What is Rheumatic Fever?

Rheumatic fever is an inflammatory disease that can develop as a complication of

inadequately treated strep throat or scarlet fever.

• Rheumatic fever (RF) is a systemic illness that may occur following

group A beta-hemolytic streptococcal (GABHS) pharyngitis in
children.
• Studies in the 1950s during an epidemic on a military base
demonstrated 3% incidence of rheumatic fever in adults with
streptococcal pharyngitis not treated with antibiotics.

Pathophysiology
Rheumatic fever develops in children and adolescents following pharyngitis
with GABHS (ie, Streptococcus pyogenes).

Streptococcus pyogenes bacteria

(Pappenheim’s stain) | Centers for Disease Control and Prevention

• The organisms attach to the epithelial cells of the upper respiratory

tract and produce a battery of enzymes, which allows them to
damage and invade human tissues.
• After an incubation period of 2-4 days, the invading organisms elicit
an acute inflammatory response, with 3-5 days of sore throat, fever,
malaise, headache, and elevated leukocyte count.
 • In a small percentage of patients, infection leads to rheumatic fever
several weeks after a sore throat has resolved; only infections of the
pharynx have been shown to initiate or reactivate rheumatic fever.
• Direct contact with oral (PO) or respiratory secretions transmits the
organism, and crowding enhances transmission; patients remain
infected for weeks after symptomatic resolution of pharyngitis and
may serve as a reservoir for infecting others.
• Severe scarring of the valves develops during a period of months to
years after an episode of acute rheumatic fever, and recurrent
episodes may cause progressive damage to the valves.
• The mitral valve is affected most commonly and severely (65-70% of
patients); the aortic valve is affected second most commonly (25%).
Statistics and Incidences
Rheumatic fever is most common in 5- to 15-year-old children, though it can
develop in younger children and adults.

A typical tonsillar exudate on a culture positive

case of streptococcal pharyngitis.

• Worldwide, there are over 15 million cases of RHD, with 282,000 new
cases and 33,000 deaths from this disease each year.
 • Rheumatic fever occurs in equal numbers in males and females;
females with rheumatic fever fare worse than males and have a
slightly higher incidence of chorea.
• Rheumatic fever is principally a disease of childhood, with a median
age of 10 years; however, GABHS pharyngitis is uncommon in
children younger than 3 years, and acute rheumatic fever is
extremely rare in these younger children in industrialized countries.
Causes
Rheumatic fever is believed to result from an autoimmune response; however, the
exact pathogenesis remains unclear.

• GABHS infection. Rheumatic fever only develops in children and

adolescents following group A beta-hemolytic streptococcal
(GABHS) pharyngitis, and only infections of the pharynx initiate or
reactivate rheumatic fever.
• Streptococcal antigens.
• Decrease in regulatory T-cells. Decreased levels of regulatory T-
cells have also been associated with rheumatic heart disease and
with increased severity.

Clinical Manifestations
Revised in 1992 and again in 2016, the modified Jones criteria provide guidelines
for making the diagnosis of rheumatic fever; the modified Jones criteria for
recurrent rheumatic fever require the presence of 2 major, or 1 major and 2
minor, or 3 minor criteria for the diagnosis of rheumatic fever.

Major Diagnostic Criteria

• Carditis. Carditis in the child may be clinical and/or subclinical

(echo).
• Polyarthritis. Monoarthritis or polyarthralgia are adequate to
achieve major diagnostic criteria in Moderate/High-risk populations;
for polyarthralgia exclusion of other more likely causes is also
required.
 •Chorea. Jerky, uncontrollable body movements (Sydenham chorea,
or St. Vitus’ dance) — most often in the hands, feet, and face.
• Subcutaneous nodules. Small, painless bumps (nodules) beneath
the skin.
• Erythema marginatum. Flat or slightly raised, painless rash with a
ragged edge.
Minor Diagnostic Criteria

