DEFINITION

Acute Rheumatic Fever is a multisystem

disease resulting from an autoimmune
reaction to infection with Group A, beta
haemolytic streptococcus.
DEMOGRAPHY

• ARF and RHD are diseases of poverty.

• Direct relation to overcrowding.
• 15-19 million cases worldwide, 250,000 deaths every
year.
• In India, occurrence of ARF is about 5-7 per 1000
children in the age group of 5-15 years.
• 20-30% of hospital admissions due to Cardio-Vascular
causes in India is due to RHD.
EPIDEMIOLOGY

• For initial episodes:

• Age: 5-14 years, rare in people above 30 years.
• Recurrent Episodes:
• Peak at around 25-40 years of age.
• Gender:
• No gender association for ARF, but RHD and Chorea are more
common in females.
ETIOLOGY
• Exclusively caused by reactions to infection by Group A
Streptococci.
• Potential of skin infections and that of group C Strep is
being studied.
• Susceptibility might be an inherited characteristic, with
a 44% concordance in monozygotic twins as opposed
to 12% in dizygotic twins.
• Genes like HLA-DR7 and DR4- Susceptible.
• HLA DR-5&6, DR-51&52 appears to provide protection.
 RHEUMATIC
FEVER
- Autoimmune
- Inflammatory
- Non suppurative
- Develops as a sequale of
streptococcal infection
 PATHOGENESIS

1.Agent Factors
2.Host factors
3.Environment factors
4.Immune response
5.Result
 Agent factors

1. Infection of the URT with Group A Streptococcus

2. Classically, M serotypes (particularly 1, 3, 5, 6

14, 18, 19, 24, 27, 29) associated with ARF

3. New school of thought, any strain has the

potential to cause ARF
Host factors
1. Associated with susceptibility-
HLA class II alleles, HLA-DR7 and HLA-DR4
(7,4)

2. Associated with protection-

HLA class II alleles, HLA- DR5, HLA- DR6 and
HLA DQ (5,6, DQ)

3. Associated with-
-polymorphisms at TNF alpha locus
-circulating mannose binding protein
-toll like receptors
Environmental factors
1. Overcrowding
2. Poverty
3. Rural/ urban slums
4. Lack of treatment of
sore throat
 Immune response
1. Most widely accepted theory- Molecular Mimicry

2. Antigenic structure of Group A Streptococcus

1) Capsule- hyaluronic acid
2) Cell wall- protein antigens: M, T, R (M-major virulence factor)
- group specific antigens: N acetyl glucosamine
3) Pilli- partly M protein
- covered with lipoteichoic acid (attachment)

3. Streptococcal throat infection

Latent period: 10d- 3w Rheumatic fever
1) Immune response- targeted at streptococcal antigens- M protein
- NAG

2) Also recognizes human tissues (Cross reactive antibodies)

3) AB binds to cells on endothelial cells on heart valves

4) Activation of adhesion molecule VCAM-1 (Vascular Cell Adhesion

Molecule)

5) Recruitment of activated lymphocytes

6) Lysis of endothelial cells in the presence of complements

7) Complements lead to release of peptides (laminin, keratin,

tropomyosin) which activates the cross reactive T cells (invade the
heart and amplify the damage)
4. Alternate hypothesis
- initial invasion due to streptococcal invasion of epithelial surfaces

- binding of M protein to type IV collagen

- allowing it to become immunogenic (not through mechanism of

MM)
Result
1. Heart valves are permanently damaged during an episode
of rheumatic fever (MV, AV)

Rheumatic fever- MR (acute and transient which goes way

when inflammation goes away)
Recurrent Rheumatic Fever ---- RHD --- HF, Stroke
RHD- MS

