Acute rheumatic fever: Difference between revisions

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==Background==
*Primarily affects school age children 2-6wk after strep pharyngitis
*Connective tissue of heart, joints, CNS, subq tissues are targeted by immune reaction
==Diagnosis==
==Diagnosis==
Modified Jones Criteria (1992) for Acute Rheumatic Fever
Modified Jones Criteria (1992) for Acute Rheumatic Fever


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#Major diagnostic criteria
#Major diagnostic criteria
##Carditis
##Carditis
##Polyarthritis
###New or changing murmur, cardiomegaly, CHF, pericarditis
##Migratory polyarthritis
##Chorea
##Chorea
##Subcutaneous nodules
##Subcutaneous nodules
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##Fever
##Fever
##Arthralgia
##Arthralgia
##Prolonged PR interval on ECG
##History of previous attack of rheumatic fever
##Elevated acute-phase reactants (APRs), i.e ESR and CRP
##Prolonged PR interval
##Evidence of previous GAS pharyngitis:
##Elevated ESR, CRP
##Positive throat Cx or rapid strep test or Elevated or rising streptococcal antibody titer
#Evidence of preceding streptococcal infection
##Increased ASO or other strep ab
##Positive throat culture for Group A strep
##Positive rapid GAS
##Recent scarlet fever


==Symptoms==
==Symptoms==
#Polyarthritis: most common symptom and often earliest (70-75%). Begins in knees and ankles, migrates to other large joints.  Responds to aspirin
*Polyarthritis
#Carditis: Most serious complication and the second most common (50%). The murmurs of acute RF are from valve regurgitation, and the murmurs of chronic RF are from valve stenosis
**Most common symptom (75%)
#Sydenham Chorea: 10-30%. P/W difficulty writing, involuntary grimacing, purposeless movements of the arms and legs, speech impairment, generalized weakness, and emotional lability
**Migratory, fleeting polyarticular arthritis primarily affecting large joints
#Erythema marginatum: aka erythema annulare, occurs in 5-13%. Erythema marginatum begins as 1- to 3-cm diameter, pink-to-red nonpruritic macules or papules located on the trunk and proximal limbs but never on the face. Spreads outward to form a serpiginous ring with central clearing. Can be accentuated by warm towels/hot bath. Occurs early, remains past the resolution of other sx
*Carditis (33%)
*DDx includes sepsis, drug reactions, and glomerulonephritis.
**Most serious complication and second most common
Subcutaneous nodules: Frequency has declined to 0-8%. Extensor surfaces. Firm, nontender, mobile, seveal millimeters to 1-2 cm. 
***New murmur, pericardial rub, CHF
*Histo: Like Aschoff bodies observed in the heart. Subcutaneous nodules generallyoccur several weeks into the disease and resolve within a month
*Chorea (10%)
**May appear months following strep infection, may be sole manifestation of RF
*Erythema marginatum
**Persists only for several days
**Usually coexists with presence of carditis in some form
**Nonpruritic, located on trunk and proximal limbs, never on face  
*Nodules
**Located on extensor surfaces of wrists, elbows, knees


==Treatment==
==DDX==
#Eradicate GAS infection
#JIA
##PCN 0.6 to 1.3 million Units IM x 1 or
#septic arthritis
##Oral PCN for 10 days or
#Kawasaki disease
##Erythromycin if PCN-allergic
#viral or other forms of cardiomyopathy
#Future prophylaxis
#leukemia
#Treatment of symptoms, e.g. arthritis
#vasculitis (HSP, drug reaction)


==Source ==
==Work-Up==
#CBC
#ECG
#CXR
#ESR, CRP


7/2/09 PANI
==Treatment==
*Penicillin
**Indicated for all pts w/ rheumatic fever even if cx for strep is negative
**600K units IM if <27 kg
**1.2 million units IM if >27 kg
**Penicillin V PO x10d
**Prophylaxis
***5yr if no cardiac involvement, lifetime if cardiac involvement
***Pen G IM q month or oral penicillin daily
**Erythromycin x10d if pen allergic
*Arthritis
**High-dose aspirin therapy (75-100 mg/kg/d)
*Carditis
**Prednisone 1-2mg/kg/d
*Chorea
**Haloperidol 0.01-0.03 mg/kg/d in four divided doses


3/10 Rosen
==Disposition==
*Admit for confirmation of diagnosis


Dermatoglyphics in ARF: Axial triradius shows ulnar deviation and +/- proximal placement. (Sanyal, SK, Mukherjee, et al. Am J Dis Child, 1978: 132:692-695.
==Source ==
Tintinalli


[[Category:Peds]]
[[Category:ID]]
[[Category:ID]]

Revision as of 19:58, 27 June 2011

Background

  • Primarily affects school age children 2-6wk after strep pharyngitis
  • Connective tissue of heart, joints, CNS, subq tissues are targeted by immune reaction

Diagnosis

Modified Jones Criteria (1992) for Acute Rheumatic Fever

REQUIRE: 2 major or 1 major and 2 minor criteria and evidence of previous GAS pharyngitis.

  1. Major diagnostic criteria
    1. Carditis
      1. New or changing murmur, cardiomegaly, CHF, pericarditis
    2. Migratory polyarthritis
    3. Chorea
    4. Subcutaneous nodules
    5. Erythema marginatum
  2. Minor diagnostic criteria
    1. Fever
    2. Arthralgia
    3. History of previous attack of rheumatic fever
    4. Prolonged PR interval
    5. Elevated ESR, CRP
  3. Evidence of preceding streptococcal infection
    1. Increased ASO or other strep ab
    2. Positive throat culture for Group A strep
    3. Positive rapid GAS
    4. Recent scarlet fever

Symptoms

  • Polyarthritis
    • Most common symptom (75%)
    • Migratory, fleeting polyarticular arthritis primarily affecting large joints
  • Carditis (33%)
    • Most serious complication and second most common
      • New murmur, pericardial rub, CHF
  • Chorea (10%)
    • May appear months following strep infection, may be sole manifestation of RF
  • Erythema marginatum
    • Persists only for several days
    • Usually coexists with presence of carditis in some form
    • Nonpruritic, located on trunk and proximal limbs, never on face
  • Nodules
    • Located on extensor surfaces of wrists, elbows, knees

DDX

  1. JIA
  2. septic arthritis
  3. Kawasaki disease
  4. viral or other forms of cardiomyopathy
  5. leukemia
  6. vasculitis (HSP, drug reaction)

Work-Up

  1. CBC
  2. ECG
  3. CXR
  4. ESR, CRP

Treatment

  • Penicillin
    • Indicated for all pts w/ rheumatic fever even if cx for strep is negative
    • 600K units IM if <27 kg
    • 1.2 million units IM if >27 kg
    • Penicillin V PO x10d
    • Prophylaxis
      • 5yr if no cardiac involvement, lifetime if cardiac involvement
      • Pen G IM q month or oral penicillin daily
    • Erythromycin x10d if pen allergic
  • Arthritis
    • High-dose aspirin therapy (75-100 mg/kg/d)
  • Carditis
    • Prednisone 1-2mg/kg/d
  • Chorea
    • Haloperidol 0.01-0.03 mg/kg/d in four divided doses

Disposition

  • Admit for confirmation of diagnosis

Source

Tintinalli