ACUTE RHEUMATIC FEVER

Dr Shamshad Ahmed Khan

Prof and Head
Department of Paediatrics
Kanachur institute of medical sciences
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 RHEUMATIC FEVER

“Licks the joint, bites the

heart”

2
 INTRODUCTION

• Leading cause of Acquired Heart

disease

• Incidence : Peak 5 – 15 yrs.

3
 ORGANISM

Group‐A beta hemolytic

streptococci

4
 DISEASES CAUSED BY GROUP A
STREPTOCOCCI

• Pharyngitis
• Impetigo/pyoderma
• Pneumonia, Necrotizing fasciitis
• Rheumatic fever
• Glomerulonephritis
• Osteomyelitis
• Scarlet fever & erysipelas
• Toxic shock syndrome
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 RHEUMATIC FEVER

• Non suppurative complications of Group

A streptococcal pharyngitis
• Latent period of 1‐3 weeks
• A delayed immune response caused by
antibody cross reactivity that can involve the
heart, joints, skin, and brain (basal ganglia)
• Serotypes M types (1,3,5,6,18,24)

IAP UG Teaching slides 2015‐ 6

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 INTRODUCTION

• Untreated Group‐A beta hemolytic

streptococcal infection –
is the commonest antecedent event
that precipitates an attack of ARF.

7
 EPIDEMIOLOGY

• Prevalence RHD India ‐ 0.5 %.

• About 50% of children with ARF will suffer

from RHD

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 EPIDEMIOLOGY

• Sex –Both sex Equally Affected

• Depends on Individual
Susceptibility
• Season – Winter
• Predisposing Factors –
1.Low Socioeconomic
status 2.Overcrowding
3.Poor Medical Care
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 DRAMATIC DECLINE IN
DEVELOPED
COUNTRIES

A ‐ Antibiotic coverage has

increased B ‐ Better housing

C ‐ Conditions (economic & health) have

improved D ‐ Decreased bacterial virulence

E ‐ Easy access to medical care

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 CLINICAL FEATURES & DIAGNOSIS

Jones criteria (updated in

1992) 5‐ Major
4‐ Minor
2 majors or
1 major & 2 minors
with
Evidence of (microbiologic or serologic) of
recent Group A beta hemolytic streptococcal
infection

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 DJ
C
“Not meant as a substitute for Judgement by
clinician. Intended guidelines to restrict the
diagnosis to an acceptable clinical group’’

DJC Judgement

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 WHY JONES
CRITERIA

There is no specific lab test in diagnosis of RF

Aim
• Avoid over diagnosis
• Minimize missing an opportunity for the
safety net of secondary prophylaxis
• Avoid over diagnosis in ‘Judgement’

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 MAJORS

• Carditis Mnemonic
• Polyarthritis C2ASE
• Erythema • C – Carditis
marginatum • C – Chorea
• Subcutaneous • A – Arthritis
nodules • S–
• Chorea Subcutaneous
nodules
• E–
Erythema
marginatum 14
 MINORS

Clinical features
• Fever
• Arthralgia (in the absence of
polyarthritis)

Laboratory features
• Elevated acute phase reactants
– Raised ESR, Raised CRP
• Prolonged PR interval

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 Evidence of (microbiologic or serologic) of recent Group
A beta hemolytic streptococcal infection
(Essential criteria)

• Raised or Rising ASO Titers

• Positive Throat Culture for GAS
• Rapid streptococcal antigen test
• Anti‐DNase B, anti‐
hyaluronidase
• H/o Recent Scarlet Fever

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 3 Circumstances – Where ARF
diagnosed without strict adherence to
Jones criteria

• Indolent carditis may be sole

manifestation
• Chorea may be the sole manifestation
• ARF Recurrence may not fulfill the Jones
criteria

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 ARTHRITIS

• Most Common – 30 to 50%

• Large joint involvement
• Migratory Polyarthritis
• Dramatic response to Aspirin
• Sacroiliac, Temporo‐mandibular and Cervical joints
not involved
• No Permanent Sequelae
• Resolves in Six weeks

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 CARDITIS
PRESENTATION

• Tachycardia out of proportion to

fever
• Sleeping pulse rate raised.

