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Epidemiology of Rheumatic fever &

Rheumatic heart disease

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Definition
Rheumatic fever is an immunologically mediated
inflammatory disorder, which occurs as a sequel to group A
streptococcal pharyngeal infection.
Multisystem disease affecting the heart, joints, brain,
cutaneous and subcutaneous tissues
Major public health problem in heavily populated
underdeveloped and developing countries
Preventable disease
The most important consequence of rheumatic fever is chronic
rheumatic heart disease, characterized by fibrotic valvular
disease causing severe cardiac dysfunction decades later
Only 3% pharyngitis patients develop RHD
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Importance of RF
It is a Human Leukocyte Antigen (HLA) associated disease, with antibodies, thus
a classical autoimmune chronic disease, yet most people consider it an infectious
disease.
Unlike practically any chronic diseases, it decreases with increasing
socioeconomic status.
It is one of the few autoimmune chronic diseases where both the genetic factors
(HLA) and the environmental factors (group A Streptococci) are known.
The immunologic events leading to the disease through superantigen are
beginning to be worked out.
Many years ago rheumatic fever/heart disease was the major killer of children
worldwide. Now in developed countries most physicians will never see a case. If
we can do this with rheumatic fever, we can also do it with many other HLA
associated diseases.

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Epidemiology
Ages 5-15 yrs are most susceptible
Rare <3 yrs
Girls>boys
Common in 3rd world countries
Environmental factors-- over crowding,
poor sanitation, poverty,
Incidence more during winter & early
spring
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Clinical Features
1.Arthritis
Migratory polyarthritis, involving major joints
Commonly involved joints-knee,ankle,elbow
& wrist
Occurs in 80%, involved joints are exquisitely
tender
In children below 5 yrs arthritis usually mild
but carditis more prominent
Arthritis do not progress to chronic disease
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Arthritis

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Clinical Features (Contd)

2.Carditis
Manifest as pancarditis(endocarditis,
myocarditis and pericarditis),occurs in 40-50% of
cases
Carditis is the only manifestation of rheumatic
fever that leaves a sequelae & permanent damage
to the organ
Valvulitis occurs in acute phase
Chronic phase- fibrosis, calcification & stenosis of
heart valves.
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Heart Disease

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Clinical Features (Contd)

3.Sydenham Chorea

Occurs in 5-10% of cases

Mainly in girls of 1-15 yrs age
May appear even 6 months after the attack
of rheumatic fever
Clinically manifest as- clumsiness,
deterioration of hand-writing, emotional
lability or grimacing of face

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Sydenham Chorea

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Clinical Features (Contd)

4. Erythema Marginatum

Occur in <5%.
Unique, transient lesions of 1-2 inches in
size
Pale center with red irregular margin
More on trunks & limbs & non-itchy
Worsens with application of heat
Often associated with chronic carditis
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Erythema
Marginatum

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Clinical Features (Contd)

5.Subcutaneous nodules

Occur in 10%
Painless, pea-sized, palpable nodules
Mainly over extensor surfaces of joints,
spine, & scalp
Associated with strong seropositivity
Always associated with severe carditis

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Subcutaneous
nodules

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Clinical Features (Contd)

Other features (Minor features)

Fever – Low grade

Arthralgia
Pallor
Anorexia
Loss of weight

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Laboratory Findings
High ESR
Anemia, leucocytosis
Elevated C-reactive protein
ASO titer >200.
(Peak value attained at 3 weeks, then
comes down to normal by 6 weeks)
Anti-DNAse B test
Throat culture-GABH streptococci
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Laboratory Findings (Contd)

ECG- prolonged PR interval

Echo - valve edema, mitral regurgitation,
LA & LV dilatation, pericardial effusion,
decreased contractility

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Rheumatic fever-diagnosis
Jones criteria for initial attack of rheumatic fever, For Diagnosis:
Two Major or One major and two minor criteria following Group-A strep.
pharyngitis. ( increased AS0 titer, positive throat culture GAS)

Major criteria Minor criteria

Carditis Clinical findings-
Polyarthritis  Arthralgia
Chorea  Fever
Subcutaneous nodules Laboratory findings-
 Elevated acute phase
Erythema marginatum reactants
raised ESR
raised CRP
 Prolonged P-R interval
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The 2 primary
features of
autoimmunity are
HLA and
Antibodies. RF is
characterized by
both. RF has
antibodies to the
heart, and other
tissues.
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Superantigens may be
what causes
epidemiologic
similarities across
autoimmune diseases.
These were first
described in the early
1990s. Streptococcal A
was the first bacteria
identified as a
superantigen. RF is a
superantigen mediated
disease.
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Pathogenesis

Group A streptococcal pharyngeal infection

Body produce antibodies against streptococci.
These antibodies cross react with human tissues because
of the antigenic similarity between streptococcal
components and human connective tissues (molecular
mimicry).
Immunologically mediated damage (autoimmune) to
human tissues which have antigenic similarity with
streptococcal components- like heart, joint, brain
connective tissues
Fibrinoid degeneration, inflammation mediated by T
lymphocytes, macrophages.

