Basics
Description
- Constellation of symptoms and signs (Jones criteria)
- Follows group A streptococcal infection (GAS) also known as Streptococcus pyogenes; usually pharyngitis
- Uncommon in US; most cases are in developing nations
- Remains a major cause of cardiac morbidity and mortality worldwide with over 230,000 deaths per year
- Most common in 5- to 15-yr-olds
Etiology
- GAS infection
- Inflammatory, autoimmune response following GAS infection
Diagnosis
2 major or 1 major and 2 minor elements of the Jones criteria plus evidence of a recent GAS infection
Signs and Symptoms
Jones Criteria
- Major manifestations:
- Migratory polyarthritis in 60 " 75% of initial attacks:
- Involves larger joints: Knees, hips, ankles, elbows, and wrists
- Lower extremity joints more commonly involved
- Rheumatic arthritis generally responds to salicylates
- Carditis occurs in 1/3 to 1/2 of new cases:
- Pericardium, myocardium, and endocardium may be affected (pancarditis)
- Myocarditis may lead to heart failure but is frequently asymptomatic
- Valvular disease and endocarditis are most serious sequelae of acute rheumatic fever (ARF)
- Carditis heralded by a new murmur, tachycardia, gallop rhythm, pericardial friction rub, or CHF
- Echocardiogram aids in diagnosis
- Chorea occurs in 10% of cases:
- Sydenham chorea predominantly affects teenage girls
- Purposeless, uncoordinated movements of the extremities sometimes called St. Vitas dance
- Movements are more apparent during periods of anxiety and disappear with sleep
- Chorea may be the sole manifestation of ARF
- Other neuropsychiatric symptoms of emotional lability or obsessive compulsive disorder may also occur
- 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
- SC nodules in small percentage of patients:
- Crops of small SC, painless nodules located most commonly on extensor surfaces
- Minor manifestations:
- Clinical:
- Fever (>38 °C)
- Arthralgia
- Lab:
- Elevated acute phase reactants
- Prolonged P-R interval
- Supporting evidence of recent GAS throat infection:
- Positive throat culture or rapid antigen test
- Elevated or increasing antibody test: Antistreptolysin O (ASO) titer
History
- Fever
- Sore throat (often 2 " 4 wk prior)
- Rash
- Joint pains
- Unusual movements of extremities
- Dyspnea
- Lower extremity edema
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
- Chest x-ray
- Echocardiogram
- See other labs below
Diagnosis Tests & Interpretation
Lab
- Rapid antigen strep test
- Throat culture
- ASO titer
- CBC
- ESR or C-reactive protein
- Other serology tests to rule out other rheumatologic diseases
Imaging
- Chest radiograph
- Echocardiogram
Diagnostic Procedures/Surgery
- ECG
- Diagnosis is based on clinical picture and meeting Jones criteria
Differential Diagnosis
- Juvenile idiopathic arthritis
- Infective endocarditis
- Reiter syndrome
- Systemic lupus erythematosus
- Postgonococcal arthritis
- Other infectious causes of arthritis and carditis:
- Coxsackie B virus and parvovirus
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 US due to low incidence of strep throat and rare ARF
Prenatal counseling recommended if woman has a history of rheumatic fever due to increased cardiac risks
Treatment
Pre-Hospital
- 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
- Pericardial effusions may need drainage
- In severe carditis, start prednisone
- In case of severe chorea, start haloperidol
- Penicillin IM, IV, or PO
- Aspirin for arthritis/arthralgia
Medication
- Aspirin: 4 " 8 g/d (peds: 100 mg/kg/d) PO q4 " 6h; do not exceed 4 g/24h
- Azithromycin 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
- Erythromycin: 250 mg (peds: 30 " 50 mg/kg/d) q6h PO for 10 days
- 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 or PO
- Penicillin (benzathine benzylpenicillin): 1.2 million U (peds: 600,000 U for <27 kg) IM acutely and monthly thereafter (prophylaxis)
- Penicillin VK: 500 mg (peds: 250 mg) PO q8h for 10 days (acute treatment)
- Prednisone: 1 " 2 mg/kg/d for 14 days with taper for the next 2 wk
First Line
- Aspirin (carditis patients)
- Penicillin
- Haloperidol (for chorea)
Second Line
Corticosteroids
Follow-Up
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
- Patient has reliable follow-up option
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 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)
Pearls and Pitfalls
- Rheumatic fever is uncommon in US, but must be vigilant to treat strep infections to prevent resurgence of disease
- More common in patients living in poor and crowded conditions
- No need to do throat cultures in children under age 3
Additional Reading
- American Academy of Pediatrics. Group A Streptococcal infections. In: Pickering LK, Baker CJ, Kimberlin DW, et al., eds. Red Book 2012 Report of the Committee of Infectious Diseases. 668 " 680.
