ACUTE

ACUTERHEUMATIC
RHEUMATICFEVER
FEVER
Modul 3.2
Anindita Soetadji, Galuh Hardaningsih
 Causes
1. Immunologic response, that occurs as
a delayed sequela of group A streptococcal
infection of the pharynx but not of the skin.
The attack rate 0.3% to 3%.
2. Important predisposing factors:
family history of rheumatic fever,
Low socioeconomic status (poverty, poor hygiene,
medical deprivation)
Age 6 -15 y (peak incidence at 8 y).
Grup A Streptococcus hemoliticus
 Pathology
The inflammatory lesion is found in :
heart, brain, joints, and skin.
Rheumatic carditis:
Pancarditis (myocarditis, endocarditis, pericarditis)
Valvular damage: mitral, aorta, tricuspid, pulmonal
valves
Aschoff bodies in the atrial myocardium are
believed to be characteristic of rheumatic fever
consist of inflammatory lesions
 Clinical manifestation: Jones Criteria, revised
Guidelines for the diagnosis of initial attack of rheumatic fever:

MAJOR
MAJORMANIFESTATIONS
MANIFESTATIONS MINOR
MINORMANIFESTATIONS
MANIFESTATIONS
Carditis Clinical Findings :
Carditis Clinical Findings :
Arthralgia
Polyarthritis Arthralgia
Polyarthritis Fever
Fever
Chorea Laboratory Findings
Chorea Laboratory Findings
Erythema marginatum Elevated acute-phase
Elevated acute-phase
Erythema marginatum reactants (erythrocyte
Subcutaneous nodule reactants (erythrocyte
Subcutaneous nodule sedimentation rate, C-
sedimentation rate, C-
reactive protein)
reactive protein)
ECG: Prolonged PR interval
ECG: Prolonged PR interval
SUPPORTING EVIDENCE OF ANTECEDENT GROUP A
SUPPORTING EVIDENCE OF ANTECEDENT GROUP A
STREPTOCOCCAL INFECTION
STREPTOCOCCAL INFECTION
Positive throat culture or rapid streptococcal antigen test
Positive throat culture or rapid streptococcal antigen test
Elevated or rising streptococcal antibody titer
Elevated or rising streptococcal antibody titer
 The presence of two major manifestations or
of one major and two minor
Supported by evidence of preceding group A
streptococcal infection manifestations

high probability of acute rheumatic fever

 HISTORY OF ILLNESS

1.1.History
Historyofofstreptococcal
streptococcalpharyngitis,
pharyngitis,11toto55
weeks
weeks(average,
(average,33weeks)
weeks)before
beforethetheonset
onsetofof
symptoms.
symptoms.The Thelatent
latentperiod
periodmay
maybe beasaslong
long
asas22toto66months
months(average,
(average,44months)
months)inincases
cases
ofofisolated chorea.
isolated chorea.
2.2.Pallor,
Pallor,malaise,
malaise,easy
easyfatigability,
fatigability,and
andother
other
history,
history,such
suchasasepistaxis
epistaxis(5%
(5%toto10%)
10%)andand
abdominal
abdominalpain,pain,may
maybe bepresent
present
 MAJOR MANIFESTATIONS
Arthritis
Arthritis
most common manifestation (70% of cases),
most common manifestation (70% of cases),
large joints (e.g., knees, ankles, elbows, wrists),
large joints (e.g., knees, ankles, elbows, wrists),
> 1 joint is involved,
> 1 joint is involved,
simultaneously or in succession,
simultaneously or in succession,
characteristic
characteristic
migratory
migratory
Swelling, heat, redness, severe pain, tenderness ,limitation of motion
Swelling, heat, redness, severe pain, tenderness ,limitation of motion
salicylate-containing analgesics, signs of inflammation may be mild.
salicylate-containing analgesics, signs of inflammation may be mild.
The arthritis responds dramatically to salicylate therapy;
The arthritis responds dramatically to salicylate therapy;
if patients treated with salicylates (with documented therapeutic levels) do
if patients treated with salicylates (with documented therapeutic levels) do
not improve in 48 hours, the diagnosis of acute rheumatic fever is probably
not improve in 48 hours, the diagnosis of acute rheumatic fever is probably
incorrect.
incorrect.
 Carditis
Carditis
Carditis
Carditisoccurs
occursinin50%
50%ofofpatients.
patients.
Signs
Signsofofcarditis
carditisinclude
includesome
someororallallofofthe
thefollowing.
following.
1. Tachycardia.
1. Tachycardia.
2. A heart murmur of mitral regurgitation (MR) or aortic
2. A heart murmur of mitral regurgitation (MR) or aortic
regurgitation (AR), or both.
regurgitation (AR), or both.
3. Pericarditis (friction rub, pericardial effusion, chest
3. Pericarditis (friction rub, pericardial effusion, chest
pain, and ECG changes)
pain, and ECG changes)
4. Cardiomegaly on chest x-ray films is indicative of the
4. Cardiomegaly on chest x-ray films is indicative of the
severity of rheumatic carditis (or valvulitis) or
severity of rheumatic carditis (or valvulitis) or
congestive heart failure (CHF).
congestive heart failure (CHF).
5. Signs of CHF (gallop rhythm, distant heart sounds,
5. Signs of CHF (gallop rhythm, distant heart sounds,
cardiomegaly) are indications of severe cardiac
cardiomegaly) are indications of severe cardiac
dysfunction.
dysfunction.
 Erythema
ErythemaMarginatum
Marginatum

