Rheumatic Fever 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.
Rheumatic fever is an inflammatory disease that can
develop as a complication of inadequately treated strep • Direct contact with oral (PO) or respiratory secretions
throat or scarlet fever. transmits the organism, and crowding enhances
transmission; patients remain infected for weeks after
What is Rheumatic Fever? symptomatic resolution of pharyngitis and may serve
as a reservoir for infecting others.

Rheumatic fever is an inflammatory disease that can • Severe scarring of the valves develops during a period
develop as a complication of inadequately treated strep of months to years after an episode of acute
throat or scarlet fever. rheumatic fever, and recurrent episodes may cause
progressive damage to the valves.
• Rheumatic fever (RF) is a systemic illness that may
occur following group A beta-hemolytic streptococcal • The mitral valve is affected most commonly and
(GABHS) pharyngitis in children. severely (65-70% of patients); the aortic valve is
affected second most commonly (25%).
• Studies in the 1950s during an epidemic on a military
base demonstrated 3% incidence of rheumatic fever in Statistics and Incidences
adults with streptococcal pharyngitis not treated
with antibiotics. Rheumatic fever is most common in 5- to 15-year-old
children, though it can develop in younger children and
• Strep throat and scarlet fever are caused by adults.
an infection with streptococcus bacteria.

Pathophysiology

Rheumatic fever develops in children and adolescents

following pharyngitis with GABHS (ie, Streptococcus
pyogenes).

A typical tonsillar exudate on a culture positive case of

streptococcal pharyngitis.
• The prevalence of RHD in the United States was less • Streptococcal antigens. Streptococcal antigens,
than 0.05 per 1000 population, with only rare regional which are structurally similar to those in the heart,
outbreaks reported in Tennessee in the 1960s and in include hyaluronate in the bacterial capsule, cell wall
Utah, Ohio, and Pennsylvania in the 1980’s. polysaccharides (similar to glycoproteins in heart
valves), and membrane antigens that share epitopes
• However, a recent assessment of temporal trends of with the sarcolemma and smooth muscle.
patients diagnosed with acute rheumatic fever in the
United States from 2001-2011 showed that since 2001, • Decrease in regulatory T-cells. Decreased levels of
national acute rheumatic fever admissions has steadily regulatory T-cells have also been associated with
increased, with a peak in 2005, and decreased rheumatic heart disease and with increased severity.
thereafter.
Clinical Manifestations
• Worldwide, there are over 15 million cases of RHD,
with 282,000 new cases and 33,000 deaths from this
disease each year. Revised in 1992 and again in 2016, the modified Jones
criteria provide guidelines for making the diagnosis of
• RHD is the major cause of morbidity from rheumatic rheumatic fever; the modified Jones criteria for recurrent
fever and is the major cause of mitral insufficiency and rheumatic fever require the presence of 2 major, or 1 major
stenosis in the United States and the world. and 2 minor, or 3 minor criteria for the diagnosis of
rheumatic fever.
• Native Hawaiians and Maori (both of Polynesian
descent) have a higher incidence of rheumatic fever; Major Diagnostic Criteria
incidence of rheumatic fever in these patients is 13.4
per 100,000 hospitalized children per year, even with
antibiotic prophylaxis of streptococcal pharyngitis. • Carditis. Carditis in the child may be clinical and/or
subclinical (echo).
• Rheumatic fever occurs in equal numbers in males and
females; females with rheumatic fever fare worse than • Polyarthritis. Monoarthritis or polyarthralgia are
males and have a slightly higher incidence of chorea. adequate to achieve major diagnostic criteria in
Moderate/High-risk populations; for polyarthralgia
• Rheumatic fever is principally a disease of childhood, exclusion of other more likely causes is also required.
with a median age of 10 years; however, GABHS
pharyngitis is uncommon in children younger than 3 • Chorea. Jerky, uncontrollable body movements
years, and acute rheumatic fever is extremely rare in (Sydenham chorea, or St. Vitus’ dance) — most often
these younger children in industrialized countries. in the hands, feet, and face.

