Acute rheumatic fever: Difference between revisions

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==I. Diagnosis==
==Background==
*Primarily affects school age children 2-6 weeks after [[strep pharyngitis]]
*Connective tissue of heart, joints, CNS, subq tissues are targeted by immune reaction


Modified Jones Criteria (1992) for Acute Rheumatic Fever
==Clinical Features==
*[[Arthritis|Polyarthritis]]
**Most common symptom (75%)
**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]]
*[[Chorea]] (10%)
**May appear months following strep infection, may be sole manifestation of RF
*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


==III. Treatment==
==Evaluation==
===Work-Up===
*CBC
*[[ECG]]
*[[CXR]]
*ESR, CRP


===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, 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


A. Eradicate GAS infection
==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
**[[Erythromycin]] x10d if penicillin-allergic
*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 (75-100mg/kg/day)
*Carditis - [[Prednisone]] 1-2mg/kg/day
*Chorea - [[Haloperidol]] 0.01-0.03mg/kg/day in four divided doses


1. PCN 0.6 to 1.3 million Units IM x 1 or
==Disposition==
*Admit


2. Oral PCN for 10 days or
==External Links==


3. Erythromycin if PCN-allergic
B. Future prophylaxis
C. Treatment of symptoms, e.g. arthritis
==Source ==
7/2/09 PANI
3/10 Rosen


==See Also==




==References==
<references/>


[[Category:Pediatrics]]
[[Category:ID]]
[[Category:ID]]
[[Category:Cardiology]]

Revision as of 16:29, 14 September 2019

Background

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

Clinical Features

  • Polyarthritis
    • Most common symptom (75%)
    • 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
  • Chorea (10%)
    • May appear months following strep infection, may be sole manifestation of RF
  • 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 hip pain

Polyarthritis

Algorithm for Polyarticular arthralgia

Evaluation

Work-Up

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, 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

Management

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 (75-100mg/kg/day)
  • Carditis - Prednisone 1-2mg/kg/day
  • Chorea - Haloperidol 0.01-0.03mg/kg/day in four divided doses

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.