Clinical reasoning notes. Rheumatic heart disease is the commonest cause of valve disease in young people across much of Africa and Asia.
The reasoning chain: was there a recent group A streptococcal infection → do the Jones criteria fit → how badly is the heart involved → treat the attack, then prevent the next one → follow the valves for life.
1. What it is
Acute rheumatic fever (ARF) is an autoimmune reaction, 2 to 4 weeks after a group A streptococcal throat infection (and in some settings skin infection), mostly in children 5 to 15 years old. Antibodies against the streptococcus cross-react with heart, joint, brain and skin tissue. Repeated attacks scar the heart valves and cause rheumatic heart disease (RHD), most often mitral stenosis, then mitral regurgitation and aortic valve disease.
2. Jones criteria (2015) for the first attack
Evidence of a preceding group A streptococcal infection (raised ASO or anti-DNase B, positive throat culture or rapid antigen test, or recent scarlet fever) plus:
- 2 major criteria, or
- 1 major and 2 minor criteria.
| Major criteria | Minor criteria |
|---|---|
| Carditis (including silent carditis seen on echocardiography) | Fever |
| Arthritis (a migratory polyarthritis of large joints) | Arthralgia (joint pain without swelling) |
| Sydenham chorea (jerky, purposeless movements) | Raised ESR or CRP |
| Erythema marginatum (pink rings on the trunk, not the face) | Prolonged PR interval on ECG |
| Subcutaneous nodules (painless, over bony prominences) |
In high-risk populations the thresholds for fever, joint involvement and inflammatory markers are lower, and subclinical carditis on echocardiography counts as a major criterion.
3. Investigate with purpose
| Test | The question it answers |
|---|---|
| ASO titre, anti-DNase B | Was there a recent streptococcal infection? |
| ESR and CRP | Is there systemic inflammation (a minor criterion)? |
| ECG | Is the PR interval prolonged? Any heart block? |
| Echocardiography | Is there valve disease or silent carditis? How severe is it? |
| Blood cultures | Is this endocarditis instead? |
| Throat swab | Is the organism still there? |
4. Treatment
- Rest while there is carditis, and treat heart failure if present.
- Eradicate the streptococcus with a course of penicillin (a macrolide if penicillin-allergic).
- Anti-inflammatory therapy: aspirin or another anti-inflammatory for arthritis; corticosteroids for severe carditis.
- Chorea: reassurance and, if severe, a drug such as valproate or carbamazepine.
5. Secondary prevention: the part that saves hearts
Long-term penicillin prophylaxis (a monthly injection of benzathine penicillin, or oral penicillin) prevents recurrences and further valve damage. The duration depends on carditis and valve disease: at least 5 years or until age 21 for those without carditis, longer if the heart is involved, and often for life in severe disease.
Primary prevention: treat streptococcal sore throats properly.
6. Complications
Heart failure, mitral stenosis with atrial fibrillation and stroke, infective endocarditis, and in pregnancy a risk of decompensation. Severe valve disease needs balloon valvotomy or valve surgery.
7. Where tests can mislead
- A normal ASO does not exclude ARF (it may be low late in the illness or after starting antibiotics): repeat it, and check anti-DNase B.
- Arthritis improves within hours of aspirin, which can hide the diagnosis if given before you have seen the joints.
- Silent carditis can only be found by echocardiography.
Examiner questions
- State the Jones criteria for the first attack of rheumatic fever.
- Why does the patient need long-term penicillin?
- Which valve is most often affected in rheumatic heart disease, and what murmur results?
Practice questions
1. A 9-year-old has a migrating polyarthritis, fever and a raised ASO two weeks after a sore throat. The diagnosis needs a second major criterion. Which is one? A. Raised ESR · B. Carditis · C. Arthralgia · D. Fever · E. Prolonged PR interval Answer: B. ESR, arthralgia, fever and a long PR interval are minor criteria.
2. The valve most often damaged in rheumatic heart disease is: A. Tricuspid · B. Pulmonary · C. Mitral · D. Aortic · E. Eustachian Answer: C.
3. The aim of long-term penicillin prophylaxis after rheumatic fever is to: A. Treat chorea · B. Prevent recurrent attacks and further valve damage · C. Reduce fever · D. Treat arthritis · E. Cure existing valve disease Answer: B.