Acute rheumatic fever: Difference between revisions
 
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==Diagnosis==
==Background==
*Primarily affects school age children 2-6 weeks after Group A [[streptococcal pharyngitis]]
**Is not associated with [[impetigo]]
*Connective tissue of heart, joints, CNS, subcutaneous tissues are targeted by immune reaction


Modified Jones Criteria (1992) for Acute Rheumatic Fever
==Clinical Features==
*[[Arthritis|Polyarthritis]]
**Most common symptom (~75%)
**Aseptic arthritis with effusions
**Lasts up to 2 weeks
**Migratory, fleeting polyarticular arthritis primarily affecting large joints
*[[Myocarditis|Carditis]] (33%)
**Most serious complication and second most common
**[[Tachycardia]] out of proportion to the degree of fever is common; its absence makes the diagnosis of myocarditis unlikely<ref>Park M: Parks's Pediatric Cardiology for Practitioners, ed 6. Philadelphia, Saunders-Elsevier., 2014, (Ch) 20: p 368.</ref>
**New [[murmur]], pericardial rub, [[CHF]]
*[[Sydenham's chorea]] (10%)
**May appear months following strep infection, may be sole manifestation of RF
**Movements extinguish during sleep
*Erythema marginatum (< 10%)
**Persists only for several days
**Usually coexists with presence of carditis in some form
**Nonpruritic [[rash]], located on trunk and proximal limbs, never on face
*Nodules(< 10%)
**Located on extensor surfaces of wrists, elbows, knees


REQUIRE: 2 major or 1 major and 2 minor criteria and evidence of previous GAS pharyngitis.
==Differential Diagnosis==
*[[Scarlet fever]]
*[[Kawasaki Disease]]
*Viral or other forms of [[cardiomyopathy]]
*[[Leukemia]]
*[[Vasculitis]] ([[HSP]], drug reaction)


#Major diagnostic criteria
{{Pediatric hip DDX}}
##Carditis
##Polyarthritis
##Chorea
##Subcutaneous nodules
##Erythema marginatum
#Minor diagnostic criteria
##Fever
##Arthralgia
##Prolonged PR interval on ECG
##Elevated acute-phase reactants (APRs), i.e ESR and CRP
##Evidence of previous GAS pharyngitis:
##Positive throat Cx or rapid strep test or Elevated or rising streptococcal antibody titer


==II. Symptoms==
{{Differential Diagnosis Polyarthritis}}
#Polyarthritis: most common symptom and often earliest (70-75%). Begins in knees and ankles, migrates to other large joints.  Responds to aspirin
#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
#Sydenham Chorea: 10-30%. P/W difficulty writing, involuntary grimacing, purposeless movements of the arms and legs, speech impairment, generalized weakness, and emotional lability
#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
*DDx includes sepsis, drug reactions, and glomerulonephritis.
Subcutaneous nodules: Frequency has declined to 0-8%. Extensor surfaces. Firm, nontender, mobile, seveal millimeters to 1-2 cm. 
*Histo: Like Aschoff bodies observed in the heart. Subcutaneous nodules generallyoccur several weeks into the disease and resolve within a month


==Treatment==
==Evaluation==
#Eradicate GAS infection
===Work-Up===
##PCN 0.6 to 1.3 million Units IM x 1 or
*CBC
##Oral PCN for 10 days or
*[[ECG]]
##Erythromycin if PCN-allergic
*[[CXR]]
#Future prophylaxis
*ESR, CRP
#Treatment of symptoms, e.g. arthritis
*ASO (Antistreptolysin O) titer Eleveted 1week to 1 month after GAS infection
*Anti-DNase B titer is elevated longer than ASO and may be useful for patient presenting later than 2-4 months or with a negative ASO
*ECG - Looking for prolonged PR or other AV block
*CXR
*Echocardiogram


