Acute Rheumatic Fever

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Description 

An acute autoimmune process following pharyngitis caused by streptococcal infection. Diagnostic criteria for rheumatic fever are based on the modified Jones criteria. Has defined high risk population recognizing variability in clinical presentation and had included Echocardiography as a tool to diagnose for subclinical carditis.

Modified 2015 Jones’ criteria

Major

Minor

Carditis (clinical or subclinical)

Monoarthralgia

Arthritis – monoarthritis or polyarthritis

Fever (≥ 38.0ºC)

Polyarthralgia

ESR ≥ 30 mm/hour and/or CRP ≥ 3.0 mg/dl

Chorea

Prolonged PR interval (after considering the differences related to age: if there is no carditis as a major criterion)

Erythema marginatum

 

Subcutaneous nodules

 

ESR – erythrocyte sedimentation rate; CRP – C-reactive protein. All patients with ARF should have an Echocardiography done even in the absence of clinical suspicion of valvular done.

Two major manifestations plus evidence of preceding streptococcal infection OR one major and two minor manifestations plus evidence of preceding streptococcal infection are required to make a diagnosis.

Investigations

  • FBC/DC
  • ESR/CRP
  • Throat swab culture for Streptoccal
  • Anti Streptolysin O titres (ASOT)
  • ECG
  • ECHO
  • CXR

Treatment

  • Benzathine penicillin IM injection 600 000 IU - 1.2 MU stat OR
  • Oral phenoxymethyl penicillin 10-12.5mg/kg/dose twice daily for ten days maximum 500mg every 6 hours.
  • Patients with hypersensitivity to penicillin can be treated with oral first generation cephalosporins
    for 10 days.
  • Treat chorea (if severe)
  • Anti- heart failure medication 
  • Relieve symptoms, Bed rest, Relief of arthritis, pain and fever with Anti-inflammatory Agents

 

Arthritis

Carditis

Prednisolone 2 mg/kg/day

Nil

2-4 weeks

Aspirin 50 to 60 mg/kg/day in four to six divided doses

1 – 2 weeks

Until symptoms subside

Ibuprofen 30 mg/kg/day in 3 divided doses, where aspirin not tolerated

Until symptoms subside

Until symptoms subside

NB: The dose of prednisolone should be 2 mg/kg/day (max 60 mg); then taper by 20–25% per week. Aspirin can be reduced to 25 to 30 mg/kg/day when symptoms improve. The dose of prednisolone should be tapered, and aspirin started during the final week.

Management of Sydenham’s chorea

  • Reduce physical and emotional stress and use protective measures as indicated.
  • Benzathine penicillin IM stat (Eradicate GAS), then every 28 days for secondary prophylaxis.

Anti-inflammatory agents not indicated.

For severe chorea, any of the following drugs may be used:

  • Carbamazepine 7–20 mg/kg/day (7–10 mg/kg day usually sufficient) given TDS PO until chorea is controlled for at least 2 weeks, then trial off medication
  • Valproic acid Usually 15–20 mg/kg/day (can increase to 30 mg/kg/day) given TDS PO until chorea is controlled for at least 2 weeks, then trial off medication.
  • Phenobarbitone, Haloperidol and Chlorpromazine can be used when above not available.

Further management plan

  • ARF register (cardiac clinic), issue Acute Rheumatic Fever (ARF) prophylaxis card
  • Education of patient and family
  • Dental examination
  • Long term secondary prophylaxis plan

Secondary prophylaxis to prevent recurrent ARF is a long term, regular administration of antibiotics to:

  • Prevent group A β-Haemolytic Streptococcal (GAS) pharyngitis.
  • Prevent repeated development of ARF.
  • Prevent development of Rheumatic heart disease (RHD)
  • Reduce severity of RHD
  • Help reduce the risk of death from severe RHD

Antibiotic regimens for secondary prophylaxis

Antibiotic

Dose

Route

Frequency

First Line

Benzathine Penicillin G

1,200,000 U (900mg) ≥ 30 Kg

600,000 U (450 mg) ≤30 Kg

Deep IM injection

4-weekly

Second Line (if IM route is not possible or refused, adherence should be carefully monitored)

Phenoxymethylpenicillin (Pen V)

250mg

Oral

Once daily

Following documented penicillin allergy

Erythromycin

250mg

Oral

Twice daily

NOTE: Duration of prophylaxis in all persons with ARF is for a minimum of 10 years after the most recent episode of ARF or until age 21 years (whichever is longer). For RHD, the duration of prophylaxis is for life.