Scientific Journal of Pediatrics

e-ISSN: 3025-6224

Scientific Journal of Pediatrics

(SJPed)
Journal website: https://phlox.or.id/index.php/sjped

Acute Rheumatic Fever and Rheumatic Heart Disease: Narrative Literature Review
Bagus Winandi Arundito1*, Teti Adriana Lubis1
1Department of Pediatrics, Pratama Warmare General Hospital, Manokwari, Indonesia

ARTICLE INFO AB STR AC T

Keywords: Rheumatic fever is a systemic inflammatory disease caused by an immune and

Children inflammatory response to infection by group A beta-hemolytic streptococci in
individuals with a genetic predisposition. In its acute form, rheumatic fever is a
Pathophysiology febrile illness that occurs about 2 to 3 weeks after infection and is characterized
Rheumatic fever by inflammation of the joints, skin, nervous system, and heart. If left untreated,
Rheumatic heart disease rheumatic fever can lead to scarring and structural deformities of the heart
resulting in rheumatic heart disease (RHD). This literature review aimed to
describe acute rheumatic fever and rheumatic heart disease in children.
*Corresponding author:
Therapy for acute rheumatic fever is aimed at eradicating streptococcal infection
Bagus Winandi Arundito regimen 10 days of antibiotics NSAIDs are used as agent anti-inflammatory for
rheumatic carditis and arthritis and help relieve symptoms, but does not
prevent complications. Serious carditis may require diuretics and vasodilators,
E-mail address: and recovery may take up to 12 months. Damaged valve repair surgery may be
bagusarundito@gmail.com necessary in cases of recurrent chronic rheumatic fever or carditis. In
conclusion, rheumatic fever is a systemic inflammatory disease caused by an
immune and inflammatory response to infection by group A beta-hemolytic
All authors have reviewed and approved streptococci in individuals with a genetic predisposition. If left untreated,
the final version of the manuscript. rheumatic fever can lead to scarring and structural deformities of the heart
resulting in rheumatic heart disease (RHD).
https://doi.org/10.59345.v1i1.15

improvements and changes in group A streptococcal

virulence. It occurs most frequently in children
between 5 and 15 years of age. Appropriate antibiotic
1. Introduction
therapy given within the first 9 days of infection
Rheumatic fever is a systemic inflammatory
usually prevents rheumatic fever. Individuals who
disease caused by an immune and inflammatory
have experienced one attack of acute rheumatic fever
response to infection by group A beta-hemolytic
are more susceptible to repeated attacks. 4-7 This
streptococci in individuals with a genetic
literature review aimed to describe acute rheumatic
predisposition. In its acute form, rheumatic fever is a
fever and rheumatic heart disease in children.
febrile illness that occurs about 2 to 3 weeks after
infection and is characterized by inflammation of the
Pathophysiology
joints, skin, nervous system, and heart. If left
Acute rheumatic fever can develop only as a sequel
untreated, rheumatic fever can lead to scarring and
to pharyngeal infection by group A beta-hemolytic
structural deformities of the heart resulting in
streptococci. Streptococcal skin infections do not
rheumatic heart disease (RHD). 1-3
develop into acute rheumatic fever because the strains
The incidence of acute rheumatic fever declined in
of microorganisms that infect the skin do not have the
the United States during the 1960s, 1970s, and early
same antigenic molecules in their cell membranes as
1980s because of medical and socioeconomic
those that cause pharyngitis and therefore do not elicit
this type of response same immunity. Acute rheumatic

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fever is the result of an abnormal humoral and cellular cross-reactivity to laminin, a protein present in the
immune response to the M protein in microorganisms extracellular tissue around heart cells and in valves.
that cross-react with normal tissues (Figure 1). Cardiac myosin and vimentin are other target
Antibodies to streptococcal bacterial antigens show antigens.8,9

Figure 1. Pathogenesis and structural changes of acute rheumatic heart disease.

Autoimmunity and associated intense involves the endocardium, which includes the heart
inflammation produce diffuse, proliferative, and valves. Inflammation of the endocardium causes
exudative lesions in connective tissue, especially in the swelling of the valve leaflets, with secondary erosion
heart, joints, and skin. Repeated bouts of acute along the leaflet contact lines. Small bead-like clumps
rheumatic fever lead to chronic proliferative changes of vegetation containing platelets and fibrin were
with resulting scarring, granulomas, and thrombosis. deposited on the eroded valve tissue and on the
About 10% of cases of rheumatic fever develop RHD. chordae tendineae (Figure 2). The valve loses its
RHD begins as carditis or inflammation of the heart. elasticity, and the leaflets can stick together. Scar
Although rheumatic fever can cause carditis in all tissue and shortening of the structures involved occur
three layers of the heart wall (endocardium, over time.10,11
myocardium, pericardium), the primary lesion usually

