Acute rheumatic fever and rheumatic heart disease

• Acute rheumatic fever (ARF) is a delayed immune reaction to infection with group A Streptococcus (Streptococcus pyogenes), usually starting 1 to 5 weeks after a sore throat or, less certainly, a skin infection. ^[2]
• ARF inflames the heart in about 50% to 70% of first episodes; repeated or severe inflammation leaves permanent valve damage called rheumatic heart disease (RHD). ^[2]
• Diagnosis is clinical, using the 2015 revision of the Jones criteria (American Heart Association), with separate minor cut-offs for low-risk and moderate- or high-risk populations. ^[1][5]
• Arthritis appears in up to 75% of episodes, carditis in 50% to 70%, and Sydenham chorea (the brain-driven dancing movement disorder) often appears days to months later. ^[2][6]
• Eradicate the streptococcus first, then treat symptoms; severe carditis and heart failure must be managed separately from the joints. ^[7][6]
• Secondary antibiotic prophylaxis with benzathine penicillin G, 1,200,000 units intramuscularly every 4 weeks in those 20 kg or more by Australian guidance (27 kg or more by AHA/Seckeler references; 600,000 units below those cut-offs), prevents recurrences and is the single intervention that limits valve damage. ^[7][6]
• More than 15 million people worldwide are estimated to live with RHD, with roughly 282,000 new cases and 233,000 deaths each year. ^{[6] [4]}

By the end of this page you should be able to:

1. Explain why some streptococcal infections lead to ARF and how molecular mimicry damages the valves and brain.
2. Recognise ARF as a multisystem illness and describe carditis, polyarthritis or monoarthritis, Sydenham chorea, erythema marginatum and subcutaneous nodules from their mechanisms.
3. Apply the Jones criteria (initial and recurrent illness) and the low-risk versus moderate- or high-risk thresholds for ESR, CRP, fever and joint involvement.
4. Decide when subclinical carditis on echocardiography counts as a major criterion and what an echo cannot diagnose in ARF. ^[1]
5. Plan acute treatment, including carditis with heart failure, chorea, and the dose and duration of secondary prophylaxis.
6. Explain the World Heart Federation echocardiographic criteria for definite and borderline RHD and why they do not diagnose ARF itself.
7. Recognise special-group, low-resource and prevention issues, including endocarditis prophylaxis and antibiotic reactions.

ARF is not an infection of the heart. The streptococcus is gone by the time the valves inflame. The illness is a case of mistaken identity: the immune response to a streptococcal infection also recognises human tissue, particularly the heart. That makes primary prevention (treating strep throat) and secondary prevention (preventing recurrences) more valuable than any treatment of the established valve damage.

Clinically, ARF is described by what is hit hardest:

• Carditis: valvulitis (and sometimes myocarditis and pericarditis), usually mitral, less commonly aortic. Subclinical carditis means valve disease is seen on echocardiogram even without an audible murmur. ^[2]
• Arthritis: migratory, polyarticular involvement of large joints, or (in higher-risk populations and selected cases) monoarthritis or polyarthralgia. ^[1]
• Sydenham chorea: the neurologic form, with involuntary movements, decreased muscle tone and often psychiatric symptoms, appearing later than the other manifestations. ^[2][6]
• Minor skin and subcutaneous findings: erythema marginatum and subcutaneous nodules. ^[2]

RHD is classified by which valves and how severely. The common long-term patterns are mitral regurgitation, mitral stenosis, and mixed lesions, sometimes with aortic regurgitation. For echocardiographic screening, the 2012 World Heart Federation criteria define definite RHD, borderline RHD (only in people up to 20 years) and normal, but those criteria are for RHD, not for carditis during ARF. ^[4]

A useful clinical ARF classification, used by the Australian guideline, is definite, probable or possible ARF according to how many criteria are met and how certain the diagnosis remains. Definite ARF fulfils the Jones criteria. Probable or possible ARF labels are used when one major or one minor criterion, or streptococcal evidence, is missing but ARF is still considered likely or not yet excluded. ^[7]