==Source ==
===Modified Jones Criteria (1992) for Acute Rheumatic Fever===
''REQUIRE: 2 major or 1 major and 2 minor criteria and evidence of previous GAS pharyngitis''
*Major diagnostic criteria (CASES)
**Carditis (new or changing [[murmur]], cardiomegaly, [[CHF]], [[pericarditis]])
**[[Arthritis]], migratory polyarthritis (typically affects knees, ankles, elbows, wrists)
**Sydenham's Chorea (abrupt, involuntary, purposeless movements)
**Erythema marginatum (non-pruritic, trunk/extremities, with facial sparing)
**Subcutaneous nodules (painless, firm, usually over bones/tendons)
*Minor diagnostic criteria
**[[Fever]]
**[[Arthralgia]]
**History of previous attack of rheumatic fever
**Prolonged PR interval
**Elevated ESR, CRP
*Evidence of preceding [[streptococcal]] infection
**Increased ASO or other strep ab
**Positive throat culture for [[Group A strep]]
**Positive rapid GAS
**Recent [[scarlet fever]]


7/2/09 PANI
==Management==
*[[Penicillin]] - Indicated for all with rheumatic fever even if culture for [[strep]] is negative
**[[Penicillin G]] 600K units IM if <27 kg, 1.2 million units IM if >27 kg
**[[Penicillin V]] PO x10d
*For those who are penicillin allergic
**Narrow spectrim cephalosporins such as [[cephalexin]] 20mg/kg/dose BID x10d
**[[Clindamycin]] 7mg/kg/dose TID (max of 300mg/dose) PO x10d
**[[Azithromycin]] 12mg/kg once (500mg max) and then 6mg/kg (250mg max) daily for the next 4 days
*Prophylaxis
**[[Penicillin G]] IM x1 month
::{| class="wikitable" border="1"
|-
! Category
! Duration
|-
| Rheumatic fever without carditis
| 5 years or until age 21 years, whichever is longer
|-
| Rheumatic fever with carditis but without residual heart disease (no valvular disease)
| 10 years or well into adulthood, whichever is longer
|-
| Rheumatic fever with carditis and residual heart disease (persistent valvular disease)
| 10 years since last episode and at least until age 40 years; most commonly lifelong
|}
*Arthritis
**High-dose [[aspirin]] therapy (50-100mg/kg/day) has historically been first line
**Naproxen may be just as effective with less frequent dosing and fewer side effects <ref>The efficacy and safety of naproxen in acute rheumatic fever: The comparative results of 11-year experience with acetylsalicylic acid and naproxen.Turk J Pediatr. 2016;58(5):473. </ref>
*Carditis
**Aspirin is primary treatment
**[[Prednisone]] 1-2mg/kg/day has no benefit over aspirin in proventing heart disease at one year <ref>  Cilliers A, Adler AJ, Saloojee H. Anti-inflammatory treatment for carditis in acute rheumatic fever.Cochrane Database Syst Rev. 2015 </ref>
***Some consider for severe carditis
**Standard medical therapy for [[Congestive Heart Failure]]
*Chorea - It is self limited but several agents had been described as effective, although not well studied
**[[Haloperidol]] 0.01-0.03mg/kg/day in four divided doses
**[[Valproate]]
**[[Diazepam]]
**[[Phenobarbital]]
**[[Chlorpromazine]]


3/10 Rosen
==Complications==
*Rheumatic Heart Disease
**Worldwide most common cause of valvular heart disease
**Occurs usually 10+ years after RF
**Mitral valve is most commonly affected, followed by aortic valve
**50% of those with carditis during acute RF
*Jaccoud arthropathy
**Painless deformities without joint inflammation


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


==External Links==
* [http://www.emdocs.net/an-understated-myth-strep-throat-rheumatic-fever/ emDocs - An Understated Myth? Strep Throat & Rheumatic Fever]
* [https://www.cdc.gov/groupastrep/diseases-hcp/strep-throat.html/ CDC guidelines for GAS Disease]
==See Also==
*[[Sydenham's chorea]]
==References==
<references/>
[[Category:Pediatrics]]
[[Category:ID]]
[[Category:ID]]
[[Category:Cardiology]]

Latest revision as of 21:48, 3 May 2024

Background

  • Primarily affects school age children 2-6 weeks after Group A streptococcal pharyngitis
  • Connective tissue of heart, joints, CNS, subcutaneous tissues are targeted by immune reaction