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Figure 2. Vegetation of the valve in mitral stenosis. Mitral stenosis and clumps of vegetation (V) contain platelets and
fibrin. Mitral leaflets are thickened and fused and have clumps of vegetation containing platelets and fibrin.
If the inflammation penetrates the myocardium,
local fibrin deposits develop, which are surrounded by
areas of necrosis. These necrotic fibrinoid deposits are
called Asrhof's bodies. Pericardial inflammation is
usually characterized by a serofibrinous effusion
within the pericardial cavity. Cardiomegaly and left
heart failure may occur during untreated episodes of
acute or recurrent rheumatic fever. Conduction
defects and atrial fibrillation are frequently associated
with rheumatic heart disease.
Clinical manifestations
Common symptoms of acute rheumatic fever are
fever, lymphadenopathy, arthralgia, nausea, vomiting,
epistaxis (nosebleeds), abdominal pain, and
tachycardia. The main clinical manifestations of acute
rheumatic fever (carditis, acute migratory
polyarthritis, subcutaneous nodules, chorea, and
erythema marginatum) usually occur alone or in
combination 1 to 5 weeks after streptococcal infection
of the pharynx.12-17
Carditis
Carditis occurs several weeks after the initial
infection in approximately 50% of patients with acute
rheumatic fever, with the mitral valve being the most
affected structure. Cardiac manifestations of acute
rheumatic fever may include previously undetected
murmurs caused by mitral or aortic valve dysfunction,
chest pain, and pericardial friction caused by
pericardial inflammation or cardiomegaly unexplained
by heart failure.
Polyarthritis
Acute migratory asymmetric polyarthritis
(inflammation of more than one joint) occurs in the
majority of individuals with rheumatic fever, although
severe monoarthritis is also an emerging feature in
high-risk populations. Although any synovial joint
may be involved, the large limb joints are most
commonly involved. Two or more joints are usually
involved simultaneously or successively, with each
joint being symptomatic for approximately 2 to 3 days,
while polyarthritis as a whole persists for up to 3
weeks. Exudative synovitis causes heat, redness,
swelling, intense pain, and tenderness but does not
cause permanent disability.
Subcutaneous nodules
Palpable subcutaneous nodules occur in less than
5% of cases of acute rheumatic fever. They develop
over bony prominences and along the extensor
tendons of the elbows, wrists, knees, and ankles. They
do not interfere with joint function and often go
unnoticed.
Chorea
Sydenham chorea, or St. Vitus, is a CNS disorder
characterized by sudden, aimless, disorganized, and
involuntary movements. It affects up to 15% of people
with rheumatic fever and is the most common
acquired chorea in children. Korea is self-limiting,
although severe cases may require the use of
dopamine receptor blockers and antiepileptic drugs. It
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 Heart Association (Table 1). Supportive evidence for
resolves in 1 to 6 months and has no permanent
group A beta-hemolytic streptococci includes positive
neurological sequelae.
throat cultures and measurement of serum antibodies
to hemolytic factor streptolysin O. However, cultures
Marginal erythema
may be negative when a rheumatic attack begins.
Erythema marginata is a rare manifestation
Several other antibody tests are sensitive
and presents as a characteristic rash that
prognosticators of streptococcal infection, including
often accompanies acute rheumatic fever. It
anti-deoxyribonuclease B (anti-DNase B), anti-
consists of annular erythema with a nonpruritic
hyaluronidase, and anti-streptozyme (ASTZ).
appearance and pink, erythematous macules
Measurements of elevated white blood cell count,
radiating outward. The rash is temporary and can
erythrocyte sedimentation rate, and C-reactive protein
change within minutes or hours.
suggest inflammation. All three usually increase when
Evaluation and treatment the heart or joint symptoms begin to appear. 18-20
The original Jones criteria for the diagnosis of
rheumatic fever have been updated by the American

Table 1. Major and minor diagnostic criteria for acute rheumatic fever according to the Jones criteria.

For all patient populations with evidence of previous group A streptococcal infection: one major plus two
minor manifestations

Major criteria in a low-risk population Major criteria in a high-risk population

Carditis (clinical and/or subclinical) Carditis (clinical and/or subclinical)

Arthritis (polyarthritis only) Arthritis (monoarthritis or polyarthritis; polyarthralgia)
Chorea Chorea
Marginal erythema Marginal erythema
Subcutaneous nodules Subcutaneous nodules

Minor criteria in a low-risk population Minor criteria in moderate and high-risk populations

Polyarthralgia Monoarthralgia
Fever (≥38°C; 100.4°F) Fever (≥38°C; 100.4°F)
ESR ≥60 mm/hour and/or CRP> 3,0 mg / dL ESR ≥30 mm/hour and/or CRP> 3.0 mg / dL
Prolonged PR interval Prolonged PR interval

CRP, C-reactive protein; ESR, erythrocyte sedimentation rate.

Therapy for acute rheumatic fever is aimed at continuous prophylactic antibiotic therapy may be
eradicating streptococcal infection regimen 10 days of required for up to 5 years.
antibiotics. NSAIDs are used as agents of anti-
inflammatory for rheumatic carditis and arthritis and 2. Conclusion
help relieve symptoms but do not prevent Rheumatic fever is a systemic inflammatory
complications. Serious carditis may require diuretics disease caused by an immune and inflammatory
and vasodilators, and recovery may take up to 12 response to infection by group A beta-hemolytic
months. Damaged valve repair surgery may be streptococci in individuals with a genetic
necessary in cases of recurrent chronic rheumatic predisposition. If left untreated, rheumatic fever can
fever or carditis. Persistent chorea requires lead to scarring and structural deformities of the heart
psychosocial support, protection against falls and resulting in rheumatic heart disease (RHD).
injuries, and treatment with antiepileptic or
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