Causes • Subcutaneous nodules. Small, painless bumps

(nodules) beneath the skin.
Rheumatic fever is believed to result from an autoimmune
response; however, the exact pathogenesis remains • Erythema marginatum. Flat or slightly raised,
unclear. painless rash with a ragged edge.

Minor Diagnostic Criteria

• GABHS infection. Rheumatic fever only develops in
children and adolescents following group A beta-
hemolytic streptococcal (GABHS) pharyngitis, and only • Fever. Fever of ≥38.5°C ( ≥38°C to achieve a minor
infections of the pharynx initiate or reactivate diagnostic criteria in Moderate/High-risk populations.
rheumatic fever.
• Polyarthralgia. Painful and tender joints — most
• Molecular mimicry. So-called molecular mimicry often in the knees, ankles, elbows, and wrists.
between streptococcal and human proteins is felt to
involve both the B and T cells of peripheral blood, with • Prolonged PR interval. Prolonged PR interval for
infiltration of the heart by T cells; some believe that an age on electrocardiography.
increased production of inflammatory cytokines is the
final mechanism of the autoimmune reaction that
• Increased ESR. Elevated peak erythrocyte
causes damage to cardiac tissue in RHD.
sedimentation rate during acute illness ≥60 mm/h
and/or C-reactive protein ≥3.0 mg/dl.
 Assessment and Diagnostic Findings • Chest radiography. Cardiomegaly, pulmonary
congestion, and other findings consistent with heart
Although there’s no single test for rheumatic fever, failure may be observed on chest radiograph in
diagnosis is based on medical history, physical exam and individuals with rheumatic fever.
certain test results.
• 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

Extensive thickening of mitral valve, thickened chordae inflammatory manifestations of acute rheumatic fever
tendineae, and hypertrophied left ventricular myocardium. | consists of salicylates and steroids; aspirin in anti-
Public Health Image Library (PHIL) inflammatory doses effectively reduces all
• Throat culture. Throat cultures for GABHS infections manifestations of the disease except chorea, and the
usually are negative by the time symptoms of response typically is dramatic.
rheumatic fever or rheumatic heart disease (RHD)
appear; make attempts to isolate the organism prior • Corticosteroids. If moderate to severe carditis is
to the initiation of antibiotic therapy to help confirm a present as indicated by cardiomegaly, third-degree
diagnosis of streptococcal pharyngitis and to allow heart block, or CHF, add PO prednisone to salicylate
typing of the organism if it is isolated successfully. therapy.

• Rapid antigen detection test. This test allows rapid • Anticonvulsant medications. For severe involuntary
detection of group A streptococci (GAS) antigen, movements caused by Sydenham chorea, your doctor
allowing the diagnosis of streptococcal pharyngitis to might prescribe an anticonvulsant, such as valproic
be made and antibiotic therapy to be initiated while acid (Depakene) or carbamazepine (Carbatrol,
the patient is still in the physician’s office. Tegretol, others).

• Antistreptococcal antibodies. Clinical features of • Antibiotics. Your child’s doctor will prescribe
rheumatic fever begin when antistreptococcal penicillin or another antibiotic to eliminate remaining
antibody levels are at their peak; thus, these tests are strep bacteria.
useful for confirming previous GAS infection;
antistreptococcal antibodies are particularly useful in • Surgical care. When heart failure persists or worsens
patients who present with chorea as the only after aggressive medical therapy for acute
diagnostic criterion. RHD, surgery to decrease valve insufficiency may be
lifesaving; approximately 40% of patients with acute
• Acute-phase reactants. C-reactive protein and rheumatic fever subsequently develop mitral stenosis
erythrocyte sedimentation rate are elevated in as adults.
individuals with rheumatic fever due to the
inflammatory nature of the disease; both tests have • Diet. Advise nutritious diet without restrictions except
high sensitivity but low specificity for rheumatic fever. in patients with CHF, who should follow a fluid-
restricted and sodium-restricted
• Heart reactive antibodies. Tropomyosin is diet; potassium supplementation may be necessary
elevated in persons with acute rheumatic fever. because of the mineralocorticoid effect of
corticosteroid and the diuretics if used.
• Rapid detection test for D8/17. This
immunofluorescence technique for identifying the B- • Activity. Initially, place patients on bed rest, followed
cell marker D8/17 is positive in 90% of patients with by a period of indoor activity before they are
rheumatic fever and may be useful for identifying permitted to return to school; do not allow full activity
patients who are at risk of developing rheumatic fever. until the APRs have returned to normal; patients with
 chorea may require a wheelchair and should be on examine for erythema marginatum, subcutaneous
homebound instruction until the abnormal nodules, swollen or painful joints, or signs of chorea.
movements resolve.

