300.1 🎓 醫孞生版

300.1.0.1 📌 䞀頁重點

300.1.0.1.1 Acute Rheumatic Fever (ARF)
300.1.0.1.1.1 Epidemiology
  • Children 5-15 years most affected
  • Develops 2-4 weeks after GAS pharyngitis
  • High-burden countries: sub-Saharan Africa, South Asia, Pacific Islands
  • Taiwan: significant decline; rare now
  • Global: ~ 470,000 new cases/year, 33 million RHD prevalent
300.1.0.1.1.2 Etiology + Pathogenesis
  • Group A β-hemolytic Strep (S. pyogenes) pharyngitis (NOT skin infection — unlike post-strep glomerulonephritis)
  • Type II hypersensitivity (molecular mimicry)
  • M protein cross-reacts with cardiac myosin, neural tissue, synovium
  • Genetic susceptibility (HLA, family history)
  • 0.3-3% of untreated GAS pharyngitis → ARF
300.1.0.1.1.3 Major Manifestations (Mnemonic: J♥NES or CASES)
  • Carditis (CARDIAC)
  • Arthritis (large joint, migratory polyarthritis)
  • Sydenham chorea
  • Erythema marginatum
  • Subcutaneous nodules
300.1.0.1.1.4 Carditis
  • ~ 50% of ARF cases
  • Pancarditis: endocarditis + myocarditis + pericarditis
  • Valvulitis (mitral most affected — then aortic)
  • New murmur, friction rub, HF symptoms
  • Persistent tachycardia
  • May be asymptomatic on echo (subclinical)
  • Reversible in some; permanent damage in others
300.1.0.1.1.5 Arthritis (Most Common)
  • Large joint, migratory, polyarthritis
  • Knees, ankles, wrists, elbows
  • Asymmetric
  • Resolves without permanent damage
  • ↓ With NSAIDs / aspirin
300.1.0.1.1.6 Sydenham Chorea
  • Late manifestation (1-6 months post-strep)
  • Especially girls 8-15 yo
  • Involuntary movements + emotional lability
  • ANE-bodies: anti-basal ganglia antibodies
  • Usually self-limited
300.1.0.1.1.7 Erythema Marginatum
  • Pink rash with serpiginous outline
  • Trunk + proximal extremities
  • Evanescent
  • Trunk-sparing in some
300.1.0.1.1.8 Subcutaneous Nodules
  • Painless, firm, small (0.5-2 cm)
  • Extensor surfaces (elbows, knees, scalp)
  • Associated with severe carditis
300.1.0.1.1.9 Minor Manifestations
  • Fever
  • Arthralgia (vs arthritis)
  • ↑ ESR / CRP
  • Prolonged PR interval on ECG
  • Previous rheumatic fever or RHD
300.1.0.1.1.10 Jones Criteria (2015 AHA Modified)
  • Definite Initial ARF: 2 major OR 1 major + 2 minor + GAS infection evidence
  • Definite Recurrence: 2 major OR 1 major + 1 minor OR 3 minor + GAS evidence
  • Subclinical Carditis: now accepted for high-risk populations (echo without auscultation findings)
300.1.0.1.1.11 Evidence of GAS Infection
  • ASO titer ≥ 240 (or rising)
  • Anti-DNase B ≥ 240
  • Positive throat culture or rapid antigen test
  • Recent scarlet fever
300.1.0.1.2 Diagnosis
300.1.0.1.2.1 Laboratory
  • Throat culture / rapid antigen test for GAS
  • ASO + Anti-DNase B (rising titer over 2-3 wk)
  • CBC + ESR + CRP (acute phase reactants)
  • ECG (PR prolongation, QT, ST/T)
  • Echocardiogram (subclinical carditis)
300.1.0.1.2.2 Echocardiography Findings
  • Mitral regurgitation (often present)
  • Mitral leaflet thickening
  • Aortic regurgitation
  • Pericardial effusion
  • LV function
  • 2024 WHO + AHA: echo for all suspected ARF
300.1.0.1.3 Treatment of ARF
300.1.0.1.3.1 Primary Treatment of GAS Pharyngitis
  • Penicillin V 250-500 mg PO TID/QID × 10 days OR
  • Benzathine penicillin G (BPG) IM × 1 (single dose)
  • Allergic: macrolide (azithromycin) or clindamycin
  • Important to complete full course
300.1.0.1.3.2 Treatment of Acute ARF
  • Anti-inflammatory:
