and prevention, you can prevent permanent damage.

How is it treated?

1. Treat the Strep Infection:

• Antibiotics: You'll get penicillin (or alternative if allergic) to treat the strep infection
• Duration: Usually 10 days
• Why: To eradicate the infection

2. Anti-Inflammatory Treatment:

• Aspirin or steroids: You'll get medicines to reduce inflammation
• If no heart involvement: Aspirin
• If heart involvement: Steroids (prednisolone)
• Duration: Usually 2-6 weeks
• Why: To reduce inflammation and prevent damage

3. Supportive Care:

• Bed rest: Especially if your heart is affected
• Symptom management: Pain, fever
• Heart failure treatment: If your heart is affected

4. Long-Term Prevention (Most Important):

• Penicillin: You'll need to take penicillin regularly (usually monthly injection or daily tablets) for many years (until age 21 or 10 years, whichever longer)
• Why: To prevent another strep infection, which could cause another episode of ARF and more heart damage
• This is essential: Without this, you're at high risk of recurrence and more heart damage

The goal: Treat the current episode, prevent recurrence, and prevent permanent heart damage.

What to expect

Recovery:

• Acute episode: Usually recovers within weeks to months
• Symptoms: Should improve with treatment
• Heart: May have permanent damage if your heart was affected (rheumatic heart disease)

After Treatment:

• Prophylaxis: You'll need to take penicillin regularly for many years (this is essential)
• Follow-up: Regular follow-up to monitor your heart
• Lifestyle: Usually can live normally, but need to take prophylaxis

Recovery Time:

• Acute episode: Usually weeks to months
• Long-term: Need prophylaxis for many years
• Heart damage: May be permanent if your heart was affected

When to seek help

See your doctor if:

• You've had a recent strep throat and develop fever, joint pain, or other symptoms
• You have symptoms that concern you
• You have a known history of ARF and develop symptoms

Call 999 (or your emergency number) immediately if:

• You have severe chest pain or difficulty breathing
• You feel very unwell
• You have symptoms that concern you

Remember: If you've had a recent strep throat (especially if untreated) and develop fever, joint pain, or other symptoms, see your doctor. ARF can cause permanent heart damage if not treated. Also, if you've had ARF before, it's essential to take your penicillin prophylaxis regularly to prevent recurrence and further heart damage.