Global Considerations
• ARF and RHD: Diseases of poverty, previously
common worldwide.
• Decline in incidence in industrialized nations
due to improved hygiene and antibiotics.
• Persist in developing countries—major cause
of child heart disease and adult mortality.
• Estimated 30–43 million cases globally,
>300,000 deaths annually.
• 95% of ARF and RHD cases in developing
countries.
Epidemiology
• ARF: Primarily affects ages 5–14; initial
episodes rare >30 years.
• Recurrent ARF common in adolescents and
young adults.
• RHD prevalence peaks between 25–40 years.
• RHD more common in females (up to 2:1
ratio).
• Multiple socioeconomic and access barriers
are risk factors.
Pathogenesis – Organism Factors
• Group A Streptococcus (GAS) infection causes
ARF.
• Historically associated with specific M-
serotypes.
• Recent evidence: skin infections also play a
role.
• Role of Groups C and G Streptococcus remains
unclear.
Pathogenesis – Host Factors
• ~3–6% of population genetically susceptible.
• Family clustering, twin concordance confirm
inherited traits.
• Associated with HLA class II alleles (e.g., HLA-
DR7, DR4).
• Polymorphisms in TNF-α, mannose-binding
lectin, Toll-like receptors implicated.
Pathogenesis – Immune Response
• Molecular mimicry theory: cross-reactivity of
antibodies.
• Streptococcal antigens mimic human tissue
components (e.g., M protein, N-
acetylglucosamine).
• Triggers inflammation in heart valves, leading
to carditis.
• VCAM-1 activation recruits immune cells,
damages endothelium.
Clinical Features
• Latent period ~3 weeks after GAS infection
(longer in chorea, indolent carditis).
• Common: polyarthritis (60–75%), carditis (50–
75%).
• Chorea: 2–30%, more common in females.
• Rare: erythema marginatum, subcutaneous
nodules (<5%).
Heart Involvement
• Mitral valve always involved; may lead to
regurgitation → stenosis over time.
• Aortic valve may also be affected (rarely
alone).
• Carditis may cause PR prolongation, soft S1,
AV block.
• RHD progresses silently; echocardiography
essential for diagnosis.
Joint Involvement
• Polyarthritis—migratory, asymmetric, affects
large joints.
• Arthralgia may be seen, especially in high-risk
populations.
• Responds rapidly to salicylates and NSAIDs.
Chorea
• Occurs after long latency; mostly in females.
• Affects face and limbs, may cause emotional
lability.
• Often coexists with carditis; echo
recommended.
Skin Manifestations
• Erythema marginatum: serpiginous, transient
rash.
• Subcutaneous nodules: over bony
prominences, appear late.
Diagnosis and Jones Criteria
• Diagnosis = clinical features + evidence of GAS
infection.
• Jones Criteria (updated): Differentiates high
vs. low risk populations.
• Requires combination of major and minor
criteria.
• Echocardiography now included for detecting
subclinical carditis.
Treatment
• Antibiotics: penicillin (oral or IM), amoxicillin
alternative.
• NSAIDs/salicylates: for arthritis and fever, not
carditis.
• Steroids: reserved for severe carditis or
chorea.
• Bed rest as needed; chorea managed with
anticonvulsants/steroids.
Prevention
• Primary prevention: Treat sore throat
promptly (within 9 days).
• Secondary prevention: Benzathine penicillin
every 3–4 weeks.
• Duration depends on carditis, age, recurrence
history.
• Lifelong prophylaxis may be needed in severe
RHD.
Prognosis and Follow-Up
• ARF lasts ~12 weeks untreated, 1–2 weeks
treated.
• Monitor markers (CRP, ESR) and echo at 1
month.
• Registry enrollment and family education
essential.
• RHD may progress silently; long-term
surveillance required.