- Carapetis JR, McDonald M, Wilson NJ. Acute rheumatic fever. Lancet. 2005;366:155 " 168.
- Chang, C. Cutting edge issues in rheumatic fever. Clin Rev Allergy Immunol. 2012;42:213 " 237.
- Cilliers AM. Rheumatic fever and its management. BMJ. 2006;333:1153 " 1156.
- Gerber MA, Baltimore RS, Eaton CB, et al. Prevention of rheumatic fever and diagnosis and treatment of acute Streptococcal pharyngitis: A scientific statement from the American Heart Association Rheumatic Fever, Endocarditis, and Kawasaki Disease Committee of the Council on Cardiovascular Disease in the Young, the Interdisciplinary Council on Functional Genomics and Translational Biology, and the Interdisciplinary Council on Quality of Care and Outcomes Research: Endorsed by the American Academy of Pediatrics. Circulation. 2009;119:1541 " 1551.
- Miyake CY, Gauvreau K, Tani LY, et al. Characteristics of children discharged from hospitals in the United States in 2000 with the diagnosis of acute rheumatic fever. Pediatrics. 2007;120:503 " 508.
- Weiner SG, Normandin
PA. Sydenham chorea: A case report and review of the literature. Pediatr Emerg
Care.
2007;23:20 " 24.
See Also (Topic, Algorithm, Electronic Media Element)
Pharyngitis
Codes
ICD9
- 390 Rheumatic fever without mention of heart involvement
- 391.9 Acute rheumatic heart disease, unspecified
- 714.0 Rheumatoid arthritis
- 392.9 Rheumatic chorea without mention of heart involvement
- 391.0 Acute rheumatic pericarditis
- 391.1 Acute rheumatic endocarditis
- 391.2 Acute rheumatic myocarditis
- 391.8 Other acute rheumatic heart disease
- 391 Rheumatic fever with heart involvement
- 392.0 Rheumatic chorea with heart involvement
- 392 Rheumatic chorea
ICD10
- I00 Rheumatic fever without heart involvement
- I01.9 Acute rheumatic heart disease, unspecified
- M06.9 Rheumatoid arthritis, unspecified
- I02.9 Rheumatic chorea without heart involvement
- I01.0 Acute rheumatic pericarditis
- I01.1 Acute rheumatic endocarditis
- I01.2 Acute rheumatic myocarditis
- I01.8 Other acute rheumatic heart disease
- I01 Rheumatic fever with heart involvement
- I02.0 Rheumatic chorea with heart involvement
- I02 Rheumatic chorea
SNOMED
- 58718002 Rheumatic fever (disorder)
- 14175009 Rheumatic joint disease (disorder)
- 194708008 Rheumatic fever with heart involvement
- 46826000 Rheumatic chorea (disorder)
- 15676002 Acute rheumatic pericarditis (disorder)
- 18192007 Acute rheumatic endocarditis (disorder)
- 194709000 Acute rheumatic myocarditis (disorder)