<<10% of patients
10% of patients
nonpruritic
nonpruriticserpiginous
serpiginousororannular
annular
erythematous
erythematousrashesrashes
trunk and the inner proximal portions of
trunk and the inner proximal portions of the the
extremities;
extremities;never
neverseen
seenononthe
theface.
face.
disappearing
disappearingon onexposure
exposuretotocold
coldand
and
reappearing
reappearingafter
afteraahot
hotshower
showerororwhen
whenthethe
patient
patientisiscovered
coveredwith
withaawarm
warmblanket.
blanket.
 Subcutaneous Nodules
2% to 10% of patients
particularly in cases with recurrences; they are almost
never present as a sole manifestation of rheumatic
fever.
hard, painless, nonpruritic, freely movable, swelling,
and 0.2 to 2 cm in diameter.
symmetrically, singly or in clusters, on the extensor
surfaces of both large and small joints, over the scalp,
or along the spine.
have a significant association with carditis.
 Sydenham's
Sydenham'sChorea
Chorea
may
maybe
berelated
relatedtotodysfunction
dysfunctionofofbasal
basalganglia
ganglia
and
andcortical
corticalneuronal
neuronalcomponents
components
Subcutaneous Nodules
Erythema Marginatum
Erythema marginatum
Prolonged PR interval
Erythrocyte sedimentation
Revised Jones criteria
 Differential Diagnosis
1. Juvenile rheumatoid
2. Other collagen vascular diseases (systemic lupus
erythematosus, mixed connective tissue disease);
3. Virus-associated acute arthritis (rubella,
parvovirus, hepatitis B virus, herpesviruses,
enteroviruses) is much more common in adults
4. Hematologic disorders, such as sicklemia and
leukemia, should be considered in the differential
diagnosis
 Clinical Course
Only carditis can cause permanent cardiac damage.
Only carditis can cause permanent cardiac damage.
Signs of mild carditis disappear in weeks, severe
Signs of mild carditis disappear in weeks, severe
carditis
carditismay
maylast
lastfor
for22toto66months.
months.
Arthritis subsides within a few days to several
Arthritis subsides within a few days to several
weeks,
weeks,even
evenwithout
withouttreatment
treatmentand
anddoes
doesnot
notcause
cause
permanent
permanentdamage.
damage.
Chorea gradually subsides in 6 to 7 months or longer
Chorea gradually subsides in 6 to 7 months or longer
and
andusually
usuallydoes
doesnotnotcause
causepermanent
permanentneurologic
neurologic
sequela
sequela
 Management
1. When acute rheumatic fever is suggested by history and
physical examination, obtain the laboratory studies:
complete blood count,
acute phase reactants (erythrocyte sedimentation rate and C-
reactive protein),
throat culture,
ASO titer (and a second antibody titer, particularly with chorea),
chest x-ray films, and
ECG.
Cardiology consultation is indicated to clarify whether
there is cardiac involvement echocardiography.
 Streptococci eradication
1.1. Benzathine penicillin G, 0.6 to 1.2 million
Benzathine penicillin G, 0.6 to 1.2 million
units
unitsintramuscularly,
intramuscularly,isisgiven
giventotoeradicate
eradicate
streptococci.
streptococci.
This
Thisserves
servesasasthe
thefirst
firstdose
doseofofpenicillin
penicillin
prophylaxis
prophylaxisasaswell
well(see
(seelater
laterdiscussion).
discussion).
2.2. InInpatients
patientsallergic
allergictotopenicillin,
penicillin,
1.1. erythromycin,
erythromycin,40
40mg/kg
mg/kgper
perday
dayinintwo
twototofour
four
doses for 10 days,
doses for 10 days,
 Anti-inflammatory or suppressive therapy with
salicylates or steroids must not be started until
a definite diagnosis is made
When the diagnosis of acute rheumatic fever is
confirmed, one must educate the patient and
parents about the need to prevent subsequent
streptococcal infection through continuous
antibiotic prophylaxis.
In patients with cardiac involvement, the
need for prophylaxis against infective
endocarditis should also be emphasized.
 Bed rest of varying duration is recommended.
The duration depends on the type and severity of the
manifestations and may range from a week (for isolated
arthritis) to several weeks for severe carditis.
