Author:
ChristopherGardner
DaltonCox
Description
- Constellation of symptoms and signs (revised Jones criteria)
- Follows group A streptococcal infection (GAS) also known as Streptococcus pyogenes; usually pharyngitis
- Uncommon in the U.S.; most cases are in developing nations, more common in indigenous populations in Australia and New Zealand
- Remains a major cause of cardiac morbidity and mortality worldwide with over 230,000 deaths per year
- Most common in 5-15-yr olds
Etiology
- GAS pharyngitis (not associated with GAS skin infections)
- Inflammatory, autoimmune response following GAS infection attacking connective tissue
- 2 major or 1 major and 2 minor elements of the revised Jones criteria plus evidence of a recent GAS infection
- In patients with prior documented acute rheumatic fever (ARF), presence of 3 minor criteria with recent GAS infection also diagnostic
Signs and Symptoms
Revised Jones Criteria
- Criteria differs for low risk and moderate-high risk populations
- Low-risk populations are defined as:
- ARF incidence <2/100,000
- Children 5-14 yr old
- RHD incidence <1/1,000 per year
- Otherwise, considered moderate-high risk
- Moderate-high risk criteria
- Children not clearly from a low risk population
- These criteria are italicized below
- Major manifestations:
- Migratorypolyarthritis in 35-66% of initial attacks:
- Frequently earliest presenting symptom
- Involves larger joints: Knees, hips, ankles, elbows, and wrists
- Lower extremity joints more commonly involved
- Generally responds rapidly to salicylates and NSAIDs
- Monoarthritis OR polyarthralgia can fulfill “major manifestation” criterion in moderate-high risk populations
- Carditis occurs in 50-70% of new cases:
- Pericardium, myocardium, and endocardium may be affected (pancarditis)
- Myocarditis may lead to heart failure but is frequently asymptomatic
- Rheumatic heart disease (RHD) and endocarditis are most serious sequelae of ARF
- Carditis heralded by a new murmur, tachycardia, gallop rhythm, pericardial friction rub, or CHF
- Echocardiogram with Doppler may detect valvulitis in absence of auscultatory findings (subclinical carditis)
- Chorea occurs in 10% of cases:
- Sydenham chorea predominantly affects teenage girls
- Purposeless, uncoordinated movements of the extremities sometimes called St. Vitus dance
- More apparent during periods of anxiety
- Chorea may be the sole manifestation of ARF
- Erythema marginatum occurs in <5% of cases:
- Nonpruritic pink eruptions with central clearing and well-demarcated irregular borders
- Usually seen on the trunk and the extremities
- Subcutaneous nodules in small percentage of patients:
- Crops of small subcutaneous painless nodules located most commonly on extensor surfaces
- Minor manifestations:
- Clinical:
- Fever >38.5°C (38°C in mod-high risk)
- Polyarthralgia (monoarthralgia in mod-high risk))
- Lab:
- ESR >60 mm, CRP >3 mg/dL (ESR≥30 in mod-high risk))
- Prolonged P-R interval
- Supporting evidence of GAS throat infection:
- Positive throat culture or rapid antigen test
- Elevated or increasing antibody test: Antistreptolysin O (ASO) titer
History
- Fever (>38.5°C) (38°C in mod-high risk)
- Sore throat (often 2-4 wk prior)
- Rash
- Joint pains
- Unusual movements of extremities
- Dyspnea
- Personal or family history of ARF
Physical Exam
- Pharyngeal erythema
- Rash consistent with erythema marginatum
- SC nodules
- New heart murmur consistent with mitral or aortic disease
- Evidence of fluid overload/CHF
Essential Workup
- Careful exam to look for skin lesions/joint swelling
- Careful heart and lung exam
- Throat swab for rapid strep test or culture
- ECG
- CXR
- Echocardiogram with Doppler
- See other labs below
Diagnostic Tests & Interpretation
Lab
- Rapid antigen strep test
- Throat culture
