Rheumatic Fever and Rheumatic Heart Disease: Jones Criteria and Prophylaxis

Acute rheumatic fever is an inflammatory illness that follows a throat infection with group A Streptococcus. Repeated or severe attacks damage the heart valves, causing rheumatic heart disease. It is now rare in high-income countries but remains an important cause of heart disease in children and young adults in South Asia, Africa and other regions.

How it happens

Two to four weeks after a streptococcal sore throat, antibodies against the bacteria cross-react with tissues in the heart, joints, skin and brain (molecular mimicry). The mitral valve is affected most often, followed by the aortic valve.

Diagnosis: the revised Jones criteria

Diagnosis requires evidence of a preceding group A streptococcal infection (raised or rising ASO or anti-DNase B titre, positive throat culture or rapid antigen test) plus:

• Initial episode: 2 major criteria, or 1 major and 2 minor criteria.
• Recurrent episode: 2 major, 1 major and 2 minor, or 3 minor criteria.

Major criteria

• Carditis — clinical or on echocardiography (pancarditis: endocarditis, myocarditis, pericarditis).
• Arthritis — classically a migratory polyarthritis of large joints. In moderate- and high-risk populations, monoarthritis or polyarthralgia can count.
• Sydenham chorea — involuntary, jerky movements, often appearing later.
• Erythema marginatum — pink rings with clear centres on the trunk.
• Subcutaneous nodules — painless, over bony prominences and tendons.

Minor criteria

• Fever.
• Arthralgia.
• Raised ESR or CRP.
• Prolonged PR interval on ECG (if carditis is not counted as a major criterion).

The exact thresholds for fever, ESR and joint findings depend on whether the population is low or moderate-to-high risk (American Heart Association 2015 revision).

Management of acute rheumatic fever

• Antibiotics to eradicate streptococci (for example penicillin).
• Anti-inflammatory treatment for arthritis (for example aspirin or NSAIDs, as directed by specialists).
• Treatment of heart failure if present; corticosteroids are sometimes used for severe carditis.
• Echocardiography for every patient.
• Treatment of chorea if needed.

Secondary prophylaxis — the most important step

To prevent recurrences that cause further valve damage, patients receive long-term antibiotic prophylaxis, usually intramuscular benzathine penicillin G every 3–4 weeks (oral penicillin is an alternative). The duration depends on age, time since the last attack and whether there is carditis or valve disease; it may continue for 10 years or more, sometimes into adulthood or lifelong.

Rheumatic heart disease

• Mitral regurgitation is common in young patients.
• Mitral stenosis develops over years. It causes breathlessness, atrial fibrillation, stroke and pulmonary hypertension, with a mid-diastolic rumbling murmur and a loud first heart sound.
• Aortic valve disease may coexist.
• Severe valve disease needs balloon valvotomy or surgery.
• Pregnancy is high-risk and needs specialist care.

Prevention

Primary prevention means prompt antibiotic treatment of streptococcal sore throat. Better housing, less crowding and access to health care reduce the disease in communities.

Exam pearls

• Remember the major criteria with "J♥NES": Joints, ♥ (carditis), Nodules, Erythema marginatum, Sydenham chorea.
• Chorea may be the only finding and can appear months after the infection.
• The most common long-term valve lesion is mitral stenosis.

For more on valvular heart disease, see the Medicine Companion and our Paediatrics 2.0 Exam Night notes.

Test yourself

5 exam-style single-best-answer questions. Choose your answer, then tap “Show answer”.

1. Which is a MAJOR Jones criterion?

   1. Prolonged PR interval
   2. Arthralgia
   3. Sydenham chorea
   4. Fever
   5. Raised ESR
   Show answer

   Answer: C. Sydenham chorea
   Major: carditis, arthritis, chorea, erythema marginatum, subcutaneous nodules. The others are minor.

2. What else is required to diagnose acute rheumatic fever besides the Jones criteria?

   1. Abnormal chest X-ray
   2. Raised troponin
   3. Evidence of preceding group A streptococcal infection
   4. Positive blood culture
   5. Positive ANA
   Show answer

   Answer: C. Evidence of preceding group A streptococcal infection
   Diagnosis needs evidence of prior group A strep infection (ASO/anti-DNase B, culture or rapid antigen test).

3. What is the standard secondary prophylaxis?

   1. Steroids for 1 year
   2. Oral amoxicillin for 5 days
   3. Daily aspirin
   4. IM benzathine penicillin G every 3–4 weeks
   5. Annual vaccination
   Show answer

   Answer: D. IM benzathine penicillin G every 3–4 weeks
   Long-term benzathine penicillin prevents recurrences that worsen valve damage.

4. Which valve is most commonly affected in rheumatic heart disease?

   1. All equally
   2. Aortic
   3. Pulmonary
   4. Tricuspid
   5. Mitral
   Show answer

   Answer: E. Mitral
   The mitral valve is most often affected, followed by the aortic valve.

5. A mid-diastolic rumbling murmur with a loud S1 in a young woman suggests:

   1. Aortic stenosis
   2. VSD
   3. Aortic regurgitation
   4. Mitral stenosis
   5. Mitral regurgitation
   Show answer

   Answer: D. Mitral stenosis
   Mitral stenosis gives a loud S1 and a low-pitched mid-diastolic murmur, often from rheumatic disease.

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Disclaimer

For education and exam preparation only. This is not medical advice and must not be used to diagnose or treat patients. Always follow current clinical guidelines and the advice of qualified senior colleagues.