    • Aspirin (high-dose) OR NSAIDs (ibuprofen) for arthritis
    • Glucocorticoids for severe carditis with HF
  • HF management: standard (diuretic, β-blocker, ACEi)
  • Bed rest during active disease
  • Antibiotic eradication of GAS: penicillin even if culture negative
  • Sydenham chorea: valproate, haloperidol, carbamazepine
300.1.0.1.3.3 Hospitalization
  • Severe carditis with HF
  • Symptomatic chorea
  • Diagnostic uncertainty
300.1.0.1.4 Secondary Prevention (Lifelong)
300.1.0.1.4.1 Rationale
  • Prevent recurrent ARF (which causes worsening RHD)
  • Especially important in children + young adults
300.1.0.1.4.2 Antibiotic Regimens
  • Benzathine penicillin G 1.2 MU IM q3-4 weeks (preferred, especially in endemic areas q3 wk)
  • Penicillin V 250 mg PO BID
  • Erythromycin / azithromycin for penicillin-allergic
  • Sulfadiazine 1 g/d
300.1.0.1.4.3 Duration
Category Duration
ARF without carditis 5 years OR until age 21 (whichever longer)
ARF with carditis (mild) 10 years OR until age 21
ARF with carditis + RHD Lifelong (or 10 years OR age 40 — guideline variations)
Post-valve surgery Lifelong
300.1.0.1.5 Rheumatic Heart Disease (RHD)
300.1.0.1.5.1 Chronic Sequelae of Recurrent ARF
  • Mitral stenosis (MS) — most characteristic
  • Mitral regurgitation (MR)
  • Aortic regurgitation (AR)
  • Aortic stenosis (AS) — late
  • Often multi-valvular involvement
300.1.0.1.5.2 Mitral Stenosis (Ch285)
  • Rheumatic almost universal cause globally
  • Commissural fusion, leaflet thickening, chordal shortening
  • PMBV (Wilkins ≀ 8) or MVR
300.1.0.1.5.3 Mitral Regurgitation
  • Leaflet thickening, retraction, chordal involvement
  • Repair vs replacement
300.1.0.1.5.4 Aortic Regurgitation
  • Cuspal thickening + retraction
  • Often combined with AS
  • Surgical AVR
300.1.0.1.5.5 Aortic Stenosis
  • Calcific change of rheumatic AV
  • TAVR / SAVR considerations
300.1.0.1.5.6 Echocardiographic Diagnosis of RHD (2012 WHF criteria)
  • Definite RHD: typical morphologic changes + regurgitation
  • Borderline RHD: subtle changes
  • Normal: no abnormality
300.1.0.1.5.7 RHD Action Plan (2024 WHO + WHA)
  • Increase access to echo screening
  • Penicillin G access globally
  • Education and surveillance
  • Vaccines (under development)
300.1.0.1.5.8 Pregnancy + RHD
  • mWHO classification (Ch290)
  • MS particularly poorly tolerated
  • LMWH for AF or thromboembolism
300.1.0.1.6 Prevention Strategies
300.1.0.1.6.1 Primary Prevention
  • Treat GAS pharyngitis adequately
  • Improve hygiene + crowding
  • Universal access to penicillin
300.1.0.1.6.2 Secondary Prevention
  • Long-term penicillin prophylaxis
  • BPG IM q3-4 wk most effective
300.1.0.1.6.3 Vaccine Development (Future)
  • StreptAnova, MJ8VAX, S2 antigen
  • Multiple candidates in trial
  • Promising but no commercial vaccine yet
300.1.0.1.7 Pearls + Special Topics

300.1.0.2 🩺 床邊速查

  • ARF: 2-4 weeks post-GAS pharyngitis; Jones criteria (2 major or 1 major + 2 minor + GAS evidence)
  • Major: carditis, arthritis (migratory polyarthritis), Sydenham chorea, erythema marginatum, subcutaneous nodules
  • GAS pharyngitis treatment: penicillin V 10 d OR BPG IM single dose
  • Acute ARF treatment: ASA / NSAIDs (arthritis) + steroids (severe carditis) + bed rest + GAS eradication
  • Secondary prevention: BPG IM q3-4 wk; duration varies by carditis + RHD status
  • RHD: chronic valve disease (MS most common); PMBV or surgery
  • 2024 RHD Action Plan: echo screening + penicillin access + vaccine R&D