CardiologyTier 1Disease (DEADMAN)

Acute rheumatic fever

Description

Autoimmune, post-streptococcal systemic illness - the preventable cause of rheumatic heart disease
Follows group A streptococcal pharyngitis (and possibly skin infection in high-incidence settings) by 1-5 weeks
In Australia, managed under a notifiable disease / register-based framework in high-risk populations

Near-eliminated in high-income settings generally, but Australia has among the highest rates in the world concentrated in Aboriginal and Torres Strait Islander communities (esp. northern/central/remote Australia)
Peak age 5-14 years
Overcrowding, poverty, and limited healthcare access are the structural drivers - not a genetic predisposition alone, though HLA associations exist

Molecular mimicry - antibodies against group A streptococcal M protein cross-react with cardiac myosin, valve tissue, synovium, and basal ganglia
-> pancarditis (endocardium/valves, myocardium, pericardium), synovitis, chorea, erythema marginatum, subcutaneous nodules
Mitral valve is the most commonly and earliest affected (regurgitation acutely; stenosis develops over years with repeated episodes)
Recurrent episodes cause cumulative valve damage -> rheumatic heart disease

Revised/Australian-modified Jones criteria - risk-stratified by population

High-risk population (incidence >=2/100,000 school-age children, or RHD prevalence >=1/1000, including all Aboriginal and Torres Strait Islander Australians) uses lower thresholds than low-risk criteria

	Major	Minor
	Carditis (clinical or echocardiographic - subclinical carditis counts), polyarthritis (or polyarthralgia/monoarthritis in high-risk), chorea, erythema marginatum, subcutaneous nodules	Fever, raised ESR/CRP, polyarthralgia (if not major), prolonged PR interval

Diagnosis (initial episode): 2 major, OR 1 major + 2 minor, plus evidence of preceding GAS infection (ASOT/anti-DNase B titres, throat culture/rapid antigen, or recent scarlet fever)
Echocardiography mandatory in all suspected ARF - subclinical carditis on echo counts as a major criterion even without a clinical murmur
Chorea or indolent carditis alone can establish the diagnosis without evidence of preceding GAS infection (latency too long for serology)
Recurrent ARF - lower threshold (fewer criteria needed) in a patient with confirmed prior ARF/RHD

Acute episode

Treat the streptococcal infection - benzathine penicillin G IM (or oral penicillin course) regardless of throat swab result
Anti-inflammatory therapy: aspirin or NSAID for arthritis/fever; corticosteroids for moderate-severe carditis or significant heart failure
Bed rest during active carditis; treat heart failure if present
Chorea - carbamazepine or valproate if severe/functionally limiting; usually self-limiting

Secondary prophylaxis - the single most important intervention

IM benzathine penicillin G every 3-4 weeks (every 3 weeks in high-risk/high-recurrence settings) - long-acting, most reliable
Alternative: daily oral penicillin V if IM refused/contraindicated (less reliable adherence)
Duration determined by disease severity and time since last episode, per the Australian ARF/RHD guideline:
- No carditis - 10 years after last episode or until age 21 (whichever is longer)
- Mild carditis/healed - 10 years or until age 21
- Moderate-severe carditis/persistent valve disease - up to age 35-40, or lifelong
Register enrolment (state-based ARF/RHD registers) supports adherence tracking and recall

Rheumatic heart disease - the defining long-term complication
Sydenham chorea - may occur without other active features, can be delayed
Recurrent ARF - each episode adds cumulative valve damage; risk highest in the years following the first episode

Acute illness typically resolves over weeks (arthritis fastest, carditis and chorea slower)
Valve damage is cumulative and recurrence-dependent - the entire secondary prophylaxis strategy exists to prevent the next episode, not treat the current one
Untreated/under-prophylaxed disease progresses to rheumatic heart disease, heart failure, atrial fibrillation, endocarditis risk, and premature death
Community-level prevention (primary prophylaxis - treating streptococcal pharyngitis, addressing overcrowding) remains the most effective long-term strategy

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