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<languages/>

==Background==

*Primarily affects school age children 2-6 weeks after Group A [[Special:MyLanguage/streptococcal pharyngitis|streptococcal pharyngitis]]
**Is not associated with [[Special:MyLanguage/impetigo|impetigo]]
*Connective tissue of heart, joints, CNS, subcutaneous tissues are targeted by immune reaction

==Clinical Features==

*[[Special:MyLanguage/Arthritis|Polyarthritis]]
**Most common symptom (~75%)
**Aseptic arthritis with effusions
**Lasts up to 2 weeks
**Migratory, fleeting polyarticular arthritis primarily affecting large joints
*[[Special:MyLanguage/Myocarditis|Carditis]] (33%)
**Most serious complication and second most common
**[[Special:MyLanguage/Tachycardia|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 [[Special:MyLanguage/murmur|murmur]], pericardial rub, [[Special:MyLanguage/CHF|CHF]]
*[[Special:MyLanguage/Sydenham's chorea|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 [[Special:MyLanguage/rash|rash]], located on trunk and proximal limbs, never on face
*Nodules(< 10%)
**Located on extensor surfaces of wrists, elbows, knees

==Differential Diagnosis==

*[[Special:MyLanguage/Scarlet fever|Scarlet fever]]
*[[Special:MyLanguage/Kawasaki Disease|Kawasaki Disease]]
*Viral or other forms of [[Special:MyLanguage/cardiomyopathy|cardiomyopathy]]
*[[Special:MyLanguage/Leukemia|Leukemia]]
*[[Special:MyLanguage/Vasculitis|Vasculitis]] ([[Special:MyLanguage/HSP|HSP]], drug reaction)

{{Pediatric hip DDX}}

{{Differential Diagnosis Polyarthritis}}

==Evaluation==

===Work-Up===

*CBC
*[[Special:MyLanguage/ECG|ECG]]
*[[Special:MyLanguage/CXR|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 [[Special:MyLanguage/murmur|murmur]], cardiomegaly, [[Special:MyLanguage/CHF|CHF]], [[Special:MyLanguage/pericarditis|pericarditis]])
**[[Special:MyLanguage/Arthritis|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
**[[Special:MyLanguage/Fever|Fever]]
**[[Special:MyLanguage/Arthralgia|Arthralgia]]
**History of previous attack of rheumatic fever
**Prolonged PR interval
**Elevated ESR, CRP
*Evidence of preceding [[Special:MyLanguage/streptococcal|streptococcal]] infection
**Increased ASO or other strep ab
**Positive throat culture for [[Special:MyLanguage/Group A strep|Group A strep]]
**Positive rapid GAS
**Recent [[Special:MyLanguage/scarlet fever|scarlet fever]]

==Management==

*[[Special:MyLanguage/Penicillin|Penicillin]] - Indicated for all with rheumatic fever even if culture for [[Special:MyLanguage/strep|strep]] is negative
**[[Special:MyLanguage/Penicillin G|Penicillin G]] 600K units IM if <27 kg, 1.2 million units IM if >27 kg
**[[Special:MyLanguage/Penicillin V|Penicillin V]] PO x10d
*For those who are penicillin allergic
**Narrow spectrim cephalosporins such as [[Special:MyLanguage/cephalexin|cephalexin]] 20mg/kg/dose BID x10d
**[[Special:MyLanguage/Clindamycin|Clindamycin]] 7mg/kg/dose TID (max of 300mg/dose) PO x10d
**[[Special:MyLanguage/Azithromycin|Azithromycin]] 12mg/kg once (500mg max) and then 6mg/kg (250mg max) daily for the next 4 days
*Prophylaxis
**[[Special:MyLanguage/Penicillin G|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 [[Special:MyLanguage/aspirin|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
**[[Special:MyLanguage/Prednisone|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 [[Special:MyLanguage/Congestive Heart Failure|Congestive Heart Failure]]
*Chorea - It is self limited but several agents had been described as effective, although not well studied
**[[Special:MyLanguage/Haloperidol|Haloperidol]] 0.01-0.03mg/kg/day in four divided doses
**[[Special:MyLanguage/Valproate|Valproate]]
**[[Special:MyLanguage/Diazepam|Diazepam]]
**[[Special:MyLanguage/Phenobarbital|Phenobarbital]]
**[[Special:MyLanguage/Chlorpromazine|Chlorpromazine]]

==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==

* [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==

*[[Special:MyLanguage/Sydenham's chorea|Sydenham's chorea]]

==References==

<references/>

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

Acute rheumatic fever/en - Revision history

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