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Pathology

Systemic inflammatory disease occurring 2-4 weeks after pharyngeal infection 
with group A beta haemolytic Streptococcus pyogenes

Aetiology

Autoimmune antibody-antigen complex which were produced against the Streptococcus 
pyogenes cell wall and proteins on cardiac valves. It affects heart, joints, skin and brain.  Peak age 5-15 years


Signs

Symptoms of valve dysfunction, subcutaneous nodules, erythema marginatum, 

Symptoms

Polyarthritis, heart failure, fever, joint pain, involuntary movements, sore throat


Investigations

Bloods: FBC, CRP, Anti-streptolysin O titre, Dnase B titre
Throat swab: screens for pharyngitis
ECG: Prolonged PR
Echo: Assesses valvular and LV function
Histology: Aschoff Bodies: Granulomatous nodules with central necrosis and 
fibrinoid degeneration with a cuff of macrophages
Anitschkow Cells: Altered macrophages within Aschoff Bodies with 
ribbon like chromatin pattern with the nuclei
Diagnosis: Based on the Jones Criteria. Positive throat swab for Group A Beta 
Haemolytic Streptococcus or Elevated anti-streptolysin O titres + 2 
major criteria or 1 major and 2 minor criteria

Treatment

Acute: Bed rest, analgesia and antibiotics (single dose of IM benzylpenicillin 
otherwise oral penicillin or macrolide for 10 days.
Symptom Control: NSAIDs or Aspirin for pancarditis or arthritis, however 
NSAIDs can exacerbate heart failure. 
Chorea can be treated with haloperidol or diazepam

Prognosis

60% with carditis develop chronic rheumatic disease or valve dysfunction. 
Initial mortality is 1%.
If untreated progresses to rheumatic heart disease leading to valvular disease

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