• Fever. Fever of ≥38.5°C ( ≥38°C to achieve a minor diagnostic

criteria in Moderate/High-risk populations.
• Polyarthralgia. Painful and tender joints — most often in the knees,
ankles, elbows, and wrists.
• Prolonged PR interval. Prolonged PR interval for age on
electrocardiography.
• Increased ESR. Elevated peak erythrocyte sedimentation rate during
acute illness ≥60 mm/h and/or C-reactive protein ≥3.0 mg/dl.
Assessment and Diagnostic Findings
Although there’s no single test for rheumatic fever, diagnosis is based on medical
history, physical exam and certain test results.

Extensive thickening of mitral valve, thickened

chordae tendineae, and hypertrophied left ventricular myocardium. | Public
Health Image Library (PHIL)

• Throat culture. Throat cultures for GABHS infections usually are

negative by the time symptoms of rheumatic fever or rheumatic
heart disease (RHD) appear; make attempts to isolate the organism
prior to the initiation of antibiotic therapy to help confirm a
diagnosis of streptococcal pharyngitis and to allow typing of the
organism if it is isolated successfully.
 • Rapid antigen detection test. This test allows rapid detection of
group A streptococci (GAS) antigen, allowing the diagnosis of
streptococcal pharyngitis to be made and antibiotic therapy to be
initiated while the patient is still in the physician’s office.
• Antistreptococcal antibodies. Clinical features of rheumatic fever
begin when antistreptococcal antibody levels are at their peak; thus,
these tests are useful for confirming previous GAS infection;
antistreptococcal antibodies are particularly useful in patients who
present with chorea as the only diagnostic criterion.
• Acute-phase reactants. C-reactive protein and erythrocyte
sedimentation rate are elevated in individuals with rheumatic fever
due to the inflammatory nature of the disease; both tests have high
sensitivity but low specificity for rheumatic fever.
• Heart reactive antibodies. Tropomyosin is elevated in persons with
acute rheumatic fever.
• Rapid detection test for D8/17. This immunofluorescence
technique for identifying the B-cell marker D8/17 is positive in 90%
of patients with rheumatic fever and may be useful for identifying
patients who are at risk of developing rheumatic fever.
• Chest radiography. Cardiomegaly, pulmonary congestion, and
other findings consistent with heart failure may be observed on
chest radiograph in individuals with rheumatic fever.
• Echocardiography. In individuals with acute RHD, echocardiography
identified and quantitated valve insufficiency and ventricular
dysfunction.
Medical Management
Therapy is directed towards eliminating the GABHS pharyngitis (if still present),
suppressing inflammation from the autoimmune response, and providing
supportive treatment of congestive heart failure (CHF).

• Anti-inflammatory. Treatment of the acute inflammatory

manifestations of acute rheumatic fever consists of salicylates and
steroids; aspirin in anti-inflammatory doses effectively reduces all
manifestations of the disease except chorea, and the response
typically is dramatic.
 • Corticosteroids. If moderate to severe carditis is present as
indicated by cardiomegaly, third-degree heart block, or CHF, add PO
prednisone to salicylate therapy.
• Anticonvulsant medications. For severe involuntary movements
caused by Sydenham chorea, your doctor might prescribe an
anticonvulsant, such as valproic acid (Depakene) or carbamazepine
(Carbatrol, Tegretol, others).
• Antibiotics. Your child’s doctor will prescribe penicillin or another
antibiotic to eliminate remaining strep bacteria.
• Surgical care. When heart failure persists or worsens after
aggressive medical therapy for acute RHD, surgery to decrease valve
insufficiency may be lifesaving; approximately 40% of patients with
acute rheumatic fever subsequently develop mitral stenosis as adults.
• Diet. Advise nutritious diet without restrictions except in patients
with CHF, who should follow a fluid-restricted and sodium-restricted
diet; potassium supplementation may be necessary because of the
mineralocorticoid effect of corticosteroid and the diuretics if used.
• Activity. Initially, place patients on bed rest, followed by a period of
indoor activity before they are permitted to return to school; do not
allow full activity until the APRs have returned to normal; patients
with chorea may require a wheelchair and should be on homebound
instruction until the abnormal movements resolve.
Pharmacologic Management

Treatment and prevention of group A streptococci pharyngitis outlined here are

based on the current recommendations of the American Heart Association
Practice Guidelines on Prevention of Rheumatic Fever and Diagnosis and
Treatment of Acute Streptococcal Pharyngitis.

