Rheumatic Fever

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This is a result of immunological reaction to group A β-haemolytic streptococcus of the throat but occasionally in the skin of sensitized individual.  The disease occurs mainly in children of school age with a peak age of 5 to 25 years. The onset of symptoms is usually 1−3 weeks after the throat infection. Acute Rheumatic fever may lead to damage to the heart valves resulting Rheumatic heart disease.

Cause

  • Hypersensitivity reaction to group A β-haemolytic streptococcus. The commonest streptococcal strains in Africa are C and G

Signs and symptoms

  • Duckett-Jones' diagnostic criteria

 Major

  • Cardiac involvement (e.g. pericarditis, congestive heart failure, valve disease)
  • Sydenham's chorea
  • Erythema marginatum
  • Subcutaneous nodules
  • Migratory polyarthritis

Minor

  • Fever
  • Leucocytosis
  • Arthralgia
  • Elevated erythrocyte sedimentation rate (ESR)
  • Raised ASO titre (> 200 IU)
  • Prolonged PR interval
  • Evidence of preceding group A β- haemolytic streptococcal infection: positive throat culture or rapid streptococcal antigen

 Diagnosis

  • 2 major criteria

Or:

  • 1 major plus 2 (or more) minor criteria

Differential diagnosis

  • Malaria
  • Viral infection
  • Pyrexia of undetermined origin (PUO)
  • Connective tissue disease
  • Typhoid fever
  • Sickle cell disease
  • Myocarditis
  • Tuberculosis

Complications

  • Rheumatic heart disease
  • Arrhythmias
  • Cardiac failure

Investigations

  • Full blood count (shows raised white cell count)
  • ESR −> 30
  • C-reactive protein > 3mg/dl (Minor Criteria)
  • Antistreptolysin O (ASO) titre
  • Sickling status
  • Chest radiograph (heart may be enlarged)
  • Throat swab for microscopy, culture and sensitivity
  • Electrocardiography (Prolonged PR -interval – Minor Criteria)
  • Echocardiography for carditis (Major criteria)

Treatment objectives

  • Relieve symptoms
  • Treat the bacterial throat infection
  • Reduce or abolish inflammatory process
  • Treat cardiac failure if present
  • Prevent future Group A streptococcal infection

Non−pharmacological treatment 

  • Bed rest and supportive care

Pharmacological treatment 

Phenoxymethylpenicillin (Penicillin V) oral

Adult:

500 mg orally every 6 hours, increased up to 1g every 6 hours in severe infections

Child:

1 month - 1 year: 62.5 mg orally every 6 hours

Increased in severe infection to ensure at least 12.5mg/kg/dose

1 - 6 years: 125 mg every 6 hours

Increased in severe infection to ensure at least 12.5 mg/kg/dose

6 - 12 years: 250 mg every 6 hours

Increased in severe infection to ensure at least 12.5 mg/kg/dose

12 - 18 years: 500 mg every 6 hours, increased in severe infection up to 1 g/dose

OR

Erythromycin oral

Adult and child over 8 years:

250 - 500 mg orally every 6 hours

OR

500 mg - 1 g every 12 hours; up to 4 g daily in severe infections

Child:

Up to 2 years:125 orally mg every 6 hours

2 - 8 years 250 mg every 6 hours

Note: Double doses in severe infections

Salicylates - Acetylsalicylic acid oral

Adult:

300 mg - 1 g orally every 4 hours after food

Maximum dose in acute conditions 8 g daily

Child: not recommended for use

 

Prednisolone oral 

Prednisolone should be given in severe rheumatic fever with carditis

Initially, up to 10 - 20 mg orally daily; up to 60mg daily in severe disease.

High dose prednisolone 1mg per Kg for 2 weeks then taper dose over four weeks 

Note: Do not discontinue steroid therapy suddenly if patient has taken it for more than 5 days. Dose tapering is mandatory to prevent acute adrenal insufficiency which is life-threatening.

Rheumatic fever prophylaxis:

  • Early diagnosis and treatment of Group A streptococcus throat infections
  • Ensure continuous prophylaxis in patients who have rheumatic fever or rheumatic heart disease, as follows:

Type of rheumatic fever (RF)

Duration of prophylaxis after last attack

Secondary prophylaxis

RF with carditis and persistent valvular disease

10 years or until age 40 years, whichever is longer. Life-long prophylaxis sometimes required

Benzathine penicillin:

Weight 27 kg or less: 600,000 units IM every 4 weeks

Weight more than 27 kg: 1,200,000 units IM every 4 weeks

Macrolide (e.g. erythromycin) antibiotics for persons with allergy to penicillin

RF with carditis but no valvular disease

10 years or until age 21 years, whichever is longer

RF without carditis

5 years or until age 21 years, whichever is longer

Referral

  • Patients who have been treated for heart failure should be referred for further evaluation.