RHEUMATIC FEVER

AND
RHEUMATIC HEART DISEASE

Departemen Kardiologi dan Kedokteran Vaskular

FK USU

Introduction
Rheumatic fever is an immunologically mediated
inflammatory disease, that occurs as a delayed
sequel to group A streptococcal throat infection,
in genetically susceptible individuals.
Rheumatic heart disease is the most serious
complication of rheumatic fever
Acute rheumatic fever and rheumatic heart
disease are thought to result from an
autoimmune response, but the exact
pathogenesis remains unclear
Fuster V, et al. Acute Rheumatic Fever in Hurst the Heart 10th ed. New York: Mc GrawHill. 2001.

Causes
Rheumatic fever is caused by a group A streptococcal. It
occurs 2-3 weeks later after pharyngeal infection in a
small percentage of children aged 5-15 years. It is an
antibody-mediated autoimmune response and occurs
where antibodies directed against bacterial cell
membrane antigens cross-react and cause multiorgan
disease.

Fuster V, et al. Acute Rheumatic Fever in Hurst the Heart 10th ed. New York: Mc GrawHill. 2001.

Pathogenesis

The pathogenic mechanisms

Etiopathogenesis
: involved in the development of RF

remain unclear. But it is evident that an abnormal humoral and

cellular immune response occurs.
Antigenic mimicry between streptococcal antigens, mainly Mprotein
epitopes and human tissues, such as heart valves, myosin and
tropomyosin, brain proteins, synovial tissue and cartilage.
Molecular mimicry was first demonstrated by humoral immune
response.
Streptococcal antibodies cross-react with several human tissues
including
heart, skin, brain, glomerular basement membrane, striated and
smooth
muscles.

Jones Criteria (2003 Revision) for Diagnosis of Acute

Rheumatic Fever

Major manifestations

1.
2.
3.
4.
5.

Carditis
Polyarthritis
Chorea
Erythema marginatum
Subcutaneous nodules

. Minor manifestations
1.
2.
3.
4.
5.

Fever
Arthralgias
Previous rheumatic fever or rheumatic heart disease
Increased C- reactive protein concentrations or ESR
Prolonged PR interval on ECG

. Evidence of antecedent group A streptococcal

infection
1.
2.

Positive throat culture or rapid antigen test positive for

group A streptococcus
Increased or increasing streptococcal antibody titer
Report of a WHO Expert Consultation Geneva. Geneva 2004.

Jones Criteria (2003 Revision) for Diagnosis of

Acute Rheumatic Fever
A firm diagnosis requires
1) 2major manifestations or 1 major and 2 minor
manifestations
and
2 ) Evidence of a recent streptococcal infection.
Blood test reveal rising antistreptolysin O
(ASTO) titres when taken 2 weeks apart. The
throat swab may be positive.

Report of a WHO Expert Consultation Geneva. Geneva 2004.

Carditis

Miocarditis : resting tachycardia,

muffled or soft first heart sound, gallop
rhytm, cardiomegaly, or congestive
heart failure.
Endocarditis - valvulitis : changing
murmur (new apical systolic murmur of
mitral regurgitation and/or a basal
diastolic murmur of aortic regurgitation
Pericarditis : chest pain, friction rub,
pericardial effusion

Polyarthritis
Arthritis is the most common manifestation,
present in 60-80% of patients.
It usually affects the peripheral large joints;
small joints and axial skeleton are rarely
involved.
Knees, ankles, elbows and wrists are the
most
frequently affected. The joints are red, warm
and swollen.
Arthritis is characteristically asymmetrical,
migratory, and very painful, although some
patients may present mild joint complaints. It
usually resolves spontaneously at the most in
2 or 3 weeks.

Sydenham Chorea
Sydenhams chorea is
characterized by involuntary
movements, specially of the face
and limbs, muscle weakness,
disturbances of speech and gait.

Erythema marginatum
This is an evanescent, erythematous, non-pruritic rash with
pale centers and rounded or serpiginous margins. Lesions
occur mainly on the trunk and proximal extremities and
may be induced by application of heat

MANAGEMENT OF RHEUMATIC
FEVER/RHEUMATIC HEART DISEASE
PRIMARY PREVENTION
adequate antibiotic therapy of group A Streptococcal
Upper Respiratory Tract infections to prevent an initial
attack of acute rheumatic fever

SECONDARY PREVENTION
Continues administration of specific antibiotics to
patients with a previous attack of Rheumatic Fever

1.
2.
3.

Report of a WHO Expert Consultation Geneva. Geneva 2004.

Fuster V, Alexander RW, ORourke RA. Acute Rheumatic Fever in Hurst the Heart 10th ed. 2001.
Calleja HB, Guzman SV,eds. Rheumatic Fever And Rheumatic Heart Disease. Manila. 2001

Managements
Treatment with high-dose benzatine benzyl
penicillin is started immediately to eradicate the
causative organism.
Anti-inflammatory agents are given to suppress
the autoimmune response. Salicylates are
effective. Corticosteroids are used if there is any
carditis.
Long-term follow-up is required and any patients
who have resulting valve damage need
prophylactic antibiotics to prevent infective
endocarditis.

Antibiotic Prophylaxis
General WHO guidelines recommend that if no
carditis has developed, prophylaxis should be
continued for 5 years after an acute attack or
until the age of 18 years (whichever is longer).
In cases of mild or healed carditis, treatment
should continue for 10 years past the last attack
or until age 25 years (whichever is longer). In
cases of more severe carditis or valve surgery,
treatment should be lifelong.