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Rheumatic fever-pathogenesis

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Rheumatic fever-epidemiology

• Parallels with epidemiology of streptococcal pharyngitis

• Incidence –
•3% in epidemics of exudative streptococcal pharyngitis in
closed community
• 0.3% in civilian population with sporadic streptococcal
throat infection
• 50% if there is a past history of rheumatic fever
• first attack between 5-15 years
• M:F equal, except Sydenham’s chorea which is more
common in girls
• poor socioeconomic conditions and overcrowding
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Rheumatic fever-Epidemiology

For rheumatic fever to occur-

• Pharyngeal infection with group A streptocooci
• Certain rheumatogenic strains of GAS with M proteins
• Throat infection of sufficient duration- persistence of GAS
• Throat infection may or may not be symptomatic
• Throat infection is a must, not with pyoderma
• Infection of sufficient duration to produce antibody
• Brisk and sufficient antibody response to the infection
• Genetic predisposition

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Much of our understanding

of the etiology of RF has
come from the brilliant
work in the military. “
Previously, was said that
one would catch the death
of cold if he went out in
cold, wet, nights with many
people around”. It is here
that the agent of RF is
transmitted.
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There is an
enormous
variation of
rheumatic fever
around the world.
The geographic
variation is the
greatest seen for
almost any
chronic disease.
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The pattern of
onset starts with
few cases in the
very earlier years,
with a rapid
increase in
incidence in the
pre pubertal and
pubertal years,
followed by a
decline.
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Non-whites have
tended to have a
lower incidence
in the US.
However, this is
unlikely to be
genetic as when
socioeconomic
status was
adjusted, the
differences were
eliminated.
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Males tended to have a

greater peak age of
onset than females in the
US. This could be related
to a lower probability of
group activities for
females. However there
are also sex hormone
relationships to the
disease as well as the
autoimmune disease.

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Underweight
children are
particularly afflicted
by RF. This could
be due to them
being poorer, thus
having a greater
risk of exposure, or
due to the effect of
nutrition or immune
status.

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RF shows a
decrease in
incidence in the
warm summer
months. This is
consistent with the
geographic variation
which demonstrates
higher incidence in
the cooler climates.
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There is an extremely
successfulness in
preventing RF with
over a 98% reduction
in about 25 years. This
was not done through
screening for
antibodies, and
immunoprevention. It
was accomplished in
part by epidemiology
and public health.
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RF and other
autoimmune
diseases do not act
like typical chronic
diseases. They have
epidemics and they
are frequent. It is in
the epidemics that
the etiology of
autoimmune
diseases will be
found.
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RF is characterized
by enormous
geographic and
SES differences,
within countries as
well. There can be
a 30 fold variation
across SES, and
20 fold across
geographic area
within the same
country.
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With increasing
altitude in feet, there
is an increase in the
incidence of
rheumatic fever. It is
of interest that several
other autoimmune
diseases exhibit a
relationship to
altitude.

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The relationship between

streptococcal A infection,
and RF manifestations.
The problem is that the
infection occurs
sometimes months before
symptoms, and it is
difficult to link the
infection to Rheumatic
Fever. This may be
similar to that of other
autoimmune diseases.
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Treatment
Bed rest 2-6 weeks
Supportive therapy- treatment of heart failure
Anti-streptococcal therapy- Benzathine penicillin 1.2
million units or oral penicillin 10 days, if allergic
erythromycin 10 days
Anti-inflammatory agents-
 Aspirin 100 mg/kg per day for arthritis and in the absence
of carditis- for 4-6 weeks to be tapered off
 Corticosteroids in presence of carditis – 1-2 mg/kg per day
– for 4-6 weeks to be tapered off
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Rheumatic fever- prevention

Secondary prevention – prevention of recurrent attacks
•Benzathine penicillin G 1.2 million units IM every 4 weeks
•Or Penicillin V 250 mg twice daily orally
•Or Sulfadiazine 1 g daily orally
•If allergic to both – Erythromycin 250 mg twice daily orally
Duration of secondary rheumatic fever prophylaxis
•Rheumatic fever + carditis + persistent valve disease-
10 years since last episode or until 40 years of age,
sometimes life long
•Rheumatic fever + carditis + no valve disease –
10 years or well into adulthood whichever is longer
•Rheumatic fever without carditis-
5 years or until 21 years whichever is longer
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Rheumatic fever-prognosis
Prognosis is good if recurrence is prevented by continuous
antibiotic prophylaxis- particularly if no carditis in the
initial attack
If carditis, half of them can develop chronic rheumatic
heart disease. Recurrence following streptococcal sore
throat is high in patience with previous carditis
Takes 10-20 years in western world but earlier in
underdeveloped world due to malignant nature of the
disease
Mitral valve is most commonly affected, followed by
aortic and tricuspid valves
So these patients need long term follow up
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Rheumatic heart disease

Definition: A condition resulting from having
Rheumatic fever as a child. ( weakening of the
heart valve)
Signs and Symptoms: Damaged heart valve which
can progress into serious even a disabling
condition.
Causes: A disease process that begins with the
untreated strep. throat infection.
Treatment: Prevention is the best measure to be
taken. Treat strep. throat infections with
antibiotics so it does not progress into Rheumatic
Fever.
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Acute Rheumatic vegetations:

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Fish mouth Mitral stenosis:

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