rheumatic%20fever%20-%20acute
RHEUMATIC FEVER - ACUTE
Acute rheumatic fever is an autoimmune response to a previous group A beta-hemolytic streptococcal (GAS) infection causing acute generalized anti-inflammatory response primarily affecting the heart.
It often presents in patients 5-14 years of age and uncommon before 3 years and after 21 years of age.
Patients presenting with acute rheumatic fever are severely unwell, in extreme pain and requires hospitalization.

Principles of Therapy

  • Give oral Phenoxymethylpenicillin upon suspicion of acute rheumatic fever (ARF) & during the course of diagnosis. Once diagnosis is established, administer Benzathine penicillin G & stop Phenoxymethylpenicillin

 Secondary Prevention
Recurrent ARF is defined as 2 major or 1 major w/ 2 minor or 3 minor manifestations plus evidence of previous GAS infection

  • Continuous antimicrobial prophylaxis is recommended in patients w/ history of rheumatic fever (RF)
  • Prophylaxis of 12 months duration w/ reevaluation thereafter is recommended for patients w/ possible RF
    • Reevaluation should include repeat history, physical exam & echocardiography

Pharmacotherapy

Anti-infectives

Amoxicillin

  • Used to eradicate or prevent Group A streptococcus (GAS) infection
  • Treatment option for patients w/ penicillin allergy

Benzathine Penicillin G

  • Given to eradicate streptococci & is the 1st dose of penicillin for secondary prophylaxis
  • Drug of choice for acute rheumatic fever (ARF) patients w/ confirmed GAS not allergic to Penicillin

Cephalosporins

  • Eg Cephalexin, Cefadroxil
  • Used for the primary prevention & treatment of GAS infection in patients allergic to Penicillin

Macrolides, Oral

  • Eg Erythromycin, Clindamycin, Clarithromycin, Azithromycin, Roxithromycin
  • Used if patient has documented penicillin allergy
  • Recommended once there is suspicion of ARF & continue for 10 days or until ARF is diagnosed & Benzathine penicillin G is administered

Phenoxymethylpenicillin (Penicillin V), Oral

  • Used to eradicate GAS infection, if present
  • Start administration once there is suspicion of ARF & continue for 10 days or until ARF is diagnosed & Benzathine penicillin G is administered

Symptomatic Pharmacotherapy

Rheumatologists, cardiologists, &/or neurologists should be consulted based on the patient’s symptoms. Use of NSAID, salicylates &/or steroids are not recommended until ARF is confirmed as it will prevent development of joint manifestations & suppress acute phase reactants

Arthritis

  • Salicylates & nonsteroidal anti-inflammatory drugs (NSAID)
    • Eg Aspirin, Codeine, Ibuprofen, Naproxen, Paracetamol
    • Used for arthritis or severe arthralgia
    • Paracetamol is recommended as first-line therapy for ARF arthritis pain
    • Naproxen is a treatment option for Aspirin, w/c is no longer advised in several countries due to its adverse effects (eg Reye’s syndrome)
    • Recommended once ARF is confirmed 
  • Analgesics
    • Paracetamol & codeine are used for arthritis or severe arthralgia if ARF is not yet confirmed & patient is still undergoing diagnosis
    • Patients w/ mild arthralgia respond well to Paracetamol

Carditis/Heart Failure

  • Diuretics (eg Furosemide, Spironolactone) are used for mild-moderate heart failure
  • Angiotensin-converting-enzyme (ACE) inhibitors (eg Captopril, Enalapril, Lisinopril) may be used for more severe  heart failure [dysfunctional left ventricular (LV) function]
  • Corticosteroids (eg Prednisone, Prednisolone) are indicated for severe carditis, heart failure, or pericarditis w/ effusion
  • Digoxin is administered if atrial fibrillation or heart failure is present
  • See Heart Failure Disease Management Chart in MIMS Cardiology

Sydenham’s chorea

  • Start pharmacotherapy when symptoms interfere w/ normal activities
    • Sedatives or anticonvulsants (eg Carbamazepine, Valproic Acid, Haloperidol, Chlorpromazine & Diazepam), may be used to relieve symptoms

Fever

  • Paracetamol is indicated for fever
Secondary Prevention
Benzathine Benzylpenicillin G
  • Recommended 1st-line antibiotic regimen for the prevention of recurrent RF
  • An intramuscular dose every 4 weeks is recommended

Anti-infectives, Oral

Phenoxymethylpenicillin

  • The only oral anti-infective recommended for the prevention of recurrent RF
  • 2nd-line antibiotic regimen for the prevention of ARF next to Penicillin G

Sulfadiazine or Sulfafurazole

  • Recommended only in patients allergic to penicillins

Macrolides

  • Erythromycin, Clarithromycin, or Azithromycin may be used in patients allergic to penicillins, sulfadiazine, & sulfafurazole

Duration of Prophylaxis

  • RF patients w/ persistent valvular disease should receive prophylaxis for 10 years or until 40 years old, whichever is longer
    • Lifelong prophylaxis may be recommended in high-risk patients
  • RF patients w/ carditis but no residual heart disease may receive prophylaxis for 10 years or until 21 years old, whichever is longer
  • RF patients w/o carditis may continue prophylaxis up to 5 years or until 21 years old, whichever is longer
  • Discontinuation of prophylaxis should be thoroughly discussed w/ the patient & his/her family w/ emphasis on risks vs benefits

Non-Pharmacological Therapy

Initial episode of acute rheumatic fever (ARF) requires hospitalization for thorough clinical assessment, observation & guided management

General care

  • Examine daily for pattern of arthritis, presence of heart murmur, choreiform movements, skin rash & subcutaneous nodules
  • Seek medical attention upon the 1st sign of pharyngitis
  • Document cardiac signs & symptoms w/ carditis
  • Educate & involve family members
Diet
  • Advise nutritious diet w/o restrictions except in patients w/ congestive heart failure (CHF)
    • Patients w/ CHF should follow a fluid-limited & sodium-restricted diet
  • Potassium supplementation may be necessary due to mineralocorticoid effect of corticosteroid & diuretics, if used

Bed rest & indoor ambulation

  • Recommended duration of bed rest
    • 1-2 weeks w/ arthritis alone
    • 2-3 weeks w/ mild carditis, 4-6 weeks w/ moderate carditis, 2-4 months w/ severe carditis
  • Recommended duration of indoor ambulation
    • 1-2 weeks after hospital discharge w/ arthritis alone
    • 2-3 weeks after hospital discharge w/ mild carditis, 4-6 weeks after hospital discharge w/ moderate carditis, 2-4 months after hospital discharge w/ severe carditis
  • Do not allow full activity until the C-reactive protein (CRP) & erythrocyte sedimentation rate (ESR) have returned to normal
  • Patients w/ Sydenham’s chorea may require a wheelchair & should be on homebound instruction until abnormal movements resolve
  • No physical restrictions are needed after the initial episode subsides in the absence of carditis
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