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ACUTE RHEUMATIC FEVER

The annual incidence of ARF in developing
countries is as high as 282/100,000 population
Worldwide, rheumatic heart disease remains
the most common form of acquired heart
disease in all age groups, accounting for as
much as 50% of all cardiovascular disease and
as much as 50% of all cardiac admissions in
many developing countries

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Pick incidence ARF is 5–15 yr of age, the age of
greatest risk for GAS pharyngitis

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Etiology
• Occurs as a delayed sequel of group A
streptococcal infection of the pharynx but not of
the skin
• The attack rate of ARF after streptococcal infection
varies with the severity of the infection, ranging
from 0.3% to 3%
• Predisposing factors include family history of
rheumatic fever, low socioeconomic status
(poverty, poor hygiene, medical deprivation)
• Certain serotypes of GAS (M types 1, 3, 5, 6, 18,
24) are more frequently isolated from patients ARF

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2/3 of the patients with an acute episode of
rheumatic fever have a history of an upper
respiratory tract infection several weeks before

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Pathogenesis
Two theories has been proposed
[Link] theory
GAS produces cytotoxic enzymes
especially streptolysin O, that have
cytotoxic effect on mammalian cells
• But an able to explain the latent period
between GAS pharyngitis and the onset of
acute rheumatic fever

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2. Immune mediated
Molecular mimicry between GAS antigens
and host antigens, results antibodies directed
to GAS antigens cross react with mammalin
tissue
Common antigenic determinants are shared
between certain components of GAS e.g. M
protein and specific mammalian tissues e.g.,
heart, brain, joint

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C/M and Diagnosis
The diagnosis of ARF is based on the modified
Jones criteria
There are 5 major and 4 minor criteria and an
absolute requirement for evidence
(microbiologic or serologic) of recent GAS
infection
Diagnosis of ARF is established by 2 major
criteria or 1 major and 2 minor criteria and
meets the absolute requirement

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There are 3 circumstances in which the
diagnosis of ARF can be made without strict
adherence to the Jones criteria
Chorea
indolent carditis
 recurrences of acute rheumatic fever

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The five major manifestations

Migratory arthritis (predominantly involving

the large joints)
Carditis and valvulitis (eg, pancarditis)
Central nervous system involvement (eg,
Sydenham chorea)
Erythema marginatum
Subcutaneous nodules

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The four minor manifestations
Arthralgia (in the absence of polyarthritis as
a major criterion)
Fever (typically temperature ≥102°F and
occurring early in the course of illness)
Elevated acute phase reactants [erythrocyte
sedimentation rate (ESR), C-reactive protein
(CRP)]
Prolonged PR interval

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 Supporting evidence of antecedent group a
streptococcal infection
 Positive throat culture or rapid streptococcal
antigen test
 Elevated or rising streptococcal antibody titer

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Migratory polyarthritis
Earliest manifestation of ARF
Arthritis occurs in about 75% of patients ARF
Typically involves larger joints, particularly the
knees, ankles, wrists, and elbows migratory in
nature
The joint is hot, red, swollen, and tender
Mild involvment resolution with in 1-3 days
severe joint involvement may take several
weeks

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Inverse relationship between the severity of
arthritis and the severity of cardiac
involvement
Responsive for salicylate treatment
Synovial fluid analysis sterile inflammatory
fluid, 10,000–100,000 white blood cells/mm 3
with a predominance of neutrophils, a protein
of about 4 g/dL, a normal glucose
Typically non deforming

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Valvular insufficiency is characteristic,
whereas valvular stenosis takes several years
or even decades
Tacycardia and cardiac murmurs with or
without sign of involvment of myocardium or
pericardium
Moderate to severe rheumatic carditis can
result in cardiomegaly and congestive heart
failure

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Sydenham chorea
Occurs in about 10–15% of patients
Longer latent period from acute GAS infection to
chorea
presents as an isolated, frequently subtle,
neurologic behavior disorder occasionally is
unilateral
 Emotional lability, incoordination, poor school
performance, uncontrollable movements, and
facial grimacing, exacerbated by stress and
disappearing with sleep, are characteristic
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Clinical manuovers
(1) Milkmaid's grip -irregular contractions of
the muscles of the hands while squeezing the
examiner's fingers
(2) spooning and pronation of the hands
when the patient's arms are extended,
(3) wormian darting movements of the
tongue upon protrusion
(4) examination of handwriting to evaluate
fine motor movements

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Erythema marginatum
Occurs in <3% of patients with ARF but
characteristic rash of acute rheumatic fever.
 It consists of erythematous, serpiginous,
macular lesions with pale centers that are not
pruritic
It occurs primarily on the trunk and
extremities, but not on the face, and it can be
accentuated by warming the skin