Pharmacologic Management Nursing Diagnoses

Treatment and prevention of group A streptococci Based on the assessment data, the major nursing
pharyngitis outlined here are based on the current diagnoses are:
recommendations of the American Heart Association
Practice Guidelines on Prevention of Rheumatic Fever and
Diagnosis and Treatment of Acute Streptococcal • Acute pain related to joint pain when extremities are
Pharyngitis. touched or moved.

• Deficient diversional activity related to prescribed

• Antibiotics. The roles for antibiotics are to (1) initially
bed rest.
treat GABHS pharyngitis, (2) prevent recurrent
streptococcal pharyngitis, rheumatic fever (RF), and
rheumatic heart disease (RHD), and (3) provide • Activity intolerance related to carditis or arthralgia.
prophylaxis against bacterial endocarditis.
• Risk for injury related to chorea.
• Anti-inflammatory agents. Manifestations of acute
rheumatic fever (including carditis) typically respond • Risk for noncompliance with prophylactic drug
rapidly to therapy with anti-inflammatory agents; therapy related to financial or emotional burden of
aspirin, in anti-inflammatory doses, is DOC; prednisone lifelong therapy.
is added when evidence of worsening carditis and
heart failure is noted. • Deficient knowledge of caregiver related to the
condition, need for long-term therapy, and risk
• Therapy for congestive heart failure. Heart failure factors.
in RHD probably is related in part to the severe
insufficiency of the mitral and aortic valves and in part Nursing Care Planning and Goals
to pancarditis; therapy traditionally has consisted of an
inotropic agent (digitalis) in combination with diuretics Acute Rheumatic Fever Nursing Care Plans
(furosemide, spironolactone) and afterload reduction
(captopril).
The major nursing care planning goals for rheumatic fever
are:
Nursing Management

Nursing care of a child with rheumatic fever include: • Reducing pain.

• Providing diversional activities and
sensory stimulation.
Nursing Assessment • Conserving energy.
• Preventing injury.
Nursing assessment for a child with rheumatic fever are as
follows: Nursing Interventions

• History. Obtain a complete up-to-date history from Nursing interventions for a child with rheumatic fever
the child and the caregiver; ask about a recent sore include:
throat or upper respiratory infection; find out when
the symptoms began, the extent of the illness, and • Provide comfort and reduce pain. Position the
what if any treatment was obtained. child to reduce joint pain; warm baths and gentle
range-of-motion exercises help to alleviate some of
• Physical exam. Begin with a careful review of all the joint discomforts; use pain indicator scales with
systems, and note the child’s physical condition; children so they are able to express the level of their
observe for any signs that may be classified as major pain.
or minor manifestations; in the physical exam, observe
for elevated temperature and pulse, and carefully • 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.

Documentation Guidelines

Documentation in a child with rheumatic fever includes:

• Baseline and subsequent assessment findings to

include signs and symptoms.

• Individual cultural or religious restrictions and personal

preferences.

• Plan of care and persons involved.

• Teaching plan.

• Client’s responses to teachings, interventions, and

actions performed.

• Attainment or progress toward the desired outcome.

• Long-term needs, and who is responsible for actions

to be taken.