Rheumatic Fever

Also known as acute rheumatic fever (ARF), is an inflammatory

disease that can involve the heart, joints, skin, and brain. The disease typically
develops two to four weeks after a streptococcal throat infection. Signs and
symptoms include fever, multiple painful joints, involuntary muscle movements, and
occasionally a characteristic non-itchy rash known as erythema marginatum. The
heart is involved in about half of cases. Damage to the heart valves, known
as rheumatic heart disease (RHD), usually occurs after repeated attacks but can
sometimes occur after one. The damaged valves may result in heart failure, atrial
fibrillation and infection of the valves.

May occur following an infection of the throat by the bacterium Streptococcus

pyogenes. If the infection is untreated rheumatic fever can occur in up to three
percent of people. The underlying mechanism is believed to involve the production
of antibodies against a person's own tissues. Due to their genetics, some people are
more likely to get the disease when exposed to the bacteria than others. Other risk
factors include malnutrition and poverty. Diagnosis of RF is often based on the
presence of signs and symptoms in combination with evidence of a recent
streptococcal infection.

Rheumatic fever is most common in 5- to 15-year-old children, though it can

develop in younger children and adults.

Rheumatic fever can cause permanent damage to the heart, including damaged
heart valves and heart failure. Treatments can reduce damage from inflammation,
lessen pain and other symptoms, and prevent the recurrence of rheumatic fever.

Etiology:
Although the mechanism by which streptococcal organisms cause disease is not
entirely clear, overwhelming epidemiologic evidence suggests that ARF is caused by
streptococcal infection, and recurrences can be prevented with prophylaxis.
Strains of group A streptococci that are heavily encapsulated and rich in M protein
(signifying virulence in streptococcal strains) seem to be most likely to result in
infection.
Group A Streptococcus is thought to cause the myriad of clinical diseases in which
the host's immunologic response to bacterial antigens cross-react with various
target organs in the body, resulting in molecular mimicry. In fact, autoantibodies
reactive against the heart have been found in patients with rheumatic carditis. The
antibody can cross-react with brain and cardiac antigens, and immune complexes
are present in the serum. The problem has been the uncertainty of whether these
antibodies are the cause or result of myocardial tissue injury.

Pathophysiology:
Rheumatic fever is a systemic disease affecting the connective
tissue around arterioles, and can occur after an untreated strep throat infection,
specifically due to group A streptococcus (GAS), Streptococcus pyogenes. It is
believed to be caused by antibody cross-reactivity. This cross-reactivity is a type II
hypersensitivity reaction and is termed molecular mimicry. Usually, self reactive B
cells remain anergic in the periphery without T cell co-stimulation. During a
streptococcal infection, mature antigen-presenting cells such as B cells present the
bacterial antigen to CD4+T cells which differentiate into helper T2 cells. Helper
T2 cells subsequently activate the B cells to become plasma cells and induce the
production of antibodies against the cell wall of Streptococcus. However the
antibodies may also react against the myocardium and joints, producing the
symptoms of rheumatic fever.

S. pyogenes has a cell wall composed of branched polymers which

sometimes contain M protein that are highly antigenic. The antibodies which the
immune system generates against the M protein may cross-react with heart muscle
cell protein myosin, heart muscle glycogen and smooth muscle cells of arteries,
inducing cytokine release and tissue destruction. However, the only proven cross-
reaction is with perivascular connective tissue.This inflammation occurs through
direct attachment of complement and Fc receptor-mediated recruitment of
neutrophils and macrophages. Characteristic Aschoff bodies, composed of swollen
eosinophilic collagen surrounded by lymphocytes and macrophages can be seen on
light microscopy. The larger macrophages may become Anitschkow cells or Aschoff
giant cells. Rheumatic valvular lesions may also involve a cell-mediated
immunity reaction as these lesions predominantly contain T-helper cells
and macrophages.

In rheumatic fever, these lesions can be found in any layer of the heart
causing different types of carditis. The inflammation may cause a serofibrinous
pericardial exudate described as "bread-and-butter" pericarditis, which usually
resolves without sequelae. Involvement of the endocardium typically results in
fibrinoid necrosis and verrucae formation along the lines of closure of the left-sided
heart valves. Warty projections arise from the deposition, while subendocardial
lesions may induce irregular thickenings called MacCallum plaques.

