Rheumatic Fever

Rheumatic Fever: Excerpt from The 5-Minute Pediatric Consult

David Hehir, MD

Rheumatic Fever - BASICS

Rheumatic Fever - description

A postinfectious inflammatory disease caused by rheumatogenic strains of group A β-hemolytic Streptococci (GABHS). Clinically diagnosed using the Jones criteria, acute rheumatic fever (ARF) results in a wide range of disease, from mild joint involvement to chronic carditis. The most significant health care and socioeconomic impact is caused by its most severe form, rheumatic heart disease (RHD).

Rheumatic Fever - epidemiology

• Classic teaching is that GABHS strains that cause pharyngitis are associated with ARF, whereas strains that cause impetigo are associated with glomerulonephritis. Although this holds true in temperate regions, recent research reveals that ARF can be associated with skin infections in tropical and underdeveloped areas of the world.
• Initial episode seen primarily in patients 5–15 years of age
• No racial or ethnic predilections

Rheumatic Fever - incidence

• Historically, untreated GABHS infection results in ARF in 0.1–0.3% of cases, with attack rates as high as 3% in endemic areas.
• Recent incidence data reveal 0.5/100,000 school-aged children in industrialized countries are affected. Incidence is as high as 500/100,000 in tropical and underdeveloped countries.
• Decrease in incidence due to increased use of antibiotics, improved environmental factors such as overcrowding, and changing virulence patterns of GABHS strains.

Rheumatic Fever - prevalence

12 million people are affected by ARF worldwide, with 400,000 cases of RHD. This accounts for 25–40% of all cardiac disease worldwide.

Rheumatic Fever - risk factors

Rheumatic Fever - genetics

No specific genetic risk factor identified, although numerous studies have demonstrated an association of ARF with specific human leukocyte antigen (HLA) alleles.

Rheumatic Fever - pathophysiology

• GABHS triggers a complex inflammatory host response affecting the heart, joints, brain, blood vessels, and subcutaneous tissue.
• Classic example of molecular mimicry, in which the host produces antibodies to certain GABHS M proteins which are similar in structure to host proteins such as myosin, resulting in autoimmune tissue damage.
• Aschoff nodules are proliferative lesions noted in the myocardium that may persist for months to years after initiation of disease.

Rheumatic Fever - etiology

Immune-mediated inflammatory reaction to specific rheumatogenic strains of GABHS.

Rheumatic Fever - DIAGNOSIS

Rheumatic Fever - signs & symptoms

Rheumatic Fever - history

• A clinical diagnosis based on the modified Jones criteria (updated 1992): Evidence of recent GABHS infection with the presence of either 2 major or 1 major and 2 minor criteria:
• Major criteria:
  • Polyarthritis: 70%; migratory arthritis of major joints; more common in adults
  • Carditis: 50%; mitral regurgitation most common (85%), then aortic valve involvement (54%); symptoms range from asymptomatic murmur to fulminant heart failure; more common and more severe in children
  • Sydenham chorea: 15%; abnormal behavior and/or involuntary, purposeless movements
  • Erythema marginatum: 10%; evanescent, pink rash with serpiginous borders
  • Subcutaneous nodules: 2–10%; painless nodules over extensor surfaces of large joints, the occiput, and/or vertebral processes
• Minor criteria:
  • Fever
  • Arthralgia (mild pain without objective findings): Can only be considered without finding of arthritis
  • Elevated acute-phase reactants: ESR, C-reactive protein
  • Prolongation of the PR interval on electrocardiogram
• Exceptions to the Jones criteria include:
  • Sydenham chorea alone
  • Subclinical carditis (echocardiogram evidence of RHD) in the absence of other criteria should be treated as ARF.
  • Jones criteria cannot be applied to recurrence; World Health Organization (WHO) recommends treating recurrence in a patient with RHD and presence of any major or minor criterion.

Rheumatic Fever - physical exam

• Cardiac:
  • Murmur of valvulitis: Holosystolic mitral regurgitant murmur, Carey–Coombs apical mid-diastolic murmur, or a basal diastolic murmur of aortic insufficiency (major criterion)
  • Pericardial friction rub: Pericardial effusion
• Musculoskeletal:
  • Pain, limited motion, erythema, warmth of 2 or more large joints: Arthritis (major criterion)
• Neurologic: Choreiform movements (must be differentiated from tics, athetosis, and hyperkinesis): Sydenham chorea (major criterion)
• Dermatologic:
  • Evanescent, pink rash with pale centers and serpiginous borders on the trunk and proximal extremities: Erythema marginatum (major criterion)
  • Firm, painless nodules over the extensor surface of large joints, occiput, and/or spinous processes: Subcutaneous nodules (major criterion)

Rheumatic Fever - tests

Rheumatic Fever - lab

• Specific tests: No specific diagnostic test is available.
• Nonspecific tests:
  • Throat culture: Neither sensitive nor specific. May be negative in 2/3 of affected patients, or patient may be a chronic carrier.
  • Elevated or rising streptococcal antibody titers, antistreptolysin O, anti-DNase B, antihyaluronidase
  • ESR and C-reactive protein elevation

Rheumatic Fever - imaging

• EKG: Prolonged PR interval (minor criterion), junctional rhythm, transient arrhythmias, ST-T wave changes
• Chest x-ray: Cardiomegaly may indicate carditis or pericardial effusion. Pulmonary edema may reflect left heart failure due to valvulitis.
• Echocardiogram: Assess valve involvement, ventricular dilatation, function, and pericardial effusion.

