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ARF and RHD: Global Epidemiology and Management

Acute Rheumatic Fever (ARF) and Rheumatic Heart Disease (RHD) are prevalent in developing countries, with an estimated 30–43 million cases and over 300,000 annual deaths, primarily affecting children and young adults. The diseases are linked to Group A Streptococcus infections and have genetic susceptibility factors, with clinical features including carditis and polyarthritis. Diagnosis relies on clinical features and Jones Criteria, while treatment involves antibiotics and anti-inflammatory medications, with prevention strategies focusing on prompt treatment of sore throats.

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0% found this document useful (0 votes)
4 views14 pages

ARF and RHD: Global Epidemiology and Management

Acute Rheumatic Fever (ARF) and Rheumatic Heart Disease (RHD) are prevalent in developing countries, with an estimated 30–43 million cases and over 300,000 annual deaths, primarily affecting children and young adults. The diseases are linked to Group A Streptococcus infections and have genetic susceptibility factors, with clinical features including carditis and polyarthritis. Diagnosis relies on clinical features and Jones Criteria, while treatment involves antibiotics and anti-inflammatory medications, with prevention strategies focusing on prompt treatment of sore throats.

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doctor vivek
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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.

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