Risk Factors:
The primary risk for rheumatic fever is a recent bout of strep throat. Other infections
with group A streptococci may also lead to rheumatic fever; one such condition is
called pyoderma (a skin infection). Age is also a risk factor. Rheumatic fever occurs
most commonly before the age of 35 and is most frequent in children.

S/Sx:
abdominal pain
fever
Heart problem may resulf of shortness of breath and Chest pain
Joint pain, Arthritis (knees, elbows, ankles and wrist)
Joint swelling; redness
nosebleed (epistaxis)
skin nodules (swelling)
Skin rash (erythma maginatum)
skin eruptions on trunks and upper part of the arms or legs
eruptions that look ring shapes or snake like
 Sydenham chorea (neurological disorders of childhood) emotional stability,
muscle weakness, uncoordinated jerkey movements that mainly affects the
face, feet and hands.

Medical Management:
Treatment strategies for acute rheumatic fever (ARF) can be divided into
management of the acute attack, management of the current infection, and
prevention of further infection and attacks.

The primary goal of treating an ARF attack is to eradicate streptococcal organisms

and bacterial antigens from the pharyngeal region. Penicillin is the drug of choice in
persons who are not at risk of allergic reaction. A single parenteral injection of
benzathine benzylpenicillin can ensure compliance. Oral cephalosporins, rather than
erythromycin, are recommended as an alternative in patients who are allergic to
penicillin. However, be cautious of the 20% cross-reactivity of the cephalosporins
with penicillin.

Prompt treatment of streptococcal pharyngitis in susceptible hosts can prevent

repetitive exposure to pathologically reactive antigens. However, management of
the current infection will probably not affect the course of the current attack.
Antimicrobial therapy does not alter the course, frequency, or severity of cardiac
involvement.

Analgesia is optimally achieved with high doses of salicylates, which often induce
dramatic clinical improvement. However, a lower dose may be required to avert
symptoms of nausea and vomiting. When salicylates are used as therapy, the
dosage should be increased until the drug produces either a clinical effect or
systemic toxicity characterized by tinnitus, headache, or hyperpnea.

Corticosteroids should be reserved for the treatment of severe carditis. After 2-3
weeks, the dosage may be tapered, reduced by 25% each week. Overlap with high-
dose salicylate therapy is recommended as the dosage of the prednisone is tapered
over a 2-week period to avoid poststeroid rebound. In extreme cases, intravenous
methylprednisolone may be used.

Mild heart failure usually responds to rest and corticosteroid therapy. Digoxin can be
useful in patients with severe carditis, but its use should be monitored closely
because of the possibility of heart block.

Surgical Care
Valve replacement should be considered in patients with active carditis, especially
those with cases that are refractory to medical care or require high doses of
vasodilators and diuretics.
Regurgitant lesions respond to valve replacement. Pure stenotic lesions may benefit
from more conservative balloon mitral commissurotomy.

Nursing Interventions
1. Monitor temperature frequently, and patients response to antipyretics.
2. Monitor the patients pulse frequently, especially after activity to determine
degree of cardiac compensation.
3. Auscultate the hear periodically for development of new heart murmur or
pericardial or pleural friction rub.
4. Observe for adverse effects of salicylate or nonsteroidal anti-inflammatory
drug (NSAID) therapy, such as stomach upset, tinnitus, headache, GI bleeding,
and altered mental status.
5. Monitor the patients response to long-term activity restriction.
6. Restrict sodium and fluids and obtain daily weights as indicated.
7. Administer medications punctually and at regular intervals to achieve
constant therapeutic blood levels.
8. Explain the need to rest (usually prescribed for 4 to 12 weeks, depending on
the severity of the disease and health care providers preference) and assure
the patient that bed rest will be imposed no longer than necessary.
9. Assist the patient to resume activity very gradually once asymptomatic at
rest and indicators of acute inflammation have become normal.
10. Provide comfort measures.
11. Provide safe, supportive environment for the child with chorea.
12. Observe for the disappearance or any major or minor manifestations of the
disease and report signs of increased rheumatic activity as salicylates or
steroids are being tampered.
13. Encourage continuous prophylactic antimicrobial therapy to prevent
recurrence.
 Philippine Rehabilitation Institute
Jose Abad Santos, San Matias
Guagua City Campus, Pampanga.

RHEUMATIC FEVER
Submitted by:
Navarro, Mara Samantha A. BSN.

Submitted To:
Sir. Roque, Myron. MN, RN, EMT-B.