2. Other tissues affected -carditis (endo, myo, pan)

-chorea
-arthritis
CLINICAL FEATURES OF RHEUMATIC FEVER
 REVISED JONES CRITERIA

Major criteria Minor criteria Additional evidence

Arthritis (J) Arthralgia Evidence of previous

streptococcal infection
Carditis (O) Fever Raised ASLO titres

Subcutaneous Previous H/o Throat culture positive for

nodules (N) Rheumatic fever streptococcus
or RHD
Erythema Raised WBC
marginatum (E) count, ESR, CRP
Sydenham’s Prolonged P-R
chorea (S) interval
CLINICAL FEATURES
Polyarthritis (60-75% of the cases)
Carditis (50-60% of the cases)
Chorea (10-15% of the cases)
Erythema marginatum(<3% of the cases)
Subcutaneous nodules (<1% of the cases)
ARTHRITIS (J)
Commonest manifestation of RF and has an acute onset
Described as fleeting polyarthritis (multiple joints affected, each joint being
successively inflamed for few days)
Larger joints affected initially followed by wrist, elbow etc.
Though pain is severe, X rays do not show articular damage
No residual deformity
Carditis (O)
1. Severest of all manifestations of RF and occurs in half of the children affected
by RF.
2. Valvular damage is the hallmark of rheumatic carditis.
3. Most common valve to be affected is Mitral valve.
4. Most common valvular lesion in rheumatic fever is Mitral regurgitation.
CARDITIS (O)

During rheumatic fever, diffuse inflammation

and Aschoff bodies may be found in any of the
three layers of the heart. Thus, it is also called
PANCARDITIS.
 ASCHOFF’S NODULE

Aschoff’s body is pathognomic of Rheumatic

fever. – Robbins
Focal inflammatory lesions seen during acute
RF
During acute RF they can be found in any of
the three layers viz. pericardium,
myocardium and endocardium.
ASCHOFF’S NODULE
Consist of foci of swollen eosinophilic collagen surrounded by
Lymphocytes primarily T-cells
Occasional plasma cells
Aschoff giant cells (macrophages of RF)
Anitschkow cells
Modified macrophages with abundant cytoplasm and central round to ovoid
nuclei in which the chromatin is disposed in the central, slender wavy ribbon like
pattern – caterpillar cells
 MC. CALLUM’S PATCH

Region of endocardial surface in the posterior wall

just above the posterior leaflet of the mitral
valve.
Appears as a map like area of thickened, roughened,
and wrinkled part of endocardium.
It is produced as a result of regurgitant jets.
 SEROFIBRINOUS PERICARDITIS

This causes precordial pain and

pleural rub
Typically called bread and butter
pericarditis
SYMPTOMS Signs
Pulse: tachycardia,
Malaise and tiredness bounding pulse, water-
(early) hammer pulse
Exertional Pedal edema
breathlessness
Raised JVP
Pedal edema
Inspection: bulging
Abdominal pain precordium
Precordial pain Palpation: shifting of
apex with thrills
Percussion: cardiomegaly
AUSCULTATION
Soft S1 due to prolonged P- MR murmur
R interval Pansystolic, best heard at
apex, radiating to axilla and
Loud S3 may give rise to back
gallop rhythm
Carey-Coomb’s murmur
Due to inflamed and Mid-diastolic murmur, heard
thickened mitral valve at apex which occurs due to
Soft delayed diastolic mitral blood flow across diseased
murmur mitral valve
Pericardial rub AR murmur
Early diastolic murmur,
heard best in left
parasternal region
SYDENHAM’S CHOREA (S)
St. Vitus dance
Occurs after a prolonged latent period after group A streptococcal infection
Usually occurs in the absence of any manifestation
Usually occurs in females
Presents as rapid, jerkey, involuntary movements affecting predominantly head, neck
and face.
SYDENHAM’S CHOREA
Primarily a neurological disorder consisting of
Involuntary movements (chorea)
Emotional lability: improper smiles, weird expressions
Incoherent speech
Hypotonia
Weakness of muscles
Hung up reflexes
 TYPICAL SIGNS

Pronator sign
Pronation of hands
when they are above
head Choreic hand (spooning)
• Flexion at wrist
• Extension at MCP joint
Jack-in-the box tongue • Abduction of thumb
On asking the patient to
protrude his tongue, the
tongue flits in and out
 MILK MAID’S GRIP