ARF and RHD have become rare in affluent populations but remain a serious problem in low- and middle-income countries and in marginalised communities in high-income countries. ^[3][6]

• Age. The first episode most commonly occurs in children 5 to 14 years old. ^[3][2] It is rare in children 3 years and under in the United States, and first-onset is rare in adults over 35 years, although recurrences can occur through adulthood. ^[2]
• Global scale. An older but widely cited estimate is more than 15 million people with RHD, 282,000 new cases and 233,000 deaths annually. ^[6] Recent echocardiographic screening has raised recognised prevalence, because subclinical disease is common. ^[6]
• Outcome. Where measured, carditis occurs in about 50% to 70% of first episodes and arthritis in up to 75%. ^[2] Erythema marginatum and subcutaneous nodules occur in fewer than 10% of episodes, and nodules are most common in patients with carditis. ^[2]
• Regional variation in presentation. In a pooled analysis of ARF reports, carditis occurred in about 60% of cases and arthritis in about 60%, with recurrence rates from 8% to 34% depending on region. ^[6]
• Risk groups. High-risk settings include communities with ARF incidence above 30 per 100,000 children 5 to 14 years per year, or RHD all-age prevalence above 2 per 1000. ^[7] Crowded living conditions, inadequate access to sanitation and health care, and Indigenous or migrant populations carry the greatest burden. ^[7]

A WHO 2024 framing is that recurrent episodes are most common within one year of the first episode, though they can occur across life. ^[3]

The cause is an abnormal immune response to infection with Streptococcus pyogenes group A beta-haemolytic streptococcus, in a susceptible host. ^[3][6]

• Infections that matter. Group A strep pharyngitis is the classic trigger; superficial skin and skin structure infections such as impetigo may also matter, though this link is still being clarified. ^[3][2]
• Timing. The streptococcal infection precedes ARF by 1 to 5 weeks. ^[2]
• Why only some people. Only a minority of the population (less than 10%) develop ARF after streptococcal infection, pointing to host immunogenetics and repeated exposure. ^[7]
• Household and social risk. Crowding, poor sanitation, poor access to health care and lower socioeconomic status raise risk. ^[7] Crowded settings such as daycare, schools, military facilities and shelters increase spread. ^[2]
• Age and history. Risk peaks at 5 to 15 years; a prior attack of ARF raises the risk of further episodes, and that risk is highest in the first few years. ^[2]
• No modifiable individual behaviour beyond seeking care. The key risk is untreated or subclinical streptococcal infection; in about one-third of patients, ARF follows subclinical infections or infections for which no medical care was sought. ^[2]

Group A streptococci express an M-protein on their surface. The immune system builds antibodies and T cells against it. In susceptible people the M-protein resembles, by molecular mimicry, human proteins — especially cardiac myosin and valve tissue, and gangliosides in neurons of the basal ganglia. ^[6]

• Antibodies and inflammatory cells directed at streptococcal antigens cross-react with valve tissue, leading to inflammation of all layers of the heart: valvulitis, sometimes myocarditis, sometimes pericarditis. ^[6]
• Regurgitation occurs through oedematous, inflamed, distorted leaflets; severe acute regurgitation can cause heart failure in the first episode. ^[6]
• With repeated or prolonged inflammation, valves scar, thicken and fuse. Leaflets and chordae shorten, rheumatic stenosis develops, and the valve may become both stenotic and regurgitant. ^[6]
• The mitral valve is involved most often, because damage there is associated with the autoantibody response; the aortic valve is second. Right-sided valves are rarely involved without mitral disease. ^[4][6]

ARF can involve rapid, shifting inflammation in large joints. Anti-inflammatory therapy usually relieves it within 24 to 48 hours, which is one clue distinguishing ARF from other arthritides. ^[6]

Sydenham chorea is caused by cross-reacting antibodies against neuronal gangliosides in the basal ganglia, especially the caudate nucleus. This disrupts pathways that normally inhibit motor impulses, producing chorea, decreased muscle tone, and sometimes behavioural and speech changes. Chorea can appear alone, weeks to months after infection, and may be the sole manifestation of ARF. ^[2][6]