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 CARDITI
S
• 50 ‐60%
• Usually Pancarditis
• Pericarditis never occurs in isolation
• Variable Severity
• Clinical Signs
– Pericarditis – Effusion , Rub,
Pain
– Myocarditis – Tachycardia,
Arrhythmia, cardiomegaly, failure
– Endocarditis – Murmurs 
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 MURMUR

• High pitched apical holosystolic murmur radiating to

axilla

– – Mitral regurgitation.

• An apical mid diastolic murmur.

• High pitched decrescendo diastolic murmur‐ upper

sternal border

– ‐ Aortic regurgitation.
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 CARDITIS SEQUELAE (CHRONIC)

Mitral insufficiency
• Some loss of valvular substance
• Shortening & thickening of Chordae
tendinae

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 CARDITIS
SEQUALAE

Mitral stenosis
• Takes longer duration to develop after an attack of
ARF
• Fibrosis of mitral ring, commissural adhesions
• Contracture of the valve leaflets, chordae &
papillary muscles
• Opening snap, low pitched, rumbling mitral diastolic
murmur with pre systolic accentuation ending in
loud first sound
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 CARDITIS SEQUELAE (CHRONIC) –
CONTD..

Aortic insufficiency

• Sclerosis of aortic valve‐ distortion & retraction

of the cup
• Characteristic early diastolic murmur
• An apical pre systolic murmur ( Austin flint)

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 SYDENHAM’S
CHOREA

• 10 – 15 % of patients

• Usually delayed/often sole manifestation of

ARF

• Involuntary movements of the face and

limbs,

– Muscle weakness

– Disturbances of speech and gait,

– Poor scholastic performance 25

 SYDENHAM’S
CHOREA
• Milkmaid's grip
– irregular contractions of the muscles of the
hands while squeezing the examiner's fingers
• Spooning and pronation of the hands when
the patient's arms are extended(St. Vitus
Dance)
• Wormian movements of the tongue upon
protrusion (Jack in the Box)
• Handwriting to evaluate fine motor movements

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27
 SYDENHAM’S
CHOREA

• Facial grimacing

• Emotional liability

• Exacerbated by stress

• Disappear at sleep

• Rarely leads to permanent neurological

sequelae

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 CHOREA

• Seen more in females

• Minimum 3 months after sore
throat

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 ERYTHEMA MARGINATUM

• Occurs < 10% of Patients

• Macular non pruritic rash
• Serpiginous border, raised edges, central
clearing
• Most common on trunk
• Never seen on face
• Evanescent, warmth accentuates lesion
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ERYTHEMA MARGINATUM

31
ERYTHEMA MARGINATUM

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Erythema marginatum on the trunk, showing erythematous
lesions with pale centers and rounded or serpiginous
margins 33
Closer view of erythema marginatum in the same
patient 34
 SUBCUTANEOUS
NODULES
• Seen in around 5 %

• Small, pea sized, 0.5 to 2 cms in diameter

• Firm, mobile, PAINLESS

• Seen over the extensor surface of WRIST, ELBOW,

SPINE

• Usually seen in individuals with long standing carditis

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SUBCUTANEOUS
NODULE

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Subcutaneous nodule on the extensor surface of elbow of
a patient with acute rheumatic fever

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 LINK OF CARDITIS
WITH
Polyarthritis - 50 - 75 %
SC Nodule - > 95 %
Chorea - 60 - 75 %

