Add a suitable educational image of rheumatic fever, streptococcal infection and heart-valve damage here.
Learning objectives
- Define acute rheumatic fever (ARF) and distinguish it from rheumatic heart disease (RHD).
- Explain the relationship between group A Streptococcus (GAS) infection, autoimmune inflammation and valve damage.
- Identify risk factors, clinical manifestations and complications in children, adolescents and adults.
- Apply the revised Jones criteria in a structured way while recognising that diagnosis requires clinical judgement and local guidance.
- Describe the investigations used to confirm preceding GAS infection, identify carditis and exclude important differentials.
- Outline acute medical treatment, management of heart failure and chorea, and long-term secondary prophylaxis.
- Plan safe, holistic nursing care, patient education, follow-up and prevention of recurrence.
1. Definitions and key distinctions
| Term | Meaning | Clinical significance |
|---|---|---|
| Group A Streptococcus (GAS) | Streptococcus pyogenes, a beta-haemolytic bacterium that can cause pharyngitis and some skin infections. | Prompt recognition and treatment of susceptible infections reduces the risk of ARF. |
| Acute rheumatic fever | A delayed, immune-mediated inflammatory syndrome that usually occurs after GAS infection of the throat and, in some settings, superficial skin infection. | It affects several organs, especially joints, heart, skin and the central nervous system. It is not the same as active bacterial infection of the heart. |
| Rheumatic carditis | Inflammation of one or more layers of the heart during ARF; valvulitis is particularly important. | It may be clinical or subclinical on examination and may cause acute heart failure. |
| Rheumatic heart disease | Chronic structural and functional damage, most often scarring and deformity of heart valves, after one or more ARF episodes. | Valve disease can cause stenosis or regurgitation, arrhythmias, pulmonary hypertension, heart failure, stroke and pregnancy complications. |
2. Epidemiology and public-health importance
The first episode commonly appears in school-age children, especially between 5 and 14 years, but recurrent disease and established valve disease may be seen in adolescents, young adults and pregnant women. The burden is greatest where overcrowding, poverty, delayed access to care, shortages of diagnostic services and interrupted antibiotic courses make GAS infections difficult to treat. Rheumatic fever and RHD remain important in sub-Saharan Africa and other underserved regions.
The World Health Organization describes RF/RHD as a preventable public-health problem. RHD often becomes clinically obvious years after the initial inflammatory episode, when a young person presents with exertional dyspnoea, a murmur, arrhythmia, stroke or heart failure. Prevention therefore requires both early treatment of GAS infection and reliable long-term follow-up after ARF.
- Children and adolescents may have repeated exposure to GAS in crowded homes, schools, boarding facilities or communities.
- Families may interpret a sore throat as minor, use leftover medicines or stop antibiotics when symptoms improve.
- Limited access to throat testing, echocardiography and specialist review can delay diagnosis.
- Pregnancy increases circulatory demand; previously silent valve disease may decompensate during pregnancy, labour or the puerperium.
- Every missed prophylaxis dose creates an opportunity for another GAS infection and additional valve injury.
3. Cause and pathophysiology
3.1 The initiating infection
ARF is a post-infectious complication of GAS. The typical trigger is untreated or inadequately treated GAS pharyngitis; WHO guidance also recognises a possible contribution from superficial skin and skin-structure infection. ARF is not caused by every sore throat: viral pharyngitis and non-rheumatogenic bacteria do not produce the same syndrome.
3.2 Immune cross-reaction
After the infection, the immune system produces antibodies and T-cell responses against streptococcal antigens. In susceptible people, some microbial components resemble proteins in human tissues. This molecular mimicry can cause cross-reactive inflammation in the heart valves, myocardium, pericardium, joints, skin and basal ganglia. The organism may already be gone when the inflammatory illness begins.
3.3 From ARF to valve damage
- GAS infection occurs, often with a sore throat or skin lesion.
- A latent period follows; inflammatory manifestations usually appear weeks later.
- Immune-mediated inflammation causes arthritis, carditis, rash, nodules or chorea.
- Repeated inflammation thickens, shortens and fuses valve leaflets and chordae.
- Chronic regurgitation or stenosis increases cardiac workload and may eventually cause atrial fibrillation, pulmonary hypertension or heart failure.