Erythema marginatum and subcutaneous nodules are inflammatory lesions related to the same systemic immune process. ^[6]

ARF usually declares itself with fever and then organ-specific findings. Onset is generally 1 to 5 weeks after group A strep infection. ^[2]

A child may simply have fever and joint pain, with subtly inflamed or only painful joints, producing a limping child. Or the picture may be florid migratory polyarthritis. Carditis may be silent aside from mild echocardiographic valve thickening and MR. RHD often presents later, with heart failure, fatigue on exertion, stroke, endocarditis, or complications of valvular disease. ^[7]

Fever is common in early ARF. Classical descriptions cite 39 degrees C or higher. The 2015 AHA/CDC Jones criteria use a fever minor manifestation threshold of 38.5 degrees C or higher in both risk groups; the Australian 2025 guideline (Noonan et al.) uses 38.0 degrees C or higher in moderate- or high-risk populations and 38.5 degrees C or higher in low-risk populations. ^[6][1]

Carditis is the major cardiac manifestation. Clinical signs include a new murmur suggesting valvular regurgitation, cardiomegaly, congestive heart failure, pericardial effusion, pericardial friction rub, and a prolonged PR interval on the ECG. Subclinical carditis may exist without a murmur, found only on echocardiography or Doppler. ^[2]

• Carditis occurs in 50% to 70% of first episodes, with valvulitis of the mitral valve, or less commonly the aortic valve. ^[2]
• It can be isolated: fever with echocardiographic mitral thickening and mild MR may be the whole clinical picture. ^[7]
• RHD is the most important long-term sequela and can cause arrhythmias, endocarditis, heart failure, pericarditis, stroke and valvular damage. ^[2]

Arthritis is present in up to 75% of episodes. ^[2] The arthritis is typically migratory, affecting large joints such as ankles, elbows, knees and wrists. It can range from arthralgia to painful inflammatory arthritis. Each joint may last a day to a few days before moving. ^[2]

In higher-risk populations, aseptic monoarthritis or polyarthralgia can qualify as major manifestations. ^[1] Joint pain without redness or swelling counts as arthralgia (a minor manifestation, not the arthritis major). ^[6]

Sydenham chorea is a neuropsychiatric major manifestation characterised by abrupt, purposeless, non-rhythmic, involuntary movements, often with muscle weakness and emotional lability. ^[2][6]

• It may occur alone, weeks to months after the streptococcal infection, sometimes long after fever and arthritis have gone. ^[2]
• Fever, elevated inflammatory markers and elevated streptococcal serology may be absent by that time. ^[7]
• MRI studies can show changes in the caudate nucleus and subcortical white matter in some cases. ^[6]
• Chorea can last for months; recurrence or relapse is common, particularly with hormonal changes later in life. ^[6]

Erythema marginatum is a transient erythematous, non-pruritic, non-painful macular rash on the trunk or proximal extremities, extending outward with central clearing and often serpiginous. ^[2][6]

Subcutaneous nodules are firm, painless, variable-sized lumps over extensor joint surfaces, classically small, from a few millimetres to 2 centimetres. They occur in fewer than 10% of episodes and are most common in carditis. ^[2]

For carditis, listen for new murmurs of mitral or aortic regurgitation, assess for heart failure, and obtain ECG for PR prolongation. Do not anchor on a normal examination: subclinical carditis may only be visible on echo. ^[2]

Because ARF mimics many disorders, diagnosis is partly exclusion. ^[2][6]

Investigations serve three purposes: evidence of preceding streptococcal infection, evidence of inflammation, and detecting carditis. ^[1][2]

• Streptococcal evidence. Positive throat culture or rapid streptococcal antigen test, or elevated or rising streptococcal antibody titres (anti-streptolysin O, anti-DNase B or streptokinase). ^[1][6]
• Inflammation. ESR and CRP repeatedly, often to confirm resolution. ^[1][6]
• Cardiac. ECG for PR prolongation; routine echocardiography or Doppler is recommended for all confirmed or suspected ARF, regardless of murmur, because subclinical carditis is common. ^[1][2]
• Blood cultures if endocarditis or infected vegetations must be excluded.
• Initial analgesia during diagnostic uncertainty. Plain paracetamol is preferred, because anti-inflammatory drugs can mask migratory joint symptoms, fever and inflammatory markers before a diagnosis is secure. ^[7]

