RHEUMATIC FEVER AND RHD

DR. GLORY SIMON JOSEPH

PEDIATRICIAN, CARDIOLOGIST
BUGANDO MEDICAL CENTRE
LECTURER CUHAS
MWANZA TANZANIA
 WHATIS
RHEUMATIC FEVER
?
• Rheumatic fever is an immunologically mediated
inflammatory disorder, which occurs as a sequel to
group A streptococcal pharyngeal infection.
• Multisystem disease affecting connective tissue
particularly of the heart, joints, brain, cutaneous and
subcutaneous tissues
• RF – not a communicable disease
but results from a communicable disease
(streptococcal pharyngitis).
• The illness is so named because of its similarity in
presentation to rheumatism.
• RF->-> RHD (rheumatic heart
disease); a crippling disease.
• Epidemiological point of view these
cannot be separated.
[WHO CHRONICLE 1969]
• RF and RHD - diseases of the poor
> most prevalent in underdeveloped and
developing countries.
• Preventable disease.
 PROBLEM STATEMENT
• RF and RHD is the most common cause of
heart disease in 5-30 age groups
throughout the world.
• It accounts for 12-65% of hospital
admissions related to CVD in developing
countries.
• There has been marked decrese in cases of
RF and RHD in places that have
implemented preventive programs.
IN INDIA
• RHD is prevalent in range of 5-7/1000 in 5-15
age groups.
• About 1 million cases of RHD
• RHD constitutes 20-30% hospital admissions
due to CVD.
• Streptococcal infections common in children
living in under – privileged conditions and RF
accounts for 1-3% of the cases.
• Important cause of chronic disease and death in
developing world
• Underdiagnosed and undertreated
• Ages 5-15 yrs are most susceptible
• Rare <3 yrs
• Common in 3rd world countries
• Environmental factors  over crowding, poor
sanitation, poverty, poor housing.
• Incidence more during fall, winter & early spring.
 FAC
AGENT
.

ENVIRONMENTAL
FAC. HOST
FAC.
 AGENT FACTORS
• Streptococcal sore throat
• Not all strains of Group A Streptococci
(GAS) lead to RF
• Rheumatogenic potential
• Recently virus (coxsackie B-4) has been
suggested as causative agent with
streptococcus acting as conditioning agent.
 HOST AND ENVIRONMENTAL FACTORS
• AGE -> adolescents 5-15 yrs
initial attack at younger age ->valvular
lesion faster
Juenile mitral stenosis
• SEX -> equal
• IMMUNITY -> Toxic –immunological
hypothesis
• SOCIO-ECONOMIC STATUS -> Disease for
Low Social economic status.
• HIGH RISK GROUP -> 5-15 yrs school-age
children living in closed community
PATHOPHYSIOLOGY
 How does streptococci cause Rheumatic fever?
•The streptococci contain antigens , to these antigens antibodies
are formed in the body.
•But before these antibodies are formed the streptococcal
infection is over.
•So these antibodies are searching streptococci but find none
there
•Instead these antibodies now react to various tissues in the
body.
•Since most cells of body have same constituents as that of
streptococcal cells therefore react with cells of body causing
antigen antibody reaction. Thus rheumatic fever is immune
mediated.
•These auto immune antibodies produce inflammatory lesions
primarily involving the heart, joints, and subcutaneous tissue
• Group A strep pharyngeal infection precedes clinical
manifestations of ARF by 2 - 6 weeks.

• 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) [there is certain amino acid
sequence that is similar btw GAS and human tissue]

• Immunologically mediated inflammation & damage

(autoimmune) to human tissues which have antigenic
similarity with streptococcal components- like heart,
joint, brain, subcutanous.
• Epitopes present in cell wall ,CM, str. M protein are
immunologically similar to molecules in human myosin,
tropomyosin,keratin,actin,etc.-->MOLECULAR MIMICRY
• Because of the similarity btw hyaluronic acid
in GAS capsule and in the connective tissue
of the joints, Antibodies produced against
GAS capsule will start to attack the joints and
causes arthritis.
• M-protein in GAS cell wall and the
myocardium are similar, thus Antibodies
produced against GAS cell wall will attack
heart and will cause carditis and so forth.
• Similarly Antibodies against NAD in GAS will
affect cardiac valve tissue causing valvular
damage.
CLINICAL FEATURES
 FEATURES
• Following upper airway infection with GAS
Silent period of 2 - 6 weeks
• Sudden onset of fever, pallor, malaise,
fatigue.
•Commonly GAS streptococcal infection is
subclinical; such cases confirmed using
streptococcal antibody testing .
MAJOR MANIFESTATIONS

Polyarthritis

Carditis

Sydenham’s chorea

Erythema marginatum

Subcutaneous nodules
 MINOR
MANIFESTATION
S
Involvement
of lung,
Fever Epistaxis kidneys and
CNS

Arthralgia Serositis
 1. POLYARTHRITIS
 Most common feature: present in 75-90% of
patients

 Joint is arthritic -> i.e inflammed.