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Erythema marginatum
Subcutaneous nodules
are a rare ≤1% of patients with ARF
 consist of firm nodules approximately 1 cm in
diameter along the extensor surfaces of
tendons near bony prominences
 There is a correlation between the presence of
these nodules and significant rheumatic heart
disease

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 Supportive evidence
Throat culture or rapid streptococcal antigen
test only 10–20% results are positive in ARF
Elevated or increasing serum anti
streptococcal antibody titers is important for
diagnosis ASO titer is the usual test
Titer varies based on age healthy elementary
school children have 200-300 Todd units per
mL
With single determination of ASO titer 80%
positivity

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Treatment
 Bed rest
Antibiotic Therapy
Oral penicillin or erythromycin for 10 days , or a
single intramuscular injection of benzathine
penicillin to eradicate GAS from the upper
respiratory tract

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Anti-inflammatory agents (e.g., salicylates,
corticosteroids)
typical migratory polyarthritis and those with
carditis without cardiomegaly or congestive
heart failure should be treated with oral
salicylates
 The usual dose of aspirin is 100 mg/kg/day in
4 divided doses PO for 3–5 days, followed by
75 mg/kg/day in 4 divided doses PO for 4 wk

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Carditis and cardiomegaly or congestive heart
failure should receive corticosteroids
Prednisone is 2 mg/kg/day in 4 divided doses
for 2–3 wk followed by a tapering of the dose
that reduces the dose by 5 mg/24 hr every 2–3
days
At the beginning of the tapering of the
prednisone dose, aspirin should be started at
75 mg/kg/day in 4 divided doses for 6 wk

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Secondary prevention
Prophylaxis
Recurrence is common in the 1st 2 yr of an
episode
Decreases as age increases
Penicillin is given if allergic erythromycin is used
Intramuscular benzanthine penicillin every 3-
4wks is used commonly
Duration of prophylaxis based on the clinical
manifestation

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RF without carditis 5 yr or until 21 yr of age,
whichever is longer
RF with carditis but without residual heart
disease 10 yr or well into adulthood, whichever
is longer
RF with carditis and residual heart disease At
least 10 yr since last episode and at least until
40 yr of age; sometimes lifelong prophylaxis

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Sydenham Chorea
 Sedatives may be helpful early in the course of
chorea; phenobarbital (16–32 mg every 6–8 hr
PO) is the drug of choice
If phenobarbital is ineffective, then haloperidol
(0.01–0.03 mg/kg/24 hr divided bid PO) or
chlorpromazine (0.5 mg/kg every 4–6 hr PO)
should be initiated

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Complications
 Arthritis and chorea resolve completely
without sequelae.
Long-term sequelae of rheumatic fever are
usually limited to the heart
Increased risk for developing infective
endocarditis

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Prognosis
Depends on the C/M, the severity of the
initial episode, and the presence of
recurrences
 70% of carditis during the initial episode of
ARF recover with no residual heart disease
The more severe the initial cardiac
involvement, the greater the risk for residual
heart disease

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Rheumatic heart disease
Most severe sequale of ARF
Major cause of valvular heart disease
Mitral valve commonly involved followed by
aortic valve then tricuspid valve
Pulmonary valve rarely involved