A rising titre is more informative than a single value, because baseline titres vary by age and region. In patients with chorea or indolent carditis months after infection, streptococcal tests may be negative by the time they are seen. ^[7][6]

Echocardiography is essential to detect carditis and to grade valve disease, but it is not specific for ARF. The WHF definite and borderline RHD categories are designed for RHD screening in people without known ARF, not for diagnosing carditis during ARF and not for diagnosing RHD in someone with a known ARF history. ^[4]

Because ARF is a post-streptococcal condition, a throat culture may be negative even when the infection triggered everything earlier. Treat for possible persisting streptococci regardless. ^[7]

The 2015 Jones revision stratifies patients before applying minor features. ^[1][5]

• Low risk: ARF incidence under 2 per 100,000 school-age children, or all-age RHD prevalence of 1 per 1000 or fewer. ^[1]
• Moderate or high risk: anyone not clearly low risk. ^[1]

This matters because arthritis is major only as polyarthritis in low-risk populations, whereas monoarthritis or polyarthralgia can count in moderate- and high-risk populations; fever and ESR thresholds also differ slightly. ^[1]

A high probability of initial ARF requires evidence of preceding group A streptococcal infection plus 2 major manifestations OR 1 major and 2 minor manifestations. ^[1][6]

A person with prior ARF or RHD is at high risk of recurrence and may be diagnosed with 2 major, 1 major and 2 minor, or 3 minor manifestations, with a documented preceding streptococcal infection. ^[1]

Presumptive diagnosis. In high-incidence settings, and in cases of chorea or indolent chronic carditis appearing months after infection, ARF may be diagnosed without meeting the criteria, using clinical judgement. ^[1] A possible case is one that meets criteria but lacks evidence of preceding streptococcal infection, or has 1 major and 1 minor criterion with evidence of preceding infection. ^[1][6] Definitions of possible or probable ARF differ by guideline; Australian 2025 guidance, for example, uses definite, probable and possible categories with its own duration rules. ^[7]

Both clinical and subclinical carditis (valve disease found only on echocardiogram) count as a major criterion in both risk groups. ^[1][5] Doppler evidence of valve regurgitation supports the diagnosis when audible murmurs may be absent. ^[1][5]

If ARF inflammation leaves chronic valve disease, RHD is graded by echocardiography. The 2012 World Heart Federation criteria, for people up to 20 years of age, define:

• Definite RHD: pathological MR with at least 2 morphological MV features, or MS mean gradient at least 4 mmHg, or pathological AR with at least 2 morphological AV features, or borderline disease of both valves. ^[4]
• Borderline RHD: at least 2 morphological MV features without pathological MR or MS, or pathological MR alone, or pathological AR alone. ^[4]
• Normal: physiological MR or AR not meeting all four criteria, or isolated morphological features without regurgitation or stenosis. ^[4]

Pathological mitral regurgitation requires a jet seen in 2 views, at least 2 cm in length in 1 view by the 2012 WHF criteria (the 2023 WHF criteria adopted in the Australian 2025 guideline use at least 1.5 cm under 30 kg and at least 2.0 cm at 30 kg or more), velocity 3 m/s or higher for a complete envelope, and a pan-systolic jet. Pathological aortic regurgitation requires 2 views, at least 1 cm in length in 1 view, velocity 3 m/s or higher in early diastole, and a pan-diastolic jet. ^[4]

Morphological mitral features include anterior mitral leaflet thickening of at least 3 mm in those up to 19 years by the 2012 WHF criteria (the 2023 WHF criteria state at least 3.0 mm in those up to 20 years), chordal thickening, restricted leaflet motion, and excessive leaflet tip motion. Aortic features include irregular or focal thickening, coaptation defect, restricted leaflet motion and prolapse. ^[4]