HALF ARE
INAPPARENT
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 DIFFERENTIAL
DIAGNOSIS
• Arthritis‐ Rheumatoid arthritis (JRA)
‐SLE
‐ Reactive arthritis –
Shigella, Salmenolosis, Yersenia
‐ Lyme’s disease
‐ Chikungunya
• Carditis‐ viral myocarditis,&
Pericarditis Infective endocarditis
Congenital heart lesions
• Chorea ‐ Huntington chorea
Wilson
disease Tics
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 INVESTIGATIONS

• Tests reflecting tissue inflammation

TC, DC, ESR, CRP (acute phase reactants)

• Tests to Prove STREP Infection

– ASO Titer, AntiDNAase, Streptozyme, Anti
Streptokinase
– Throat Culture

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 INVESTIGATION

• X ray chest
• ECG
• ECHO
• Doppler
• Blood culture
• Catheterization
studies

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Chest radiograph of an 8 year old patient with acute
carditis before treatment
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Chest radiograph of an 8 year old patient with acute
carditis after treatment
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44
 MANAGEMENT

• Bed rest
• Eradication of Streptococci
• Anti inflammatory therapy
• Treatment of CCF
• Treatment of Chorea
• Prevention of Recurrences
• Surgical – Acute and
Chronic
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 ARTHRITIS

• Bed rest
• Aspirin only for 4‐6
Weeks
• Local measures

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 CARDITI
S
• Carditis alone ‐ ASPIRIN Only

• Carditis with mild Cardiomegaly ‐ ASPIRIN Only

• Carditis in failure ‐ASPIRIN with STEROIDS and

decongestive measures
• Bedrest
• Low sodium diet

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 SYDENHAM’S CHOREA ‐
TREATMENT

• Anti inflammatory agents usually not required

• Phenobarbitol 15‐30 mg tid or qid oral ‐ drug of

choice

• Haloperidol 0.01‐ 0.03mg/kg/24hrs bd oral

• Chlorpromazine 0.5mg/kg every 4‐6 hrs. oral

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 PREVENTION IN
RF

Primordial Preventing Strep Throat ‐ vaccine ?

Primary Treating Strep Throat

infection
Secondary Preventing Rheumatic
recurrence by
chemoprophylaxis
Tertiary Treating
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RHD
 PRIMARY PROPHYLAXIS

Treating Streptococcal sore throat with

Antibiotics
1. Oral Penicillin
2. Procaine Penicillin IM
3. Benzathine Penicillin
4. Oral Erythromycin

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 PRIMARY
PROPHYLAXIS
Vulnerable children from 5 to 15 yrs. with pharyngitis

Oral
• Penicillin 250 ‐500mg bd/tds 10 days
• Erythromycin 20‐ 40 mg/kg/day tds/qid‐ 10 days
• First generation Cephalosporin‐ 10 days
• Azithromycin 12mg/kg/day single dose – 5days
max‐500mg/day

Parenteral
• < than 27kg single dose IM Benzathine penicillin 6,00,000 U
• > than 27kg single dose IM Benzathine penicillin 1,20,0000 U

Therapy instituted before 9th day of symptoms of acute

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Pharyngitis
 SECONDARY
PROPHYLAXIS

• To prevent recurrences
– Benzathine Penicillin once in 3 weeks
IM
– Oral Penicillin daily
– Erythromycin daily
– Sulfadiazine daily

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 SECONDARY
PROPHYLAXIS

Route Antibiotic Dose Frequenc

y
IM Benzathine 1200000 Every 3rd
Penicillin U wk.
Oral Penicillin V 250mg BD daily

Oral Erythromycin 250mg BD daily

Oral Sulphadiazine 500mg to BD daily

1000mg 53
 HOW LONG TO
GIVE
RF;No 5 years from last
Carditis Episode or till 21 years
RF; Carditis ;
No residual 10 years from last episode
RHD ; or till 25 years
RF Carditis; 10 years from last episode
RHD ; or till 40 years / lifelong

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 NEW MODES OF
TREATMENT

? IVIG
? ? Valproate for chorea
? Anti‐ cytokines ‐ adjuvants
? ? Other NSAIDS

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THANK
YOU

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