4. Risk factors and protective factors
| Risk factor | How it increases risk | Practical nursing response |
|---|---|---|
| Overcrowding and poor ventilation | Facilitates spread of GAS respiratory infection. | Teach cough etiquette, hand hygiene, ventilation and early assessment of sore throat outbreaks. |
| Poverty or difficult transport | Delays consultation and makes repeat injections or review difficult. | Plan appointments around transport, link the family to community services and document missed visits promptly. |
| Incomplete antibiotic course | May fail to eradicate GAS and encourages recurrence. | Explain the reason for the full prescribed course and assess barriers rather than blaming the patient. |
| Previous ARF or RHD | Substantially increases risk of recurrence and progression. | Maintain a prophylaxis record, use reminders and verify every dose at each contact. |
| Limited health literacy | Families may not connect a sore throat with future valve disease. | Use plain language, teach-back and written or pictorial instructions. |
| Family/community protective factors | Early attendance and reliable treatment reduce exposure and recurrence. | Involve caregivers, school health teams and community health workers in prevention. |
5. Clinical manifestations
5.1 General symptoms
- Fever, malaise, fatigue, anorexia and reduced activity.
- Recent sore throat, tonsillitis, tender neck nodes or a recent superficial skin infection.
- Pallor or weight loss when disease is prolonged or heart failure is present.
5.2 Migratory arthritis
Classically, large joints such as the knees, ankles, elbows and wrists become painful, swollen, warm and restricted. The inflammation appears to move: one joint improves as another becomes acutely painful. The pain can be severe and may prevent walking. In low-risk populations, polyarthritis is the major joint manifestation in the Jones criteria; in moderate- or high-risk settings, monoarthritis or polyarthralgia may contribute according to the applicable guideline.
5.3 Carditis
- Tachycardia out of proportion to fever or persisting during sleep.
- New murmur, especially a pansystolic murmur of mitral regurgitation or an early diastolic murmur of aortic regurgitation.
- Gallop rhythm, muffled heart sounds, pericardial rub or chest discomfort.
- Breathlessness, orthopnoea, paroxysmal nocturnal dyspnoea, cough, pulmonary crackles, hepatomegaly or peripheral oedema suggesting heart failure.
- Arrhythmias or conduction abnormalities, including a prolonged PR interval.
- Subclinical carditis found by Doppler echocardiography even when no murmur is heard.
5.4 Sydenham chorea
Chorea is a delayed neurological manifestation caused by basal-ganglia involvement. The patient may have irregular, purposeless movements, poor handwriting, clumsiness, facial grimacing, tongue movements, hypotonia, emotional lability, anxiety or difficulty concentrating. Symptoms may worsen with stress and disappear during sleep. Protect the patient from injury and embarrassment; do not interpret the movements as deliberate behaviour.
5.5 Skin and subcutaneous manifestations
- Erythema marginatum: transient, non-itchy, ring-shaped pink or red lesions with central clearing, usually on the trunk or proximal limbs; the face is usually spared.
- Subcutaneous nodules: small, firm, painless nodules over bony prominences or tendons, often associated with significant carditis.
5.6 Features of established RHD
Exertional dyspnoea, orthopnoea, fatigue, palpitations, syncope, chest discomfort, oedema, recurrent chest infections, haemoptysis or stroke-like symptoms may signal chronic valve disease. In pregnancy, breathlessness, inability to lie flat, cyanosis, fainting or rapid weight gain requires urgent assessment.
6. Diagnosis: using the revised Jones criteria
There is no single definitive test for ARF. Diagnosis is clinical and should combine the Jones criteria, evidence of preceding GAS infection, echocardiography and exclusion of more likely conditions. The threshold differs between low-risk and moderate/high-risk populations, so students must follow the current national or facility guideline used in Uganda.
| Jones domain | Major manifestations | Minor manifestations |
|---|---|---|
| Joint | Polyarthritis in low-risk populations; arthritis involving one or more joints may qualify in moderate/high-risk populations according to the guideline. | Polyarthralgia in low-risk populations; monoarthralgia may be used in moderate/high-risk populations where specified. |
| Heart | Clinical or subclinical carditis. | Prolonged PR interval on ECG, provided it is not counted as a minor criterion when carditis is already used as a major manifestation. |
| Systemic inflammation | Not a major criterion. | Fever and elevated inflammatory markers such as ESR or CRP using the thresholds in the current criteria and local guideline. |
| Skin and nervous system | Sydenham chorea; erythema marginatum; subcutaneous nodules. | Not usually counted as separate minor criteria. |
Evidence of preceding GAS infection
- Positive throat culture or rapid antigen detection test when available and appropriate.