Borderline RHD applies only up to 20 years, because mild regurgitation becomes more common with age, and all morphological criteria should be interpreted with the patient's pre-test risk of RHD. ^[4]

Management has four goals: relieve symptoms, mitigate cardiac damage, eradicate the inciting streptococcal infection, and prevent recurrences. Hospitalisation is recommended to confirm the diagnosis and obtain echocardiography, especially in endemic settings. ^[7]

Treat for possible persisting streptococci even if throat cultures are negative. Options include a single intramuscular dose of benzathine penicillin G 1,200,000 units (600,000 units under 20 kg), phenoxymethylpenicillin 500 mg orally (15 mg/kg, to 500 mg maximum) every 12 hours for 10 days, or, for non-severe penicillin reactions, cefalexin 1 g (25 mg/kg, to 1 g) orally every 12 hours for 10 days. ^[7][6] For immediate penicillin hypersensitivity, azithromycin 500 mg (12 mg/kg, to 500 mg) orally daily for 5 days is used. ^[7]

Group A streptococcus remains susceptible to penicillin; it has not been shown to express penicillin resistance. ^[6][7]

Arthritis. The modern first-line anti-inflammatory agents for rheumatic arthritis are NSAIDs, not aspirin. Naproxen 250 to 500 mg orally twice daily (children 10 to 20 mg/kg per day), up to 1250 mg daily, or ibuprofen 200 to 400 mg orally three times daily (children 5 to 10 mg/kg), up to 2400 mg daily, are used. Aspirin 50 to 60 mg/kg per day in 4 to 5 divided doses, escalating up to 80 to 100 mg/kg per day maximum, is second line. Start analgesia high and wean over 1 to 2 weeks; add a proton pump inhibitor for gastric protection if prolonged. ^[7][6]

Carditis. There is no drug that reverses valve damage. For severe carditis, particularly with heart failure, corticosteroids are recommended: prednisolone 1 to 2 mg/kg orally once daily, up to 80 mg maximum. ^[7] Expert opinion favours corticosteroids for carditis associated with heart failure, although meta-analyses of carditis treatment overall did not show clear benefit in preventing later RHD. ^[7] If NSAIDs were started for pericarditis or arthritis, they can be stopped when corticosteroids start. ^[7]

Heart failure. Manage acute carditis like acute heart failure, with furosemide, spironolactone, and ACE inhibitors as directed, and seek cardiology advice early. ^[7] Prednisone for chorea is discussed below.

Chorea. Mild chorea needs no drug. For significant movement disorder, carbamazepine 3.5 to 10 mg/kg per dose orally twice daily is first line, followed by sodium valproate 7.5 to 10 mg/kg per dose orally twice daily. Expect 1 to 2 weeks before response; continue 2 to 4 weeks after movements subside and then withdraw. For very severe chorea or chorea paralytica, add prednisolone 1 to 2 mg/kg daily (maximum 80 mg). ^[7] Intravenous immunoglobulin and plasmapheresis are experimental options. ^[7]

Proton pump inhibitors and screening for latent strongyloidiasis, tuberculosis and hepatitis B are needed before prolonged steroid courses. ^[7]

Anti-inflammatory course. Most episodes resolve within 6 weeks, and 90% within 12 weeks. Inflammatory markers guide stopping treatment; stopping can trigger rebound, sometimes requiring the drugs to be reintroduced. ^[7]

This is the mainstay of ARF and RHD management globally. Intramuscular benzathine penicillin G, 1,200,000 units (20 kg or more by Australian guidance, or 27 kg or more by AHA/Seckeler references) or 600,000 units below those weight cut-offs, is given by deep intramuscular injection every 28 days (every 21 days for selected high-risk groups). ^[7][6] Oral penicillin V 250 mg twice daily is an alternative, and erythromycin 250 mg twice daily for patients with penicillin hypersensitivity. ^[7] The intramuscular route is preferred because it is more effective in trials and because adherence is better. ^[7] Serum penicillin concentrations from regular oral therapy are less protective and adherence problems are frequent. ^{[7] [2]}