Rheumatic Fever - differencial diagnosis

• Carditis:
  • Viral
  • Bacterial
  • Rickettsial
  • Parasitic
  • Mycoplasma myocarditis
  • Kawasaki disease
• Arthritis:
  • Post-streptococcal reactive arthritis (PSRA)
  • Serum sickness
  • Septic arthritis (i.e., gonococcal)
  • Lyme disease
• Collagen vascular disease:
  • Juvenile rheumatoid arthritis (small joints, not migratory, and not relieved promptly with aspirin)
  • Systemic lupus erythematosus
  • Bacterial endocarditis
• Chorea:
  • Congenital choreoathetosis
  • Brain tumors
  • Huntington chorea
  • Wilson disease
  • Pediatric autoimmune neuropsychiatric disorders associated with streptococcus (PANDAS)
• Hematologic disorders with joint involvement:
  • Sickle cell anemia
  • Leukemia
• Congenital heart defects: Previously undiagnosed valvular heart disease
• Mitral valve prolapse with regurgitation

Rheumatic Fever - TREATMENT

Rheumatic Fever - initial stabilization

Full treatment of Streptococcal pharyngitis infection and cardiac support if heart failure present. Treatment phases include primary prevention, management of ARF, and secondary prevention of recurrence.

Rheumatic Fever - general measures

• Prevention: Appropriate treatment of culture-proven GABHS infections may prevent the development of ARF.
• Treatment of ARF:
  • Antibiotics: Full course of penicillin or equivalent to eradicate active infection; does not alter course of carditis
  • Anti-inflammatory: High dose aspirin is standard; steroids may help for severe carditis but remains controversial.
  • Cardiac support: Aggressive support of cardiac function and use of systemic afterload reduction for severe disease
  • Surgical valvuloplasty or valve replacement: May be necessary in severe cases.
  • Bed rest: Controversial; still recommended for severe cases
• Secondary prevention:
  • Ideally administered as benzathine penicillin G as a monthly IM injection, but oral daily penicillin or erythromycin is acceptable in areas of low prevalence.
  • Duration based on clinical presentation and degree of cardiac involvement:
    • ARF without cardiac involvement: 5 years or until age 18, whichever is longer
    • ARF with mild or resolved carditis: 10 years or until age 25, whichever is longer
    • ARF with severe carditis or cardiac surgery: Lifelong
• Treatment of chorea:
  • Usually supportive
  • Phenobarbital and haloperidol are most commonly used; chlorpromazine, diazepam, or valproic acid also used

Rheumatic Fever - medication

ANTI-INFLAMMATORY:

Rheumatic Fever - first line

Aspirin 60–100 mg/kg/d; may be reduced when fever and acute phase reactants have normalized for 6–8 weeks

Rheumatic Fever - second line

Prednisone 2 mg/kg/d for 2 weeks, then taper

ANTIBIOTICS IN ARF:

Rheumatic Fever - first lines

Penicillin V Potassium (Pen VK):

• Children: 250 mg 2–3 times/day for 10 days
• Adolescents, adults: 500 mg 2–3 times/day for 10 days

Rheumatic Fever - second lines

Erythromycin, amoxicillin, 1st generation cephalosporin

SECONDARY PROPHYLAXIS:

Rheumatic Fever - first liness

Benzathine penicillin G IM (600,000 U for weight <27 kg or 1,200,000 U for weight >27 kg) every 3–4 weeks

Rheumatic Fever - second liness

• Penicillin V 250 mg b.i.d
• Erythromycin, sulfadiazine

Rheumatic Fever - FOLLOW UP

• Patients without carditis:
  • Close follow-up is needed for 2–3 weeks to assess patient’s condition for development of acute carditis.
  • Long-term pediatric follow-up is needed to diagnose patients with indolent carditis.
  • Long-term follow-up is needed to evaluate patients who develop chorea.
  • Prophylaxis should be stressed even in patients without carditis.
• Patients with carditis:
  • Cardiology follow-up is needed to assess development or evolution of RHD.
  • Symptoms of worsening heart failure suggest progression of valvular or myocardial disease, recurrent ARF, or endocarditis.
  • Secondary prophylaxis and bacterial endocarditis prophylaxis should be stressed.