- Elevated or rising antistreptolysin O (ASO) or anti-DNase B antibody titres.
- A recent, clinically documented GAS infection treated or untreated.
Antibody titres demonstrate recent exposure, not necessarily active infection, and a single low result does not rule out ARF. Do not count the same joint manifestation twice; for example, arthritis used as a major criterion cannot also be counted as arthralgia as a minor criterion.
7. Assessment and investigations
7.1 Focused history
- Onset, progression and migration of joint pain or swelling.
- Sore throat, fever, skin sores, swollen tonsils, contact with a similar illness and antibiotics received during the preceding weeks.
- Dyspnoea, orthopnoea, nocturnal cough, chest pain, palpitations, syncope, reduced exercise tolerance and oedema.
- Abnormal movements, weakness, handwriting change, emotional change, sleep pattern and school performance.
- Previous ARF/RHD, prophylaxis dates, missed doses, drug allergy and adverse reactions.
- Pregnancy possibility, comorbidities, current medicines, family support, transport and financial barriers.
7.2 Physical examination
- Record temperature, pulse, respiratory rate, blood pressure, oxygen saturation, weight and general appearance.
- Inspect and palpate all large and small joints; document swelling, heat, range of motion and functional limitation.
- Assess pulse character and rhythm, capillary refill, jugular venous pressure, precordium, heart sounds and murmurs.
- Look for respiratory distress, crackles, hepatomegaly, ascites, dependent oedema and cool extremities.
- Perform a neurological examination: mental state, tone, power, coordination, gait, speech and involuntary movements.
- Inspect skin for erythema marginatum, nodules, infection sites and pressure or injury from uncontrolled movements.
7.3 Useful investigations
| Investigation | What it helps assess | Important limitation or nursing point |
|---|---|---|
| Throat culture or rapid GAS test | Evidence of current or recent GAS infection. | A negative result after the infection has resolved does not exclude ARF; collect correctly and document antibiotics already taken. |
| ASO and anti-DNase B titres | Serological evidence of recent streptococcal exposure. | Interpret with timing, local reference range and clinical findings; titres may be absent or less helpful in some skin infections. |
| Full blood count | Anaemia, leukocytosis or alternative diagnoses. | Non-specific; correlate with the full presentation. |
| ESR and CRP | Systemic inflammation and response to treatment. | Raised values are not specific to ARF; fever, infection and other inflammatory illnesses can also elevate them. |
| ECG | PR prolongation, arrhythmia, conduction disease and other cardiac effects. | Interpret for age and heart rate; repeat if symptoms change. |
| Echocardiography with Doppler | Clinical or subclinical carditis, valve regurgitation, stenosis, chamber size and ventricular function. | WHO/CDC guidance supports routine echo/Doppler in suspected or confirmed ARF where available; arrange referral without waiting for a murmur. |
| Chest radiograph | Cardiomegaly, pulmonary congestion or alternative chest disease when clinically indicated. | Use clinical judgement and pregnancy precautions. |
| Blood cultures or other tests | Exclude infective endocarditis, sepsis or other mimics when indicated. | Collect before antibiotics when possible if sepsis/endocarditis is suspected, without delaying resuscitation. |
8. Differential diagnoses
Rheumatic fever should not be diagnosed from fever and joint pain alone. Consider and investigate conditions that may look similar:
- Septic arthritis, osteomyelitis or disseminated bacterial infection.
- Juvenile idiopathic arthritis, rheumatoid arthritis and systemic lupus erythematosus.
- Viral myocarditis, infective endocarditis and Kawasaki disease where clinically relevant.
- Malaria, tuberculosis, HIV-associated disease and other infections common in the local setting.