Dose the first prophylaxis injection to cover the inciting infection (one dose usually does both). ^[7]

Who needs it and for how long depends on ARF status and RHD severity and is summarised in this table. ^[7]

The AHA no longer recommends routine bacterial endocarditis prophylaxis for rheumatic valve disease unless a prosthetic valve is present. ^[2] Some contemporary local guidance (Australian 2022) still recommends a single amoxicillin 2 g dose (50 mg/kg, to 2 g in children) 60 minutes before high-risk dental procedures, and clindamycin if a recent beta-lactam course was taken. ^[7] Follow local guidelines and cardiology advice. ^[7][2]

Secondary prophylaxis is the one intervention consistently linked to reduced long-term severity of RHD. Moderate to severe disease needs cardiology care, regular echocardiography, and early specialist advice. Heart failure is managed conventionally. Surgery for repair or replacement may be required for severe stenosis or regurgitation. ^[7][6]

Complications span heart, brain, kidneys and pregnancy. ^[2]

• Valve damage: regurgitation, stenosis, or mixed lesions, most commonly mitral; arrhythmia and heart failure. ^[6]
• Endocarditis: RHD is a substrate for infective endocarditis. ^[2][6]
• Stroke: embolic stroke from atrial fibrillation or from vegetation embolisation. ^[2]
• Recurrent ARF: progressive worsening with every recurrence; recurrences occur in roughly 8% to 34% of reported series, highest in high-burden regions. ^[6]
• Chronic paediatric morbidity: growth, nutrition and schooling; RHD is a major cause of acquired heart disease in children and young adults in endemic regions. ^[6][3]
• Mortality: RHD accounts for an important share of cardiovascular deaths in young adults in endemic regions. ^[6][3]

Prognosis is best predicted by valve damage and prevention of recurrences. Uncomplicated ARF without carditis usually resolves within 6 weeks in most and within 12 weeks in 90%, with no permanent heart disease if recurrences are prevented. ^[7] Long-term outcomes depend on three things: how severe the first episode is, how many recurrences occur, and adherence to prophylaxis. ^[7][6]

RHD can improve on echo if prophylaxis is effective and no recurrence occurs, particularly in mild disease. Some patients, however, worsen despite good adherence. ^[7]

This is where most lives are saved. ^[3]

Primary prevention is timely diagnosis and antibiotic treatment of group A streptococcal pharyngitis (and skin infections). Treating streptococcal pharyngitis within 9 days of onset reduces ARF risk by approximately 60% in endemic areas. ^[6] Penicillin V is the drug of choice; benzathine penicillin G intramuscularly is used when adherence with oral therapy is doubtful. ^[6] Cephalosporins can be used in penicillin-allergic patients (up to 5% cross-reactivity), and macrolides in areas with low resistance. ^[6][7]

Primary-prevention doses from a widely cited summary: penicillin V 250 mg orally 2 to 3 times daily if 27 kg or less, or 500 mg 2 to 3 times daily if more than 27 kg, for 10 days; or benzathine penicillin G 600,000 units intramuscularly (27 kg or less) or 1,200,000 units (more than 27 kg), as one dose. ^[6] Amoxicillin 50 mg/kg orally daily is an alternative. ^[6]

Secondary prevention is long-term prophylaxis to stop recurrences. See the table above.

Screening for early RHD uses echocardiography in high-prevalence regions. The World Heart Federation definite and borderline criteria standardise reporting so that screening identifies mild, asymptomatic valve disease early enough to enrol in prophylaxis. ^[4]

Pregnancy. Primary streptococcal infection should be treated in pregnancy; ARF in pregnancy is rare. Women with RHD in pregnancy need pre-conception counselling and specialist care; moderate to severe RHD is a serious pregnancy risk. ^[7]

Children. The incidence is highest from 5 to 15 years; under 3 years ARF is rare and school-age children carry the greatest burden. ^[2][6]

Women of childbearing age with moderate or severe RHD. Secondary prophylaxis may be continued longer in pregnancy and postpartum, specialist advice required. ^[7]

