Acute rheumatic fever: Difference between revisions

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


==Diagnosis==
==Clinical Features==
Modified Jones Criteria (1992) for Acute Rheumatic Fever
*[[Arthritis|Polyarthritis]]
 
REQUIRE: 2 major or 1 major and 2 minor criteria and evidence of previous GAS pharyngitis.
 
#Major diagnostic criteria
##Carditis
###New or changing murmur, cardiomegaly, CHF, pericarditis
##Migratory polyarthritis
##Chorea
##Subcutaneous nodules
##Erythema marginatum
#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
 
==Symptoms==
*Polyarthritis
**Most common symptom (75%)
**Most common symptom (75%)
**Migratory, fleeting polyarticular arthritis primarily affecting large joints
**Migratory, fleeting polyarticular arthritis primarily affecting large joints
*Carditis (33%)
*[[Myocarditis|Carditis]] (33%)
**Most serious complication and second most common
**Most serious complication and second most common
***New murmur, pericardial rub, CHF
**[[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%)
*[[Chorea]] (10%)
**May appear months following strep infection, may be sole manifestation of RF
**May appear months following strep infection, may be sole manifestation of RF
*Erythema marginatum
*Erythema marginatum (< 10%)
**Persists only for several days
**Persists only for several days
**Usually coexists with presence of carditis in some form
**Usually coexists with presence of carditis in some form
**Nonpruritic, located on trunk and proximal limbs, never on face  
**Nonpruritic [[rash]], located on trunk and proximal limbs, never on face  
*Nodules
*Nodules(< 10%)
**Located on extensor surfaces of wrists, elbows, knees
**Located on extensor surfaces of wrists, elbows, knees


==DDX==
==Differential Diagnosis==
#JIA
*[[Scarlet fever]]
#[[Septic Arthritis]]
*[[Kawasaki Disease]]
#[[Kawasaki Disease]]
*Viral or other forms of [[cardiomyopathy]]
#Viral or other forms of cardiomyopathy
*[[Leukemia]]
#Leukemia
*[[Vasculitis]] ([[HSP]], drug reaction)
#Vasculitis ([[HSP]], drug reaction)
 
#[[Hip Pain (Peds)]]
{{Pediatric hip DDX}}
 
{{Differential Diagnosis Polyarthritis}}
 
==Evaluation==
===Work-Up===
*CBC
*[[ECG]]
*[[CXR]]
*ESR, CRP


==Work-Up==
===Modified Jones Criteria (1992) for Acute Rheumatic Fever===
#CBC
''REQUIRE: 2 major or 1 major and 2 minor criteria and evidence of previous GAS pharyngitis''
#ECG
*Major diagnostic criteria (CASES)
#CXR
**Carditis (new or changing [[murmur]], cardiomegaly, [[CHF]], [[pericarditis]])
#ESR, CRP
**[[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]]


==Treatment==
==Management==
*Penicillin
*[[Penicillin]] - Indicated for all with rheumatic fever even if culture for [[strep]] is negative
**Indicated for all pts w/ rheumatic fever even if cx for strep is negative
**[[Penicillin G]] 600K units IM if <27 kg, 1.2 million units IM if >27 kg
**600K units IM if <27 kg
**[[Penicillin V]] PO x10d
**1.2 million units IM if >27 kg
**[[Erythromycin]] x10d if penicillin-allergic
**Penicillin V PO x10d
*Prophylaxis
**Prophylaxis
**[[Penicillin G]] IM x1 month
***5yr if no cardiac involvement, lifetime if cardiac involvement
::{| class="wikitable" border="1"
***Pen G IM q month or oral penicillin daily
|-
**Erythromycin x10d if pen allergic
! Category
*Arthritis
! Duration
**High-dose aspirin therapy (75-100 mg/kg/d)  
|-
*Carditis
| Rheumatic fever without carditis
**Prednisone 1-2mg/kg/d
| 5 years or until age 21 years, whichever is longer
*Chorea
|-
**Haloperidol 0.01-0.03 mg/kg/d in four divided doses
| 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==
==Disposition==
*Admit for confirmation of diagnosis
*Admit
 
==External Links==
 
 
==See Also==
 


==Source ==
==References==
Tintinalli
<references/>


[[Category:Peds]]
[[Category:Pediatrics]]
[[Category:ID]]
[[Category:ID]]
[[Category:Cards]]
[[Category:Cardiology]]

Revision as of 20:26, 5 October 2019

Background

  • Primarily affects school age children 2-6 weeks after strep pharyngitis
  • Connective tissue of heart, joints, CNS, subcutaneous 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

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.