- Leukaemia or other malignancy when there is pallor, bruising, weight loss, persistent bone pain or abnormal blood counts.
- Gout, reactive arthritis, serum sickness and other inflammatory or drug-related syndromes.
- For abnormal movements: epilepsy, drug-induced movement disorder, Wilson disease and other neurological causes.
9. Immediate and acute medical management
Management must be individualised by the prescriber and guided by the current Uganda Ministry of Health or facility protocol. The principles below are for study and nursing planning; they do not replace a prescription.
9.1 First assessment and stabilisation
- Use an ABCDE approach for any patient with respiratory distress, shock, altered consciousness or severe carditis.
- Place the patient in a position that supports breathing; provide oxygen if hypoxaemic or in significant respiratory distress according to protocol.
- Obtain vital signs, weight, pain score, blood glucose when indicated and baseline cardiac/neurological observations.
- Establish appropriate access and collect ordered investigations without delaying urgent treatment.
- Escalate immediately for pulmonary oedema, severe tachycardia, hypotension, cyanosis, syncope, new neurological deficit or suspected sepsis.
9.2 Eradication of GAS
Even when the sore throat has resolved, a patient diagnosed with ARF generally receives an antibiotic regimen to eradicate residual GAS. Penicillin is the usual first-line option unless a true allergy or another contraindication exists. Alternatives are selected by the prescriber according to allergy history, resistance patterns, pregnancy status, age, renal function and local policy. Confirm the medicine, route, dose, timing and documentation, and observe for immediate hypersensitivity after parenteral administration.
9.3 Control of inflammation and pain
- Prescribed anti-inflammatory therapy reduces fever and joint inflammation; monitor pain, mobility, gastrointestinal symptoms, bleeding and renal risk.
- Severe carditis may require corticosteroid therapy or specialist-directed treatment.
- Do not give aspirin, non-steroidal anti-inflammatory drugs or steroids without a prescriber’s decision, age-appropriate safety assessment and a clear indication.
- Use rest, comfortable positioning, gentle handling and non-drug comfort measures alongside prescribed analgesia.
9.4 Carditis and heart failure
Carditis ranges from mild subclinical valve inflammation to severe pancarditis and heart failure. Management may include activity restriction, diuretics, vasodilators, inotropes or other heart-failure medicines as prescribed, with cardiology review. Nurses should monitor response closely because fever, anaemia, infection, fluid overload and arrhythmia can worsen cardiac workload.
9.5 Sydenham chorea
- Provide a quiet, low-stimulation environment and protect from falls, burns, aspiration and accidental injury.
- Allow extra time for communication, eating, washing and examination; use calm reassurance and preserve dignity.
- Use prescribed medicines for severe chorea only under specialist direction, observing sedation, extrapyramidal effects and respiratory status.
- Assess school, family and emotional impact and involve occupational, physiotherapy, psychology or social-work support where available.
10. Secondary prophylaxis and long-term care
After ARF, recurrent GAS infections can trigger further attacks and increase valve damage. Secondary prophylaxis uses regular long-term antibiotics, commonly an intramuscular benzathine penicillin preparation or an approved alternative when indicated. The exact drug, interval and duration depend on previous carditis, valve disease, age, recurrence risk and national guidance. Never stop prophylaxis because the patient feels well without a documented specialist plan.
| Follow-up task | What the nurse should do |
|---|---|
| Prophylaxis record | Document medicine, date, batch/route where required, next due date, reaction and reason for any missed dose. |
| Adherence support | Explore pain, fear of injections, transport, cost, school timetable, stigma and competing family responsibilities; agree on practical solutions. |
| Cardiac surveillance | Review symptoms, murmurs, ECG/echo appointments, exercise tolerance, pregnancy planning and cardiology advice. |
| Recurrence plan | Teach the family to seek prompt care for sore throat, fever, new joint pain, breathlessness, palpitations, chorea or oedema. |
| Continuity | Use a clinic card, community health worker, school nurse or phone reminder system when available; communicate missed appointments early. |
11. Nursing management
11.1 Nursing assessment
- Establish baseline temperature, pulse, rhythm, blood pressure, respiratory rate, oxygen saturation, weight, pain, mobility and functional ability.
