RHEUMATIC FEVER

EPIDEMIOLOGY
The epidemiology of acute rheumatic fever
is identical to that of group A streptococcal
upper respiratory tract infections.
Acute rheumatic fever most often occurs
in children; the peak age-related incidence
is between 5 and 15 years.
Most initial attacks in adults take place at
the end of the second and beginning of
the third decades of life.
Rarely, initial attacks occur as late as the
fourth decade and recurrent attacks have
been documented as late as the fifth

The epidemiology of rheumatic fever is

also influenced by the serotypes of group
A streptococci present in a population.
The concept of "rheumatogenecity" of
specific strains is largely based upon
epidemiologic evidence associating
certain serotypes with rheumatic fever
(e.g., serotypes 1, 3, 5, 6, 18, etc.).
Mucoid isolates are frequently associated
with virulence and with rheumatic fever.

PATHOGENESIS
Historically, approaches to understanding
the pathogenesis of rheumatic fever have
been grouped into three major categories:
(1) direct infection by the group A
streptococcus;
(2) a toxic effect of streptococcal
extracellular products on the host tissues;
(3) an abnormal or dysfunctional immune
response to one or more as yet
unidentified somatic or extracellular
antigens produced by all (or perhaps only
by some) group A streptococci.

The hypothesis of "antigenic mimicry"

between human and group A streptococcal
antigens has been studied extensively and
has concentrated on two interactions.
The first is the similarity between the groupspecific carbohydrate of the group A
streptococcus and the glycoprotein of heart
valves;
The second involves the molecular similarity
among the streptococcal cell membrane,
streptococcal M protein sarcolemma, and
other moieties of the human myocardial cell.

The clinical forms of

rheumatic fever.
1. Acute rheumatic fever
2. Reccurent rheumatic fever
3. Chronic rheumatic heart

disease

Outcomes:
1. Recovering
2. Chronic rheumatic heart

disease
with valve lesion
without valve lesion (fibrosis
of the valves border)

DIAGNOSIS
There is no specific laboratory
test that can establish a diagnosis
of rheumatic fever.
The diagnosis, therefore, is a
clinical one but requires
supporting evidence from the
clinical microbiology and clinical
immunology laboratories.

In 1944 Jones first proposed

criteria to assist the clinician in
standardizing the diagnosis of
rheumatic fever.
The most recent modification of
the Jones criteria (Updated Jones
Criteria) was published in 1992 by
a Special Writing Group of the
American Heart Association.

Table 1. The Jones Criteria for

Rheumatic fever, Updated 1992.
Major Criteria

Minor Criteria

Carditis
Migratory polyarthritis
Sydenhams chorea
Subcutaneous nodules
Erythema marginatum

Clinical
Fever
Arthralgia
Laboratory
Elevated acute
phase reactants
Prolonged PR interval

Plus
Supporting evidence of a recent group A streptococcal
infection
(e.g., positive throat culture or rapid antigen detection
test; and/
or elevated or increasing streptococcal antibody test)

1. Carditis

The carditis of acute rheumatic

fever is a pancarditis involving the:
pericardium
myocardium
endocardium

Carditis is characterized by
one or more of the following:
sinus tachycardia,
the murmur of mitral
regurgitation,
an S3 gallop, a pericardial
friction rub,
cardiomegaly.

rheumatic valvulitis

fibrous thickening and

adhesion

valvular stenosis and/or

regurgitation.

The mitral valve is involved most

frequently, followed by the aortic valve.
However, isolated aortic valve disease
as a consequence of acute rheumatic
fever is quite rare.
In patients with aortic valve disease
due to rheumatic fever, the mitral valve
is almost always simultaneously
affected

2. Migratory polyarthritis
Is present in as many as 75% of
cases, most often affecting the:
Ankles
Wrists
Knees
Elbows

It usually does not affect the small

joints of the hands or feet and
seldom involves the hip joints.
The arthritis of acute rheumatic
fever is extremely painful.
The difference between arthralgia
(subjective joint pain) and arthritis
(joint pain and swelling) must be
understood.

3. Sydenham's chorea.
Occurs in fewer than 10% of patients
with rheumatic fever.
The latent period may be as long as
several months.
Patients with Sydenham's chorea should
be given secondary prophylaxis for
prevention of recurrent attacks, even if
they do not appear to have rheumatic
heart disease.

4. Subcutaneous nodules and

erythema marginatum.
Are rare major manifestations, usually
present in fewer than 10% of cases.
Subcutaneous nodules are found over
extensor surfaces of joints (in patients with
long-standing rheumatic heart disease)
Erythema marginatum - is an evanescent
macular eruption with rounded
bordersusually concentrated on the trunk.

The minor criteria are

nonspecific and may be present
in many clinical conditions.
To fulfill the Jones criteria, either
two major criteria, or one major
criterion and two minor criteria,
plus evidence of an antecedent
streptococcal infection are
required.

The latter may be provided by:

recovery of the organism on culture
evidence of an immune response
to one of the commonly measured
group A streptococcal antibodies:
-anti-streptolysin O
-anti-deoxyribonuclease B
-anti-hyaluronidase

TREATMENT
There are two necessary
therapeutic approaches to patients
with acute rheumatic fever:
anti-streptococcal antibiotic
therapy
therapy for the clinical
manifestations of the disease.

Antibiotic treatment should be

started immediately:
oral penicillin V (500 mg twice daily), 10day course.
erythromycin (250 mg four times daily) for
those with penicillin allergy, 10-day
course.
intramuscular benzathine penicillin G (a
single intramuscular injection of 1.2
million units)

Secondary prophylaxis
Recommendations of the American
Heart Association and of the World
Health Organization:
intramuscular injection of 1.2 million
units of benzathine penicillin G every 4
weeks, or
oral penicillin V (250 mg twice daily), or
oral sulfadiazine (1.0 g daily).

Secondary prophylaxis is
always given during the first 5
years after the attack
Medical therapy for the
manifestations of rheumatic
fever depends on the clinical
status of the patient.

Salicylates
In doses escalating to 2 g four times
daily are very effective and will result
in marked clinical improvement, often
within 12 h.
Salicylates may be given for 4 to 6
weeks and gradually tapered so as to
prevent a rebound.

The erythrocyte sedimentation rate is one

method for determining the rate of taper for
salicylates. Usually this requires at least 2
weeks.
There are no conclusive data to support
using nonsteroidal anti-inflammatory drugs
for acute rheumatic fever.
There is no indication for the use of
steroids (usually prednisone) solely for the
treatment of the arthritis of rheumatic fever.

In the past, patients with acute

rheumatic fever were kept at complete
bed rest for months.
This is inappropriate unless there is a
specific reason such as persistent
active carditis or severe heart failure.
Patients with arthritis will begin to feel
better very soon after anti-inflammatory
therapy with salicylates is begun.