 Painful, migratory, short duration.

 Usually > 5 joints affected and mainly large joints

Knees, ankles, wrists, elbows, shoulders.

 Small joints and cervical spine less commonly involved.

 Excellent response of salicylates and
NSAIDS.

 Pain and swelling come on quickly and

subsides within 5-7 days.

 In children below 5 yrs arthritis usually

mild but carditis more prominent.

 Arthritis do not progress to chronic

disease
 2. CARDITIS
• Early and most serious manifestation
• Manifest as pancarditis
• Occur in 60-70% of cases
• Carditis is the only manifestation of
rheumatic fever that leaves a sequelae &
permanent damage to the organ
• Valvular damage is the hallmark of RF
• Chronic phase- fibrosis, calcification &
stenosis of heart valves (fishmouth valves)
 CARDITIS
• Characteristic Aschoff bodies, composed of swollen
eosinophilic collagen surrounded by lymphocytes
and macrophages can be seen on light microscopy.
The larger macrophages may become Aschoff giant
cells.
 Valvular lesion most common: mitral and aortic
 Any cardiac tissue may be affected
 Seldom see isolated pericarditis or
myocarditis
RHEUMATIC HEART DISEASE
• Rheumatic Heart Disease is the permanent heart
valve damage resulting from one or more attacks of
ARF.
• It is thought that 40-60% of patients with ARF will
go on to developing RHD.
• Sadly, RHD can go undetected with the result that
patients present with debilitating heart.
 High pulse
rate

mitral or aortic regurgitation-

Murmur endocardium
involved
Cardiomegaly myocardium
involvement
Pericardial
friction Pericar
rub ditis
Prolonged PR Myocardial inflammation
interval affecting electrical
conduction
Cardiac
failure
 Investigations for evidence of carditis
• Chest x-ray – cardiomegaly, pulmonary
venous congestion.

• ECG- First degree A-V block, T wave

changes, low voltage QRS.

• Echocardiogram – cardiac dilatation, valve

involvement, pericardial effusion
 Vegetation

Stick in Perforation

Mitral Valve
 3. SYNDENHAM’S CHOREA
• Occur in 5-10% of cases
• Mainly in girls of 1-15 yrs age
• Late manifestation of RF - months after
infection.
• Spasmodic, unintentional, jerky choreiform
movements, Speech affected, restless
• Choreiform movements particularly affect the head
(darting movement of tongue) and upper limb.
• First sign: difficulty walking, talking, writing
• Usually benign and resolves in 2 - 3 months
• Disappears leaving no residual damage.
 4. ERYTHEMA MARGINATUM
• Occur in <7%.
• Unique, transient, serpiginous-looking
lesions of 1-2 inches in size
• Pink macules - Clear centrally, serpiginous
spreading edge .
• More on trunks & limbs & non-itchy
• Almost never on face
• Worsens with application of heat
• Often associated with chronic carditis
 5. SUBCUTANEOUS NODULES
• Small, painless, mobile hard lumps beneath skin.
• Most common along - extensor surfaces of joint-
Knees, elbows, wrists
• Also: on bony prominences, tendons, dorsi of
feet,occiput or cervical spine
• Appears 4 weeks after onset of RF
• Delayed manifestation, disappears – leaves no
residual damage.
• Occur in 9 - 10% of cases
• Often associated with carditis
Subcutaneous nodules- Firm, non-tender, isolated or in clusters
 6. FEVER
• Present at onset of
acute illness
• High grade fever
>39ºC
• Lasts for about 12
weeks, tends to recur
 LAB DIAGNOSIS
• High ESR
• Anemia, leucocytosis
• Elevated C-reactive protein
• Elevated ASO titre
• Anti-DNAse B test
• Throat culture - GABH streptococci
• There is no definitive test.
• Diagnosis of ARF relies on presence of
combination of typical clinical features
together with evidence of the precipitating
GAS infection .

• This uncertainty led Dr.T.Duckett Jones in

1944 to develop a set of criteria -> Jones
Criteria to aid diagnosis.