- Trend pulse against temperature; persistent tachycardia may signal carditis or heart failure.
- Assess heart sounds, new murmurs, gallop rhythm, peripheral perfusion, lung sounds, oedema, abdominal distension and urine output.
- Record each affected joint, symmetry, warmth, swelling, range of motion and response to treatment.
- Observe for chorea, falls, aspiration, altered speech, emotional distress and ability to complete activities of daily living.
- Check medicine history, antibiotic allergy, pregnancy status where relevant and the family’s understanding of prophylaxis.
11.2 Nursing interventions and rationale
| Intervention | Rationale and practical actions |
|---|---|
| Monitor cardiovascular status | Record observations at the prescribed frequency; report new murmur, gallop, worsening tachycardia, hypotension, cyanosis, chest pain, syncope or increasing breathlessness. |
| Support breathing and circulation | Position for comfort, administer prescribed oxygen/medicines, monitor fluid balance and escalate pulmonary oedema or shock immediately. |
| Promote rest without harmful immobility | During active inflammation, balance cardiac rest and pain relief with gradual, supervised mobilisation as symptoms and medical advice allow. Prevent pressure injury and deconditioning. |
| Manage pain and joints | Use the prescribed anti-inflammatory/analgesic, support painful limbs, provide gentle hygiene and reassess pain and function after intervention. |
| Prevent injury from chorea | Remove hazards, use supervision and appropriate assistance, protect the head and limbs, and assess swallowing before oral intake if movements are severe. |
| Administer antibiotics safely | Verify identity, allergy, medicine, route, dose, timing and documentation; observe for rash, wheeze, facial swelling, hypotension or other anaphylaxis signs and activate emergency care if they occur. |
| Maintain nutrition and hydration | Offer small, frequent meals and assistance when joint pain or chorea interferes with feeding; adjust fluid advice to the cardiac plan and record intake/output. |
| Provide psychological support | Listen to fears about injections, disability, school and stigma; involve caregivers and refer for counselling or social support when needed. |
| Teach before discharge | Use plain language and teach-back: the family should explain the illness, medicines, next prophylaxis date, warning signs and where to return. |
| Coordinate follow-up | Confirm cardiology/echo, prophylaxis and community follow-up; give written dates and communicate any unresolved risk to the receiving team. |
12. Prevention
12.1 Primary prevention
- Assess children and adolescents with clinically significant sore throat or compatible skin infection promptly.
- Use available diagnostic testing and treat suspected or confirmed GAS according to the current local protocol.
- Teach patients to complete the prescribed antibiotic course and avoid sharing leftover antibiotics.
- Improve ventilation, reduce overcrowding where feasible, practise hand hygiene and avoid sharing cups or utensils during acute illness.
- Strengthen community awareness that a sore throat plus fever in a child deserves assessment, especially in areas with previous ARF/RHD.
12.2 Secondary prevention
- Give every scheduled prophylaxis dose and record it in a reliable register.
- Track missed appointments actively rather than waiting for the patient to reappear.
- Provide regular cardiac review and echocardiography when indicated or available.
- Screen for dental infection, anaemia and other conditions that may increase cardiac risk according to local services.
- Discuss contraception and pregnancy planning with patients who have RHD, and arrange specialist review before pregnancy when possible.
13. Complications and prognosis
| Complication | Warning signs | Priority response |
|---|---|---|
| Acute heart failure | Increasing breathlessness, orthopnoea, crackles, gallop rhythm, hepatomegaly, oedema, fatigue or low oxygen saturation. | Urgent ABCDE assessment, oxygen if indicated, senior/cardiology review and prescribed heart-failure management. |
| Progressive valve disease | Persistent murmur, reduced exercise tolerance, palpitations, syncope or recurrent admissions. | Cardiac imaging and specialist follow-up; reinforce prophylaxis. |
| Arrhythmia or thromboembolism | Irregular pulse, sudden weakness, facial droop, speech difficulty or severe headache. | Emergency assessment; follow stroke/arrhythmia protocol. |
| Recurrent ARF | New fever, migratory joint symptoms, chorea or carditis after missed prophylaxis. | Prompt clinical review, investigations and restart/adjust prophylaxis only under the responsible clinician. |
| Injury or aspiration from chorea | Falls, bruising, choking, weight loss or inability to complete self-care. | Safety supervision, swallow assessment and multidisciplinary support. |
| Medication reaction | Urticaria, wheeze, facial swelling, collapse, severe gastrointestinal or bleeding symptoms. | Stop and escalate the suspected medicine according to emergency and prescribing policy. |
14. Worked clinical scenarios
Scenario 1: Migratory arthritis after a sore throat
A 10-year-old has fever and severe left-knee pain. Two days later the knee improves, but the right ankle becomes swollen and painful. The caregiver reports a sore throat three weeks ago and an incomplete antibiotic course. The nurse checks vital signs, assesses all joints, asks about cardiac and neurological symptoms, reports the pattern promptly, prepares ordered inflammatory and streptococcal tests, and avoids counting arthralgia separately if arthritis is accepted as a major criterion.