Bed rest is followed by a period of indoor ambulation of
varying duration before the child is allowed to return to
school.
The erythrocyte sedimentation rate is a helpful guide to
the rheumatic activity and therefore to the duration of
restriction of activities.
Full activity is allowed when the erythrocyte
sedimentation rate has returned to normal, except in
children with significant cardiac involvement.
Therapy with anti-inflammatory agents should be started as soon
as acute rheumatic fever has been diagnosed.
1. Mild to moderate carditis, aspirin alone is recommended in
a dose of 90 to 100 mg/kg per day in four to six divided
doses.
1. This dose is continued for 4 to 8 weeks, depending on the
clinical response. After improvement, the therapy is
withdrawn gradually over 4 to 6 weeks while monitoring
acute phase reactants.
2. Arthritis, aspirin therapy is continued for 2 weeks and
gradually withdrawn over the following 2 to 3 weeks.
1. Rapid resolution of joint symptoms with aspirin within 24 to 36
hours is supportive evidence of the arthritis of acute rheumatic fever.
3. Prednisone (2 mg/kg per day in four divided doses for 2 to 6
weeks) is indicated only in cases of severe carditis
Treatment of CHF
Complete bed rest with orthopneic position
and moist, cool oxygen
Prednisone for severe carditis of recent onset
Digoxin, used with caution, beginning with
half the usual recommended dose, because
certain patients with rheumatic carditis are
supersensitive to digitalis
Furosemide, 1 mg/kg every 6 to 12 hours, if
indicated
 Prognosis
The presence or absence of permanent cardiac damage determines the
prognosis.
The development of residual heart disease is influenced by the
following three factors:
1. Cardiac status at the start of treatment: The more severe the
cardiac involvement at the time the patient is first seen, the greater
the incidence of residual heart disease.
2. Recurrence of rheumatic fever: The severity of valvular
involvement increases with each recurrence.
3. Regression of heart disease: Evidence of cardiac involvement at the
first attack may disappear in 10% to 25% of patients 10 years after
the initial attack.
4. Valvular disease resolves more frequently when prophylaxis is
followed
 Prevention
Primary prevention
Secondary prevention
 PRIMARY PREVENTION
Primary prevention of rheumatic fever is possible
with a 10-day course of penicillin therapy for
streptococcal pharyngitis.
However, primary prevention is not possible in all
patients because about 30% of the patients develop
subclinical pharyngitis and therefore do not seek
medical treatment.
Another 30% of patients develop acute rheumatic
fever without symptoms of streptococcal pharyngitis
(30%).
 SECONDARY PREVENTION

Who should receive prophylaxis?

Patients with documented histories of
rheumatic fever, including those with isolated
chorea and those without evidence of
rheumatic heart disease, must receive
prophylaxis.
For how long?
For patients who had acute rheumatic fever without
carditis, the prophylaxis should continue for at least 5 years
or until the person is 21 years of age., whichever is longer.
For patients who are in a high-risk occupation (e.g.,
schoolteachers, physicians, nurses), prophylaxis should be
continued for a longer period of time.
The chance of recurrence is highest in the first 5 years after
the acute rheumatic fever.
If the patient had rheumatic carditis or residual valvular
disease as a result of rheumatic fever, the duration of
prophylaxis should be longer
What method of prophylaxis should be
used?

The method of choice for secondary prevention is benzathine

penicillin G, 1.2 million units given intramuscularly every 28
days (not once a month).

Alternative methods, although not as effective, are the

following:
1. Oral penicillin V, 250 mg, twice daily
2. Oral sulfadiazine 1 g or sulfisoxazole 0.5 g once daily
3. Oral erythromycin ethyl succinate, 250 mg, twice daily
Prophylaxis for Rheumatic Fever
Carditis
Valvular lesion