Adults. Adults with prior ARF can have recurrences; about 7% of notifications in some regions are in 35 to 44 year olds, and RHD in pregnancy is important. ^[7][6]

Families and households. Household crowding drives spread; family members need throat surveillance and prompt treatment; households with a recent ARF case should be considered high risk. ^[7]

People with penicillin allergy. Non-severe reactions: cefalexin for treatment and erythromycin for prophylaxis. Immediate (anaphylactic or severe) reactions: azithromycin for treatment and erythromycin for prophylaxis. ^[7] Erythromycin is unreliable where local streptococci are macrolide-resistant. ^[7]

• ARF without reliable echocardiography is still diagnosed by the Jones criteria, with careful clinical judgement. ^[1][6]
• Empirical treatment decisions may be used in high-burden settings, where some providers treat febrile 5 to 15 year olds with sore throat even without laboratory evidence, because penicillin is safe and ARF potentially devastating. ^[6]
• Simple throat surveillance and school-based treatment programmes are among the most cost-effective control measures. ^[6]
• If echo is unavailable, carditis may be missed; refer for echocardiography when feasible. ^[1][2]
• Benzathine penicillin every 3 weeks may be considered in highly endemic areas rather than every 4 weeks. ^[6]
• Programmatic secondary prophylaxis (registers, reminders, pain management, lidocaine with injections) is essential and often determines outcomes. ^[7]
• Benzathine penicillin supply problems may arise (for example product recalls in July 2025), so alternatives and planning matter. ^[2]

• Acute rheumatic fever is an immune reaction to group A streptococcal infection, 1 to 5 weeks later, not a persistent infection of the heart. ^[2]
• Carditis occurs in 50% to 70% of first episodes and is the major prognostic risk; valvular damage becomes RHD. ^[2]
• Sydenham chorea may appear weeks to months later, alone, with negative streptococcal serology. ^[2][7]
• Arthritis occurs in up to 75% of episodes, classically migratory polyarthritis of large joints. ^[2]
• The 2015 AHA Jones criteria require a preceding streptococcal infection plus 2 major OR 1 major and 2 minor manifestations for the first episode. ^[1][5]
• Low-risk and moderate- or high-risk populations have slightly different minor thresholds for ESR, CRP and arthritis. ^[1]
• Clinical and subclinical carditis both count as a major criterion. ^[1]
• Arthralgia does not count in addition to arthritis, and prolonged PR does not count in addition to carditis. ^[1]
• Systemic immune illness also produces erythema marginatum and subcutaneous nodules. ^[2]
• Echocardiography should be done in every suspected or confirmed case. ^[1][2]
• Arthritic pain usually settles within 24 to 48 hours with anti-inflammatory treatment. ^[6]
• Modern first-line arthritis treatment is NSAIDs, not aspirin; prednisolone is reserved for severe carditis and severe chorea. ^[7]
• Rebound of symptoms can occur when anti-inflammatories are stopped; 90% of episodes settle by 12 weeks. ^[7]
• Secondary prophylaxis with benzathine penicillin G every 4 weeks (every 3 weeks in some high-risk settings) is the intervention that changes prognosis. ^{[7][6] [2]}
• Duration of prophylaxis ranges from 12 months for possible ARF without cardiac involvement to lifelong advice after severe RHD or valve surgery. ^[7]
• World Heart Federation echo criteria define definite and borderline RHD for screening, but they do not diagnose ARF. ^[4]
• RHD is estimated at more than 15 million cases worldwide, with about 282,000 new cases and 233,000 deaths each year. ^[6]
• Endocarditis prophylaxis policy differs by guideline; know local practice. ^[2][7]
• Prevention hinges on treating streptococcal pharyngitis within 9 days of symptoms; secondary prophylaxis depends on good adherence. ^[6][7]

Primary prevention and management of group A streptococcal pharyngitis; infective endocarditis; heart failure and valvular heart disease; rheumatic mitral stenosis and mitral regurgitation; chorea and movement disorders; echocardiographic screening for RHD; and national guideline-based care of Indigenous and disadvantaged populations.