Rheumatic Fever - disposition

Rheumatic Fever - issues for referral

Patients with new murmurs or clinical evidence of heart failure should be referred to a cardiologist.

Rheumatic Fever - prognosis

• ARF recurrence rate as high as 36% without prophylaxis
• Chorea may last weeks to months and has a similarly high recurrence rate.
• Carditis may resolve spontaneously (70–80%) or progress. Severity of the initial carditis is a major determinant of progression.

Rheumatic Fever - complications

Long-term complications related to evolution of RHD:

• Mitral stenosis
• Mitral regurgitation
• Aortic stenosis
• Aortic regurgitation
• Chronic heart failure

Rheumatic Fever - bibliography

1. Carapetis JR, McDonald M, Wilson NJ. Acute rheumatic fever. Lancet. 2005;366:155–168.
2. Cilliers AM, Manyemba J, Saloojee H. Cochrane review: Anti-inflammatory treatment for carditis in acute rheumatic fever. The Cochrane Library. 2006;4:1–37.
3. Dajani A, Taubert K, Ferrieri P, et al. Treatment of acute streptococcal pharyngitis and prevention of rheumatic fever. Pediatrics. 1995;96:758–764.
4. Jones TD. Diagnosis of rheumatic fever. JAMA. 1944;126:481–484.
5. Special Writing Group of the Committee on Rheumatic Fever, Endocarditis, and Kawasaki’s Disease of the Council on Cardiovascular Disease in the Young of the American Heart Association. Guidelines for the diagnosis of rheumatic fever, Jones criteria update. JAMA. 1992;268:2069–2073.
6. Stollerman GH. Rheumatic fever in the 21st century. Clin Infect Dis. 2001;33:806–814.

Rheumatic Fever - CODES

Rheumatic Fever - icd9

398.99 Other and unspecified rheumatic heart disease

Rheumatic Fever - FAQ

• Q: Does a negative throat culture rule out ARF?
• A: No. Throat cultures may be negative in 2/3 of patients.
• Q: Is there a vaccine available to prevent ARF?
• A: Not at present; however, research efforts to develop a recombinant multivalent vaccine have been promising. Note that >90 antigenic strains of group A Streptococcus have been identified; any vaccines developed ought to focus on those with the greatest virulence.
• Q: What genetic factors predispose to ARF?
• A: Several studies done worldwide have reported a high incidence of certain HLA-DR antigens in patients with rheumatic fever. The specific antigen/allele involved varies with the ethnic group studied.
• Q: Can ECG evidence of carditis alone be used to diagnose rheumatic fever?
• A: This is currently under debate. An ECG finding of carditis without a murmur cannot be used to fulfill the Jones criteria. However, many experts would agree to treat subclinical carditis as ARF, especially in areas of high prevalence.
• Q: Can intravenous gamma globulin be used as a treatment for ARF?
• A: One study revealed that intravenous gamma globulin did not alter the natural history of ARF, with no detectable difference in the cardiac outcome, laboratory findings, or ECG parameters when compared to placebo.

Book Source Details

• Book Title: The 5-Minute Pediatric Consult
• Author(s): M. William Schwartz MD; et al.
• Year of Publication: 2008
• Copyright Details: The 5-Minute Pediatric Consult, Copyright © 2008 Lippincott Williams & Wilkins.

More About Acute rheumatic fever

• Back to disease: Acute rheumatic fever: Introduction
• Next Book Extract About Acute rheumatic fever: Surveys relating to Acute rheumatic fever
• More Book Extracts: All Online Books for Acute rheumatic fever

More Medical Textbooks Online about Acute rheumatic fever

Review other book chapters online related to Acute rheumatic fever:

Copyright notice for book excerpts: Copyright © 2008 Lippincott Williams & Wilkins. All rights reserved.

More About This Book: Title: The 5-Minute Pediatric Consult Authors: M. William Schwartz MD; et al. Publisher: Lippincott Williams & Wilkins Copyright: 2008 ISBN: 0-7817-7577-9

 » Next page: Surveys relating to Acute rheumatic fever

Rate This Website

What do you think about the features of this website? Take our user survey and have your say:

Website User Survey

Medical Tools & Articles:

Next articles:

• Surveys relating to Acute rheumatic fever
• Ask Question about Acute rheumatic fever

Tools & Services:

• Bookmark this page
• Symptom Search
• Symptom Checker
• Medical Dictionary
• Give your feedback

Medical Articles:

• Disease & Treatments Search
• Misdiagnosis Center
• Full list of interesting articles

Forums & Message Boards

• Ask or answer a question at the Boards:
• I cannot get a diagnosis. Please help.
• Tell us your medical story.
• Share your misdiagnosis story.
• What is the best treatment for my condition?
• See all the Boards.

Enter your search terms Submit search form
Search The Web Search wrongdiagnosis.com

Common Health Mistakes

Symptom
Checker

Search Specialists by State and City