Scenario 2: Tachycardia and breathlessness
A teenager with suspected ARF has a pulse of 132/min, fast breathing, a new murmur and difficulty lying flat. The nurse recognises possible carditis with heart failure, sits the patient upright, calls for urgent senior review, starts the local ABCDE response, provides prescribed oxygen, prepares access and investigations, and records response to treatment. Waiting for a routine outpatient appointment would be unsafe.
Scenario 3: Chorea and missed injections
A student with previous ARF has irregular hand movements, poor handwriting and two missed prophylaxis appointments because the family could not afford transport. The nurse protects the patient from falls, reduces stimulation, assesses swallowing and emotional wellbeing, checks the prophylaxis record, explores the transport barrier without blame and coordinates a community follow-up plan.
Scenario 4: Penicillin allergy concern
Shortly after a prescribed injection, a patient develops widespread urticaria and wheeze. The nurse stops administration, calls for emergency assistance, assesses airway, breathing and circulation, follows the anaphylaxis protocol, documents the reaction clearly and ensures the allergy is communicated before another antibiotic is prescribed.
Recent GAS infection considered · Heart and joints examined · Evidence organised with Jones criteria · Urgent red flags escalated · Medicines and allergy checks completed · Anti-inflammatory and cardiac care monitored · Tracking of prophylaxis maintained · Instruction with teach-back · Cardiology and community follow-up arranged.
15. Examination and revision questions
- Define acute rheumatic fever and rheumatic heart disease, and state three differences between them.
- Explain how molecular mimicry after GAS infection can damage the heart valves.
- List the five major manifestations and the common minor manifestations in the revised Jones criteria.
- Explain why evidence of preceding GAS infection is important and name two ways to demonstrate it.
- Describe the clinical features of migratory arthritis, carditis, chorea, erythema marginatum and subcutaneous nodules.
- Why is echocardiography useful even when no murmur is heard?
- Outline the immediate nursing priorities for a patient with suspected carditis and pulmonary oedema.
- Describe the purpose of long-term secondary prophylaxis and list four nursing actions that improve adherence.
- Differentiate a likely rheumatic joint presentation from septic arthritis that requires urgent escalation.
- Prepare a discharge teaching plan for a child and caregiver after a first episode of ARF.
Key takeaways
- ARF is an immune-mediated complication of GAS; it is not simply a persistent sore throat.
- Use the revised Jones criteria with evidence of preceding GAS infection, echocardiography and careful exclusion of mimics.
- Carditis may be silent on auscultation; persistent tachycardia, breathlessness or a new murmur requires urgent assessment.
- Every recurrence can add valve damage, making secondary antibiotic prophylaxis and appointment tracking essential.
- Safe nursing care combines clinical monitoring, medication safety, injury prevention, family education and coordinated follow-up.
Suggested references
- World Health Organization. WHO guideline on the prevention and diagnosis of rheumatic fever and rheumatic heart disease. 2024.
- World Health Organization. Rheumatic heart disease fact sheet. Updated 2025.
- Centers for Disease Control and Prevention. Diagnosing Acute Rheumatic Fever: revised Jones criteria and supplemental testing. Updated 2024–2025.
- Current Uganda Ministry of Health guidance and facility protocols for GAS infection, ARF/RHD, antibiotic prophylaxis, anaphylaxis and heart-failure care.