Clinical Features

  • Polyarthritis
    • Most common symptom (~75%)
    • Aseptic arthritis with effusions
    • Lasts up to 2 weeks
    • Migratory, fleeting polyarticular arthritis primarily affecting large joints
  • Carditis (33%)
    • Most serious complication and second most common
    • Tachycardia out of proportion to the degree of fever is common; its absence makes the diagnosis of myocarditis unlikely[1]
    • New murmur, pericardial rub, CHF
  • Sydenham's chorea (10%)
    • May appear months following strep infection, may be sole manifestation of RF
    • Movements extinguish during sleep
  • Erythema marginatum (< 10%)
    • Persists only for several days
    • Usually coexists with presence of carditis in some form
    • Nonpruritic rash, located on trunk and proximal limbs, never on face
  • Nodules(< 10%)
    • Located on extensor surfaces of wrists, elbows, knees

Differential Diagnosis

Pediatric limp

Hip Related

Other Causes of Limping

Polyarthritis

Algorithm for Polyarticular arthralgia

Evaluation

Work-Up

  • CBC
  • ECG
  • CXR
  • ESR, CRP
  • ASO (Antistreptolysin O) titer Eleveted 1week to 1 month after GAS infection
  • Anti-DNase B titer is elevated longer than ASO and may be useful for patient presenting later than 2-4 months or with a negative ASO
  • ECG - Looking for prolonged PR or other AV block
  • CXR
  • Echocardiogram

Modified Jones Criteria (1992) for Acute Rheumatic Fever

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

  • Major diagnostic criteria (CASES)
    • Carditis (new or changing murmur, cardiomegaly, CHF, pericarditis)
    • Arthritis, migratory polyarthritis (typically affects knees, ankles, elbows, wrists)
    • Sydenham's Chorea (abrupt, involuntary, purposeless movements)
    • Erythema marginatum (non-pruritic, trunk/extremities, with facial sparing)
    • Subcutaneous nodules (painless, firm, usually over bones/tendons)
  • Minor diagnostic criteria
    • Fever
    • Arthralgia
    • History of previous attack of rheumatic fever
    • Prolonged PR interval
    • Elevated ESR, CRP
  • Evidence of preceding streptococcal infection

Management

  • Penicillin - Indicated for all with rheumatic fever even if culture for strep is negative
  • For those who are penicillin allergic
    • Narrow spectrim cephalosporins such as cephalexin 20mg/kg/dose BID x10d
    • Clindamycin 7mg/kg/dose TID (max of 300mg/dose) PO x10d
    • Azithromycin 12mg/kg once (500mg max) and then 6mg/kg (250mg max) daily for the next 4 days
  • Prophylaxis
Category Duration
Rheumatic fever without carditis 5 years or until age 21 years, whichever is longer
Rheumatic fever with carditis but without residual heart disease (no valvular disease) 10 years or well into adulthood, whichever is longer
Rheumatic fever with carditis and residual heart disease (persistent valvular disease) 10 years since last episode and at least until age 40 years; most commonly lifelong
  • Arthritis
    • High-dose aspirin therapy (50-100mg/kg/day) has historically been first line
    • Naproxen may be just as effective with less frequent dosing and fewer side effects [2]
  • Carditis
    • Aspirin is primary treatment
    • Prednisone 1-2mg/kg/day has no benefit over aspirin in proventing heart disease at one year [3]
      • Some consider for severe carditis
    • Standard medical therapy for Congestive Heart Failure
  • Chorea - It is self limited but several agents had been described as effective, although not well studied

Complications

  • Rheumatic Heart Disease
    • Worldwide most common cause of valvular heart disease
    • Occurs usually 10+ years after RF
    • Mitral valve is most commonly affected, followed by aortic valve
    • 50% of those with carditis during acute RF
  • Jaccoud arthropathy
    • Painless deformities without joint inflammation

Disposition

  • Admit

External Links

See Also

References

  1. ↑ Park M: Parks's Pediatric Cardiology for Practitioners, ed 6. Philadelphia, Saunders-Elsevier., 2014, (Ch) 20: p 368.
  2. ↑ The efficacy and safety of naproxen in acute rheumatic fever: The comparative results of 11-year experience with acetylsalicylic acid and naproxen.Turk J Pediatr. 2016;58(5):473.
  3. ↑ Cilliers A, Adler AJ, Saloojee H. Anti-inflammatory treatment for carditis in acute rheumatic fever.Cochrane Database Syst Rev. 2015
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