Rheumatic Fever: Pathology and Causes

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5 Questions

What autoimmune collagen disease mainly affects the heart and joints of children and young adults?

Rheumatic fever

What are the predisposing factors for Rheumatic fever? (Select all that apply)

Age: 5-15y

Rheumatic fever only affects the heart during the chronic phase.

False

Rheumatic fever is caused by antibodies that destroy the collagen in the tissues through Type _ hypersensitivity reaction.

IV

Match the following terms with their definitions:

Rheumatic Endocarditis = Affects the inner walls of the left atrium and the valves, leading to valve stenosis or incompetence. Myocardial infarction = Massive area of coagulative necrosis of myocardium due to sudden complete stoppage of coronary arterial blood supply. Angina Pectoris = Clinical syndrome characterized by temporary acute attacks of sudden severe retro-sternal pain. Chorea minor = Reversible involuntary movements in young females due to inflammation of the basal ganglia.

Study Notes

Rheumatic Fever

  • Definition: Autoimmune collagen disease affecting the heart and joints of children and young adults
  • Predisposing factors:
    • Age: 5-15 years
    • Low socio-economic standards and overcrowding, leading to malnutrition and recurrent streptococcal infections (e.g., tonsillitis)
    • Personal susceptibility (genetic factor)
  • Aetiology and pathogenesis:
    • Acute upper respiratory tract infection (e.g., tonsillitis) caused by streptococci
    • Latent period of 2-4 weeks, during which antibodies against streptococcal antigens are formed
    • Antibodies destroy collagen in tissues through:
      • Type II hypersensitivity reaction via cross-reaction
      • Type III hypersensitivity reaction via Ag-Ab complexes
      • Type IV hypersensitivity reaction via Aschoff body formation
  • Pathology:
    • Aschoff bodies (nodules) in the heart, skin, and other tissues
    • Aschoff bodies are composed of:
      • Central zone of fibrinoid necrosis (fragmented collagen)
      • Surrounding lymphocytes and Aschoff's giant cells
    • Fate of Aschoff bodies: Fibrosis

Affected Organs

  • Heart:
    • Rheumatic pan-carditis (affecting all layers of the heart)
    • Aschoff nodules leading to fibrosis
    • Endocarditis (affecting the inner walls of the left atrium, valves, and leading to valve stenosis and incompetence)
    • Myocarditis
    • Pericarditis (serofibrinous inflammation)
  • Joints and tendons:
    • Fleeting (migratory) arthritis of large joints
  • Skin:
    • Subcutaneous nodules over bony prominences
  • Brain:
    • Reversible involuntary movements (Chorea minor) due to inflammation of the basal ganglia
  • Pleura and peritoneum:
    • Serofibrinous inflammation
  • Large arteries:
    • Affected by Aschoff's nodules

Complications of Rheumatic Fever

  • Sub-acute infective endocarditis
  • Heart failure due to repeated attacks of myocarditis and valve stenosis or incompetence
  • Adhesion of pericardium, leading to impaired cardiac action

Endocarditis

  • Classification:
    • Infective endocarditis (bacterial)
      • Acute infective endocarditis
      • Sub-acute infective endocarditis
    • Non-infective endocarditis (autoimmune allergic)
      • Rheumatic endocarditis
      • Verrucous endocarditis (in systemic lupus erythematosus)
      • Marantic endocarditis (in patients with wasting disease or malignancy)

Ischemic Heart Diseases (Coronary Heart Diseases)

  • Definition: Disease of the myocardium due to insufficient coronary blood flow
  • Acute Ischemia:
    • Sudden complete occlusion of coronary artery
    • Caused by atheroma, thrombosis, rupture of the plaque, and hemorrhage into the plaque
    • Leads to myocardial infarction
  • Chronic Ischemia:
    • Gradual incomplete occlusion of coronary artery
    • Caused by atheroma, anemia, increased cardiac load, and ventricular hypertrophy
    • Leads to angina pectoris mainly

Angina Pectoris

  • Definition: Clinical syndrome characterized by temporary acute attacks of severe retro-sternal pain
  • Causes:
    • Temporary acute ischemia on top of chronic ischemia
    • Decreased coronary arterial blood flow
  • Clinical Presentation:
    • Precipitated by physical exercise, heavy meals, anxiety, or cold
    • Pain is stabbing in nature, referred to the left shoulder and left arm
    • Relieved by rest
  • Prognosis (complications):
    • May be fatal due to ventricular fibrillation, myocardial infarction, or heart failure

Myocardial Infarction

  • Definition: Massive area of coagulative necrosis of myocardium due to sudden complete stoppage of coronary arterial blood supply
  • Aetiology:
    • Decreased coronary arterial blood flow due to acute ischemia
  • Effects of occlusion:
    • Depends on the size of the occluded artery and collateral circulation
    • May lead to sudden death, infarction, or survival with good collaterals
  • Sites of infarction:
    • Correspond to the distribution of the occluded artery
    • Commonly involves the left ventricle
  • Morphology of the infarct area:
    • Varies with duration of infarction
    • Initially, no morphological changes
    • Then, coagulative necrosis, softening of the myocardium, and infiltration by neutrophils
    • Eventually, fibrous scar develops with thinning of the ventricular wall

Learn about rheumatic fever, an autoimmune disease affecting the heart and joints, its predisposing factors, and its aetiology and pathogenesis.

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