A sign of generalized weakness

and inability to maintain
tetanic muscle contraction;
i.e. when asked to squeeze
examiners hands they do it in
a milking fashion (motion of
contraction and relaxation)
SYDENHAM’S CHOREA
Associated with excessive emotional lability and personality changes
Self limiting condition, resolves completely within 6 weeks

Factors causing reccurence

1. Pregnancy (chorea gravidarum)
2. Re-infection with streptococcus
3. Use of oral contraceptive pills
 ERYTHEMA MARGINATUM (E)

Almost never involves the face

Characteristic rash of acute RF
Typically occurs on trunk or the proximal
limbs
MARGINATUM: appears as a ring that
extends centrifugally while in the centre
of the ring the skin returns to normal.
Pink to red, non pruritic, commonly in
association with carditis
Rash leaves no induration or scarring
 SUBCUTANEOUS NODULES (N)

Delayed manifestation (after 2-3 weeks)

Typically non tender, commonly in association with
cardiac manifestations
Located along the extensor surfaces of tendons near
bony prominences
Mobile, small in size (<2 cm)
Nodules may last for few weeks (max-3 weeks)
MANAGEMENT
• SYMPTOMATIC

1.BED REST
. Recommended for acute rheumatic fever.
If there is no cardiac involvement then 2-3 weeks rest is
enough.
. If carditis is present then it should be continued for 1-3
months in the presence of congestive failure.
2.DIET
. If there is no cardiac involvement there should be no
restriction in salt intake.
. If congestive cardiac failure is present salt restriction is
necessary.
3. PENICILLIN
• A single injection of Benzathine penicillin can be
administered after diagnosis.
• Penicillin V 250mg 4 times a day for 10 days is another
alternative.
• Erythromycin 250mg 4 times a day for 10 days can be
administered for those with penicillin allergy.
SUPPRESSIVE THERAPY
• ASPIRIN
• STEROIDS
Total duration of the course is 12weeks.
• ASPIRIN
Given at a dose of 90-120 mg/kg/day in 4 divided doses
for 10 weeks and tapered in the next 2 weeks.
• STEROIDS
Prednisolone is given 2mg/kg maximum dose 60mg is
given for 3 weeks and tapered in next 9 weeks.
1.Carditis with congestive cardiac failure is treated with
steroids.

2.Carditis without congestive cardiac failure: either

steroids or aspirin but steroids are preferred.

3.If patient does not have carditis aspirin is used.

Management of chorea
• Mild cases by providing calm environment.
• In severe chorea carbamazepine or sodium
valproate is useful and preferred over
haloperidol. Should be continued for 1-2 weeks
after symptoms subside.
• Intravenous Immunoglobulin can be
recommended in case of severe chorea and
resolution is rapid.
Prevention of Rheumatic Fever
• Primary prevention
1. Primary prophylaxis i.e., timely and complete
treatment of group A streptococcal sore throat
with antibiotics. If started within 9 days of
onset cousre of penicillin will prevent almost all
cases of ARF.
2. In poor countries microbiologic diagnosis of
group A streptococcal pharyngitis is not
available thus all patients with sore throat is
treated with penicillin.
SECONDARY PREVENTION
• Long term penicillin prophylaxis to prevent
recurrences.
• 1.2 million units of benzathine penicillin G once
in every 3 weeks.
• 0.6 million units every alternate week.
• Injection is painful and some patients might get
fever 24 to 36 hours following injection thus
preferable on a weekend to avoid school
absence.
• Oral penicillin V 250mg twice daily as an
alternative or erythromycin 250 mg twice daily
for penicillin allergic patients.
Duration of secondary prophylaxis

• Rheumatic fever without carditis

For 5 years after the last episode or until they are
18 year old(whichever is longer)
• Rheumatic fever with carditis but no valvular
disease.
For 10 years after the last attack or until they are
25 year old.
• Rheumatic fever with persistent valvular
disease
For 10 years after last episode till 40 years of age
or lifelong.