• Antibiotics. The roles for antibiotics are to (1) initially treat GABHS
pharyngitis, (2) prevent recurrent streptococcal pharyngitis,
rheumatic fever (RF), and rheumatic heart disease (RHD), and (3)
provide prophylaxis against bacterial endocarditis.
• Anti-inflammatory agents. Manifestations of acute rheumatic fever
(including carditis) typically respond rapidly to therapy with anti-
inflammatory agents; aspirin, in anti-inflammatory doses, is DOC;
 prednisone is added when evidence of worsening carditis and heart
failure is noted.
• Therapy for congestive heart failure. Heart failure in RHD probably
is related in part to the severe insufficiency of the mitral and aortic
valves and in part to pancarditis; therapy traditionally has consisted
of an inotropic agent (digitalis) in combination with diuretics
(furosemide, spironolactone) and afterload reduction (captopril).
Nursing Management
Nursing care of a child with rheumatic fever include:

Nursing Assessment

Nursing assessment for a child with rheumatic fever are as follows:

• History. Obtain a complete up-to-date history from the child and

the caregiver; ask about a recent sore throat or upper respiratory
infection; find out when the symptoms began, the extent of the
illness, and what if any treatment was obtained.
• Physical exam. Begin with a careful review of all systems, and note
the child’s physical condition; observe for any signs that may be
classified as major or minor manifestations; in the physical exam,
observe for elevated temperature and pulse, and carefully examine
for erythema marginatum, subcutaneous nodules, swollen or painful
joints, or signs of chorea.
Nursing Diagnoses

Based on the assessment data, the major nursing diagnoses are:

• Acute pain related to joint pain when extremities are touched or

moved.
• Deficient diversional activity related to prescribed bed rest.
• Activity intolerance related to carditis or arthralgia.
• Risk for injury related to chorea.
• Risk for noncompliance with prophylactic drug therapy related to
financial or emotional burden of lifelong therapy.
 • Deficient knowledge of caregiver related to the condition, need for
long-term therapy, and risk factors.
Nursing Care Planning and Goals

The major nursing care planning goals for rheumatic fever are:

• Reducing pain.
• Providing diversional activities and sensory stimulation.
• Conserving energy.
• Preventing injury.
Nursing Interventions

Nursing interventions for a child with rheumatic fever include:

• Provide comfort and reduce pain. Position the child to reduce joint
pain; warm baths and gentle range-of-motion exercises help to
alleviate some of the joint discomforts; use pain indicator scales with
children so they are able to express the level of their pain.
• Provide diversional activities and sensory stimulation. For those
who do not feel very ill, bed rest can cause distress or resentment; be
creative in finding diversional activities that allow bed rest but
prevent restlessness and boredom, such as a good book; quiet
games can provide some entertainment, and plan all activities with
the child’s developmental stage in mind.
• Promote energy conservation. Provide rest periods between
activities to help pace the child’s energies and provide for maximum
comfort; if the child has chorea, inform visitors that the child cannot
control these movements, which are as upsetting to the child as they
are to others.
• Prevent injury. Protect the child from injury by keeping the side rails
up and padding them; do not leave a child with chorea unattended
in a wheelchair, and use all appropriate safety measures.
Evaluation

Goals are met as evidenced by

• Reducing pain.
• Providing diversional activities and sensory stimulation.
• Conserving energy.
• Preventing injury.