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JONES
MajorAND WORLD HEALTH ORGANIZATION
criteria CRITERIA
Minor FOR DIAGNOSIS OF
criteria
RHEUMATIC
Carditis FEVER Fever
Chorea Arthralgia
Polyarthritis Elevated Acute-Phase Reactants
Erythema marginatum Erythrocyte sedimentation rate
Subcutaneous nodules C-reactive protein
Prolonged PR interval (ECG)
Supporting evidence of antecedent group A streptococcal
pharyngeal infection
Positive throat culture or rapid streptococcal test
Elevated or rising streptococcal antibody titer
Criteria for diagnosis of:
Primary episode of RF 2 major or 1 major plus 2 minor
plus evidence of preceding strep
infection
RF recurrence in a patient 2 major or 1 major plus 2 minor
without RHD plus evidence of preceding strep
infection
RF recurrence in a patient with 2 minor plus evidence of preceding
RHD strep infection
Dagnaneh A ,MD
Definition
Infective Endocarditis (IE): microbial
infection of the heart’s endocardial surface
Classified into four groups:
Native Valve IE
Prosthetic Valve IE
Intravenous drug abuse (IVDA) IE
Nosocomial IE
Infective endarterities; microbial invasion of
the endocardial surface of the great vessels
like arteries, PDA , aneurisms,AV shunts
Epidemiology
Incidence 3.6 to 7.0 cases per 100,000 patient-
years.
Presently, the incidence of endocarditis is
approximately 1 case per 1000 hospital admissions
Over all prevalence is increasing
◦ Agresive management at NICU/PICU
◦ Increased survival of infants and children with CHD
Mortality rate varies from one clinical setting to
the other and depends on the etiology
Predisposing
Preexisting cardiacfactors
abnormalities are found in
approximately 75% of children with bacterial
endocarditis.
 Congenital heart disease
 Rheumatic heart disease
 Intravenous invasive catheter
 Cardiac surgery
 Prosthetic valves
 Dental ,GI,genitourinary procedures
 Injection drug use
Clinical manifestations
The features of bacterial endocarditis are due
to
 bacteremia,
 local cardiac invasion by organisms,
 peripheral embolization, and
 formation of immune complexes
more than 85-90% of patients are febrile.
Other historical features include chills, sweats,
anorexia, malaise, cough, headache,
myalgia/arthralgia, and confusion.
Clinical manifestations
Fever is present in 80-90% of patients with
endocarditis.
A new or changing heart murmur is noted in
80-85% of patients.
Neurologic abnormalities occur in
approximately 30-40% of patients and are
more frequent in endocarditis caused by S
aureus. Symptoms include stroke,
intracerebral hemorrhage, and subarachnoid
hemorrhage
Clinical …Peripheral symptoms
◦ Petechiae are the most common of these symptoms
(40%).
◦ Splinter and subungual hemorrhages are dark red
linear streaks in the nail beds of the fingers and
toes.
◦ Osler nodes are small, tender, subcutaneous
nodules that develop in the pulp of the digits.
CHF is due to valve destruction or distortion or
rupture of the chordae tendineae.
Splenomegaly is noted in approximately 15-50%
of patients.
Renal insufficiencyoccurs in fewer than 15% of
patients with endocarditis.
Diagnosis
The critical information for appropriate
treatment of infective endocarditis is
obtained from blood cultures.
Three to five separate blood collections
should be obtained after careful
preparation of the phlebotomy site.
In 90% of cases of endocarditis, the
causative agent is recovered from the 1st
two blood cultures
Diagnosis
Antimicrobial pretreatment of the patient
reduces the yield of blood cultures to 50–
60%.
Duke’ s Major criteria
Positive blood cultures for IE
 Typical microorganism for infective endocarditis
from two separate blood cultures
 Viridans streptococci
 Streptococcus bovis
 HACEK group - Haemophilus spp,.
Actinobacillus, Cardiobacterium hominis,
Eikenella spp, and Kingella kingae.
 Staphylococcus aureus
 Community-acquired enterococci, in the absence
of a primary focus.
Major criteria
Persistently positive blood culture, defined as
recovery of a microorganism consistent with IE
from:
 Blood cultures drawn more than 12 hours apart OR
 All of three or a majority of four or more separate
blood cultures, with first and last drawn at least
one hour apart
 Single positive blood culture for Coxiella burnetii
or antiphase I IgG antibody titer >1:800*
Evidence of endocardial involvement (histology).
Major criteria
Definition of positive echocardiogram
 Oscillating intracardiac mass, on valve or supporting
structures, or in the path of regurgitant jets, or on
implanted material, in the absence of an alternative
anatomic explanation OR
 Abscess OR
 New partial dehiscence of prosthetic valve
 New valvular regurgitation
 Increase in or change in preexisting murmur not
sufficient
 Minor criteria

Predisposition - predisposing heart condition

or intravenous drug use
Fever - 38.0°C (100.4°F)
Vascular phenomena
 major arterial emboli,
 septic pulmonary infarcts,
 mycotic aneurysm,
 intracranial hemorrhage,
 conjunctival hemorrhages,
 Janeway lesions
Minor criteria
Immunologic phenomena
 glomerulonephritis,
 Osler's nodes,
 Roth spots,
 rheumatoid factor
Microbiologic evidence - positive blood culture
but not meeting major criteria ,OR serologic
evidence of active infection with organism
consistent with IE
Echocardiographic minor criteria eliminated
Prognosis
In the pre-antibiotic era, infective
endocarditis was a fatal disease.
mortality rate is variable.
morbidity occurs in 50–60% of
children with documented infective
endocarditis;
Heart failure
Mechanical obstruction by vegitation
Myocardial abscesses
toxic myocarditis
 arrhythmias
complications
 Systemic emboli
 central nervous system .
 meningitis
 osteomyelitis, arthritis, renal abscess, and
immune complex–mediated glomerulonephritis
Pulmonary emboli in children with VSD or the
tetralogy of Fallot,
mycotic aneurysms
rupture of a sinus of Valsalva
obstruction of a valve secondary to large
vegetations,
acquired VSD
References
Nelson text book of pediatrics 18th edn.
Moss and Adams cardiac disease in infants and
children.
Emedicine
Uptodate 16.3
Braunwald’s text book of cardiology.
Tnxs