Rheumatic Fever

Question 1

Etiopathogenesis of Rheumatic Fever

Answer 1

• Multi-system disease resulting from autoimmune reaction to infection with group A beta-hemolytic Streptococcus
• In RF, antibodies against M-proteins of certain strains of Streptocossus cross-react with tissue glycoproteins in the heart, joints, and other tissues (“moleccular mimicry”)

Question 2

Manifestations of RF

Answer 2

• Latent period of 3 weeks (range from 1 to 5 weeks) between the precipitating infection and the appearance of the clinical features of acute RF with the exception of chorea and indolent carditis which may follow a prolonged latent period lasting up to 6 months

Question 3

Most common clinical presentation of RF

Answer 3

Polyarthritis and Fever

Question 4

Major Manifestations of RF

Answer 4

• Carditis (up to 60%)
• Migratory Polyarthritis (75%)
• Sydenham’s Chorea (<10%)
• Erythema Marginatum
• Subcutaneous Nodules

Question 5

[RF] Carditis

Answer 5

• Pancarditis involving the epricardium, myocardium and endocardium
  
  • Pericarditis: audible friction rub, pericardial effusion on echocardigraphy
  • Myocarditis: unexplained heart failure or cardiomegaly
  • Endocarditis/valvulitis: apical holosystolic murmur of mitral regurgitation or basal early diastolic murmur
  • If with previous RHD: a definite change in character of the murmur or appearance of a new significant murmur indicates: “carditis”

Question 6

Hallmark of Carditis in RF

Answer 6

• valvular damage (only valvulitis leads to permanent damage)
• Characteristic manifestation is:
  
  • MITRAL REGURGITATION

Question 7

Sisngle most important prognostic factor in RF

Answer 7

Carditis

Question 8

Migratory Polyarthritis

Answer 8

• Most frequent major maniffestation of RF
• Typically migratory over a period of hours
• Most often asymmetric and affecting large joints
  
  • ankles
  • wrists
  • knees
  • elbows
• Highly responsive to salicylates and NSAIDs

Question 9

Syndenham’s Chorea

Answer 9

• Involuntary head and upper limbs jerking movement mostly affecting
• Commonly occurs in females and in the absence of oter manifestations
• Usually resolves completely within 6 weeks

Question 10

Erythema Marginatum

Answer 10

• first appear as a bright pink macule or papule that spreads outward in a circular or serpiginous pattern
• Evanescent pink macular eruption with round borders and central clearing
• Usually concentrated on trunk, sometimes on the limbs, almost never on the face

Question 11

Subcutaneous nodules

Answer 11

• Round, firm, freely movable lesions varying in size from 0.5 -2cm
• Painless small lumps found over extensor surfaces of joints
• Usually a delayed manifestation (2-3 weeks after onset)
• Commonly associated with carditis

Question 12

Minor manifestations

Answer 12

• Clinical
  
  • Arthralgia (joint pains)
  • Fever ranging from 38.4 -40 degrees celsius
• Laboratory findings
  
  • Elevated acute phase reactants (ESR/CRP)
  • Prolonged PR interval on ECG

Question 13

Supporting evidence of a Preceding Streptococcal infection within the last 45 days

Answer 13

• Elevated or rising anti-steptolysin-O or other streptococcal antibody or
• A positive throat culture or
• Rapid antigen test for group A streptococcus or
• Recent Scarlet Fever

Question 14

Revised jones Cruteria for the Diagnosis of Rheumatic Fever (RF) and RHD

Answer 14

Question 15

Criteria for Primary episode of RF

Answer 15

• Evidence of preceding group-A streptococcal infection PLUS:
  
  • 2 major criteria or
  • 1 major + 2 minor criteria

Question 16

Acute Management for treatment of Streptococcal Tonsillopharyngitis

Answer 16

• Penicillin
  
  • Penicillin-V PO 500 mg BID x 10 days
  • Amoxicillin PO 50 mg/kg daily x 10 days
  • Benzathine Penicillin-G IM 1.2 million units as single dose
• If with Penicillin allergy
  
  • Clindamycin PO 300-600 mg TID x 10 days
  • Azithromycin PO 500 mg OD x 5 days
  • Clarithromycin 250 mg BID x 10 days

Question 17

Acute management for Arthritis/Mild carditis

Answer 17

• Aspirin 4-8 grams/day in 4-5 divided doses up to 2 weeks
• Dose can be decreased to 60-70 mg/kg/day for an additional 3-6 weeks

Question 18

Acute Management for Moderate-Severe Carditis

Answer 18

• may add prednisone 1-2 mg/kg/day up to maximum of 3 weeks (then dose decreased and tapered by 20-25% each week)

Question 19

Acute Management for severe chorea

Answer 19

• Carbamaepine or valproic acid

Question 20

General Measures in management of RF

Answer 20

• Hospital admission o confirm diagnosis and institute treatment
• Bed Rest and monitoring for onset of carditis

Question 21

Prophylaxis of Rheumatic Fever

Answer 21

• PRIMARY PROPHYLAXIS
  
  • to treat group A strep URTI and eradicate the organism to prevent an initial attack of acute RF
• SECONDARY PHROPHYLAXIS
  
  • prevent colonization and/or infection in patients who have had preious attack of RF (to prevent recurrence of RF)

Question 22

Drugs available for secondary prophylaxis

Answer 22

• Benzathine Penicillin-G 1.2 million units IM every 3-4 weeks (best)
• Penicillin-V 250 mg BID PO
• Erythromycin 250 mg/cap BID (if allergic to penicillin)

Question 23

Duration of Secondary Prophylaxis for RF without carditis

Answer 23

• 5 years after last attack or until 21 years olf (whichever is longer)

Question 24

Duration of Secondary Prophylaxis for RF with carditis, but no residual valvular disease (no clinical or echocardiographic evidence of valvular disease)

Answer 24

10 years after last attack or until 21 years old (whichever is longer)

Question 25

Duration of Secondary Prophylaxis for RF with carditis and persistent residual valvular heart disease

Answer 25

• 10 years after last attack or until 40 years old (whichever is longer, sometimes lifetime)