Acute Rheumatic Fever, ARF

Notes
  • It is an acute inflammatory complication of Group A streptococcal (GAS) pharyngeal infection.
  • It mainly affects children 3-15yrs old.
  • The global incidence of ARF is 5-15 per 100,000
  • The proportion of patients with GAS infections who develop ARF (attack rate) is less 1.0-3.0% but it is 50% in patients with previous episodes of ARF. This fact necessitates prophylactic measures in patients with the history of the episodes of ARF.
  • The GAS M proteins and proteins that are found in synovium, cardiac muscles and heart valves all share a common epitope.
  • The inflammatory action of GAS M protein antibodies affects the heart valve, endocardium, myocardium and pericardium in that order. In ARF, the major damage occurs due to acute interstitial valvulitis that subsequently leads to valvular edema. Where the disease progresses to chronic rheumatic heart disease (CRHD), the valves may thicken, fuse or retract. The same happens to chordae tendineae.
Symptoms
  • Mitral, aortic and tricuspid regurgitation or stenosis.
  • Carditis (endocarditis, myocarditis or pericarditis) that is characterized by fever, chills, heart murmur, aching joints, aching muscles, night sweats, shortness of breath, paleness and persistent cough.
  • Valvulitis characterized by murmurs (by auscultation, ECG or Doppler studies).
  • Chest pain or pericardial rub (due to pericarditis).
  • Heart failure and related symptoms
  • Migratory polyarthritis (the most common characteristic symptoms)
  • Sydenham’s chorea (that is characterized by rapid, irregular, and aimless involuntary movements of the arms, trunk, and facial muscles)
  • Subcutaneous nodules
  • Erythema marginatum (skin rash occurring on the trunk and its proximal extremities that
  • are characterized as rings but not on the face, soles of the feet and the palms)
Diagnosis
  1. Jones Criteria for Diagnosis of ARF that include:
  2. Required Criteria:

Evidence of previous Strep infection such as;

*Antistreptolysin O (ASO) titer (of 1:300)

*GAS throat culture

*Recent scarlet fever

*Anti-DNase B

 *Anti-hyaluronidase

  1. Major Diagnostic Criteria:

Carditis, polyarthritis, chorea, erythema marginatum and subcutaneous nodules.

  1. Minor Diagnostic Criteria:

Fever, arthralgia, previous RF or rheumatic heart disease, acute phase reactions (ESR / CRP / leukocytosis) and  prolonged PR interval

 

Using the above criteria a diagnostic decision is made based on the following:

Either;

1 Required Criteria + 2 Major Criteria + 0 Minor Criteria

Or;

1 Required Criteria + 1 Major Criteria + 2 Minor Criteria

  • Auscultation
  • ECG

Echocardiography with Doppler

Differential
  • Bacterial sepsis
  • Congenital heart disease
  • Gout and pseudogout
  • Infective endocarditis
  • Kawasaki Disease
  • Lyme disease
  • Myocarditis
  • Septic arthritis
  • Systemic Lupus Erythematosus
  • Viral arthropathy
Prevention
  • Appropriate diagnosis and adequate antibiotic treatment of GAS infections
Management
  • Eradication of GAS infection by:

 

Either;

*Adults (27 kg and above):

 Inj. Benzathine penicillin G 1.2 million units IM STAT.

*Children (below 27 kg):

Inj. Benzathine penicillin G 600,000 units IM STAT.

 

Or;

*Adults (27 kg and above):

 Tab Penicillin V 500mg BD x 10/7

 

 *Children (below 27 kg):

Tab Penicillin V 250mg BD x 10/7

 

 Or;

 

*Adults:

Caps Amoxicillin 250-500 mg TID x 10/7

 

*Children:

Syrup or caps or tabs Amoxicillin 50 mg/kg/day in 2 or 3 divided doses x 10/7

In case of penicillin allergy:

 

*Adults:

Either;

Tab Azithromycin 500 mg OD x 5/7

Or;

Tabs Clarithromycin 250 mg BD x10/7

Or

Cap Clindamycin 7 mg/kg/day in 3 divided doses x 10/7

Or;

Tabs Erythromycin 12.5mg/kg QID X 10/7

 

*Children:

Either;

Susp /Tabs Azithromycin 12 mg/kg OD x 5/7

Or;

Susp /Tabs Clarithromycin 250 mg BD x 10/7

Or;

Susp /Tabs Clindamycin 7 mg / kg / day PO in 3 divided doses x10/7

 

  • Relief of acute symptoms

 

*For arthritis and/or mild carditis;

 

**Aspirin is the NSAID of choice;

 

   Children and adolescents:

 

Tabs Aspirin 15-25 mg/kg QID (Max daily dose 4-6gm) x 2 - 4 wks and then over 4/52.

*Moderate to severe carditis

 

**Tabs Prednisolone 1 mg/kg BD (max 60 mg/day) x 2-4 wks, taper over 2-3 wks.

During the tapering process;

Tabs Aspirin 15-25 mg/kg QID (Max daily dose 4-6gm) and then continued for 2 - 4 wks after the end of the steroid therapy.

In case of unresponsive treatment with oral steroid after 2 days of treatment or in case of severe heart failure methylprednisolone succinate pulse therapy is administered;

 

*IV Methylprednisolone succinate 30 mg/kg IV OD (Max. 1 g/day) X 3/7

 

*Prophylaxis for 5 yrs or up to the age of 18yrs, whichever is longer. However, if there has been a recurrent infection give prophylaxis for life.

 

Either;

*Adults (27 kg and above):

 Inj. Benzathine penicillin G 1.2 million units IM at an interval of  3- 4 wks

*Children (below 27 kg):

Inj. Benzathine penicillin G 600,000 units IM at an interval of  3- 4 wks.

 

Or;

*Adults (27 kg and above):

 Tab Penicillin V 250mg BD

 

 *Children (27 kg and below):

Tab Penicillin V 500mg OD

 

In case of penicillin allergy:

 

*Adults:

Either;

Tab Azithromycin 250 mg OD

Or;

Tabs Erythromycin 250 mg BD

 

  • Management of resultant heart disease.

 

  • Sydenham’s chorea;

Tabs Haloperidol 25mcg/kg TID

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