Understanding Rheumatic Fever: Causes, Symptoms, and Complications

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Rheumatic Fever

Is a multisystem inflammatory disease that can involve the heart, joints, skin, and brain. The disease develops after a streptococcal infection (A beta-hemolytic – Streptococcus pyogenes) – mostly pharyngitis

Clinical Features

  • Occur most often in children
  • Develop 2-4 weeks after streptococcal infection
  • Fever, malaise, and increased erythrocyte sedimentation rate
  • Rheumatic heart disease – pancarditis; inflammation of the entire heart (3 layers)


Leads to serous effusions


Leads to cardiac failure


Leads to valvular damage

  • Rheumatic endocarditis – mitral and aortic valves (mostly); the valves become thickened, fibrotic, deformed, & calcification (may)
  • Joint involvement – arthralgia, arthritis, and migratory polyarthritis
  • Skin lesions – subcutaneous nodules and erythema marginatum (distinctive)
  • CNS involvement – Sydenham chorea (involuntary muscular movements)
  • If chronic – arrhythmias and thromboembolic complications


  • Immunologic origin rather than a result of direct bacterial involvement
  • Streptococcal antigens – molecular mimicry – elicit an antibody cross-reactivity


  • Type II hypersensitivity – attributed to antibodies against group A streptococcal molecules that cross-react with host myocardial antigens


Acute Rheumatic Fever

  • Inflammation foci within several tissues
  • Aschoff bodies – focal interstitial myocardial inflammation, with:
  • Aschoff cells – multinucleated giant cells
  • Anitschkow myocytes or “caterpillar cells”
  • Collagen and fibrinoid material
  • Aschoff bodies can be found in all three layers of the heart
  • The pericardium exhibits fibrinous exudate
  • The endocardium may contain verrucae (1-2mm vegetations)

Chronic Rheumatic Fever

  • Organization of acute diffuse inflammation and scarring
  • Aschoff bodies are replaced by fibrous scar – therefore not seen in chronic RF
  • Cardiac valves show leaflet thickening, commissural fusion and shortening, and thickening of the chordae tendinea
  • Calcification creates buttonhole stenoses
  • Neovascularization

Rheumatic Heart Disease

  • Valvular stenosis and regurgitation
  • 70% mitral valve
  • 25% mitral and aortic valves
  • 5% tricuspid valve
  • Mitral valve stenosis à left atrium dilation à atrial fibrillation à mural thrombosis

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