• Now Diagnosis based on MODIFIED JONES

CRITERIA .
Major manifestations Carditis
Polyarthritis
Chorea
Erythema marginatum
Subcutaneous nodules
Minor manifestations Clinical: fever, polyarthralgia
Laboratory: elevated erythrocyte
sedimentation rate or leukocyte count

Electrocardiogram: prolonged P-R

interval
Supporting evidence of a Elevated or rising anti-streptolysin
preceding streptococcal infection O or other streptococcal antibody,
within the last 45 days or
A positive throat culture, or
Rapid antigen test for group A
streptococcus, or

Recent scarlet fever

 2002–2003 World Health Organization Criteria for the
Diagnosis of Rheumatic Fever and Rheumatic Heart Disease
(Based on the 1992 Revised Jones Criteria)
Diagnostic Categories Criteria
Primary episode of rheumatic fever Two major or one major and two minor
manifestations plus evidence of preceding
group A streptococcal infection
Recurrent attack of rheumatic fever in a Two major or one major and two minor
patient without established rheumatic heart manifestations plus evidence of preceding
disease group A streptococcal infection
Recurrent attack of rheumatic fever in a Two minor manifestations plus evidence of
patient with established rheumatic preceding group A streptococcal infection
heart disease
Rheumatic chorea Other major manifestations or evidence of
Insidious onset rheumatic carditis group A streptococcal infection not
required
Chronic valve lesions of rheumatic heart Do not require any other criteria to be
disease (patients presenting for the first diagnosed as having rheumatic heart
time with pure mitral stenosis or mixed disease
mitral valve disease and/or aortic valve
• Step I - primary prevention
(eradication of streptococci)

• Step II - anti inflammatory

treatment (aspirin, steroids)

• Step III- supportive

management & management of
complications

• Step IV- secondary prevention

(prevention of recurrent attacks)
 PRIMARY PREVENTION
• AIM ; Prevent the first attack of RF, by identifying all
patients with streptococcal throat infection and treating
them with penicillin.
• Theoretically simple, in practise its difficult.
• Many infections are inapparent or if apparent are not
brought to attention of health services
• VIABLE APPROACH; concentrate on high risk
groups i.e school age children.
• Surveillance for streptococcal pharyngitis
STEP I:Primary Prevention of Rheumatic
Fever (Treatment of Streptococcal Tonsillopharyngitis)
• Agent Dose Mode Duration
• Benzathine penicillin G 600 000 IU for patients Intramuscular Once
< 27 kg (60 lb) Least
1 200 000 U for patients >27 kg
expen
or sive
• Penicillin V Children: 250 mg 2-3 times daily Oral metho
(phenoxymethyl penicillin) Adolescents and adults:
500 mg 2-3 times daily d
10
• For individuals allergic to penicillin d
• Erythromycin: 20-40 mg/kg/d 2-4 times daily Oral 10 d
Estolate (maximum 1 g/d)

or
• Ethylsuccinate 40 mg/kg/d 2-4 times daily Oral 10 d
(maximum 1 g/d)
Recommendations of American Heart Association
 Step II: Anti inflammatory treatment

CLINICAL CONDITION DRUG

Arthritis only Aspirin 75-100 mg/kg/day ,
give
as 4 divided doses for 6
weeks (attain a body level
20-30 mg/dl)
Carditis Corticosteroids 1-2 mg/kg
per day
– for 4-6 weeks to be tapered
off
 3. Step III: Supportive management &
management of complications

• Bed rest
• Treatment of congestive cardiac failure:
digitalis, diuretics
• Treatment of chorea:
-diazepam or haloperidol
• Rest to joints & supportive splinting
STEP IV : Secondary Prevention of Rheumatic Fever
(Prevention of Recurrent Attacks)

Agent Dose Mode

Benzathine penicillin G 1 200 000 U every 3 weeks* Intramuscular

or
Penicillin V 250 mg twice daily Oral

For individuals allergic to penicillin and sulfadiazine

Erythromycin 250 mg twice daily Oral

Recommendations of American Heart Association

Category Duration
Duration of Secondary Rheumatic Fever Prophylaxis
Rheumatic fever without Carditis At least 5 years or until 18 years
Whichever is longer
Rheumatic fever with carditis and At least 10 years since last residual
heart disease episode or at least 40 years
(persistent valvar disease*) Sometimes life long prophylaxis

Rheumatic fever with carditis but no At least 10 years or until adulthood years
residual heart disease (no valvar Whichever is longer
disease*)

More severe valvular disease Lifelong

Post-valve surgery cases

*Clinical or echocardiographic evidence.

Recommendations of American Heart Association

• Secondary prophylaxis is more effective
when done on a Register based method

• A register of cases of RF and RHD is kept.

• This is used to improve treatment adherence

in order to prevent recurrent RF and the
development of RHD, necessitating surgery.
 NON- MEDICATED MEASURES
• Improvement of living standards.
• Breaking the poverty –disease –poverty cycle.
• Improvements in socio-economic status.

EVALUATION
• The prevalence of RHD in school children
from periodic surveys of random
samples.
• Samples of school in 6-14 age groups. At
5 year interval.
• Recommended sample size 20,000 to
30,000
 PROGNOSIS
• Rheumatic fever can recur whenever the
individual experience new GABH streptococcal
infection, if not on prophylactic medicines

• Good prognosis for older age group & if no

carditis during the initial attack

• Bad prognosis for younger children & those

with carditis with valvar lesions
Rheumatic heart disease is the
only truly preventable chronic
heart condition