- ASO titer
- CBC
- ESR or C-reactive protein
Imaging
- Echocardiogram with Doppler
- CXR
Diagnostic Procedures/Surgery
- ECG
- Diagnosis is based on clinical picture and meeting Jones criteria
Differential Diagnosis
- Juvenile idiopathic arthritis
- Infective endocarditis
- Reactive arthropathy
- Systemic lupus erythematosus
- Poststreptococcal arthritis
- Other infectious causes of arthritis and carditis:
- Coxsackie B virus and parvovirus
Pediatric Considerations |
- Rheumatic fever is primarily a pediatric disease but can occur in young adults
- Testing for strep throat is not recommended under 3 yr of age in the U.S. due to low incidence of strep throat and rare ARF
|
Pregnancy Prophylaxis |
| Prenatal counseling recommended if woman has a history of rheumatic fever due to increased cardiac risks |
Prehospital
- Oxygen as needed
- Monitors if in distress
- IV access may be prudent
Initial Stabilization/Therapy
Some patients in CHF will need airway management
ED Treatment/Procedures
Medication
- Aspirin: 4-8 g/d (peds: 100 mg/kg/d) PO q4-6h; do not exceed 4 g/24 h
- Azithromycin (PCN allergy): 500 mg day 1, then 250 mg PO for 4 more days (peds: 10 mg/kg day 1 then 5 mg/kg daily PO for 4 more days)
- Digoxin: 0.25-0.5 mg (peds: 0.04 mg/kg) IV
- Furosemide: 20-80 mg (peds: 1 mg/kg/dose) IV
- Haloperidol: 2-10 mg (peds: 0.01-0.03 mg/kg/d; use only >2 yr and >15 kg) q6h IM/PO
- Naproxen: 10-20 mg/kg/d PO in divided doses every 12 h for 1-2 wk; do not exceed 1 g/d
- Penicillin G: 1.2 million units (peds: 600,000 units for <27 kg) IM acutely and monthly thereafter (prophylaxis)
- Penicillin VK: 500 mg (peds: 250 mg) PO q8h for 10 d (acute treatment)
- Prednisone: 1-2 mg/kg/d for 14 d with taper for the next 2 wk
First Line
- Aspirin (carditis patients)
- Penicillin G (GAS eradication)
- Haloperidol (for chorea)
Second Line
Corticosteroids
Disposition
Most patients with a new diagnosis should be admitted for stabilization and further evaluation of the severity of the heart disease
Admission Criteria
- CHF
- New diagnosis
- Uncontrolled chorea
- Uncontrolled pain
- Pericardial effusion
Discharge Criteria
- Pain is controlled
- Stable cardiovascular status
- Education regarding prolonged treatment and endocarditis prophylaxis with reliable follow-up
Issues for Referral
- All patients need close follow-up with their primary physician and cardiologist
- Consider referral to infectious disease specialist and rheumatologist
Follow-up Recommendations
- Cardiology for serial echocardiogram and advice on subacute bacterial endocarditis prophylaxis
- Infectious disease specialist to advise on prolonged use of penicillin to prevent recurrence
- Rheumatology if needed for chronic joint problems (uncommon)
- American Academy of Pediatrics. Group A streptococcal infection. In: KimberlinDW, BradyMT, JacksonMA, et al. Red Book 2015 Report of the Committee of Infectious Diseases. 668-680.
- ChangC. Cutting edge issues in rheumatic fever . Clin Rev Allergy Immunol. 2012;42:213-237.
- GewitzMH, BaltimoreRS, TaniLY, et al.; American Heart Association Committee on Rheumatic Fever, Endocarditis, and Kawasaki Disease of the Council on Cardiovascular Disease in the Young. Revision of the Jones Criteria for the diagnosis of acute rheumatic fever in the era of Doppler echocardiography: A scientific statement from the American Heart Association . Circulation. 2015;31:1806.
- RHDAustralia (ARF/RHD writing group), National Heart Foundation of Australia and the Cardiac Society of Australia and New Zealand . Australian guideline for prevention, diagnosis and management of acute rheumatic fever and rheumatic heart disease. 2nd ed. 2012.
- WebbRH, GrantC, HarndenA. Acute rheumatic fever . BMJ. 2015;351:h3443.
See Also (Topic, Algorithm, Electronic Media Element)
Pharyngitis