o Rheumatic fever is an immunologically mediated
inflammatory disorder, which occurs as a following to group A
streptococcal pharyngeal infection.
o Multisystem disease affecting connective tissue particularly of
the heart, joints, brain, cutaneous and subcutaneous tissues
o The illness is so named because of its similarity in
presentation to rheumatism.
Rheumatic heart disease
• It’s a crippling disease
• Preventable disease
• More common in developing countries.
• It accounts for 12-65% of hospital admissions related to CVD in
• There has been marked decrease in cases of RF and RHD in places
that have implemented preventive programs.
• RHD is prevalent in range of 5-7/1000 in 5-15 age groups.(IAP 2008)
• Streptococcal infections common in children living in under
privileged conditions and RF accounts for 1-3% of the cases.
• MOST common strains- M types-1,3,5,6,18,29.
• Immune mediated- antigenicity of several GAS
cellular and extracellular epitopes and their
immunologic cross reactivity with cardiac
antigenic epitopes(molecular mimicry)
• M proteins- M1,M5,M6 and M19.
STRETOCOCCAL SORE THROAT
• Tender lymph nodes
• Close contact with infected person
• Scarlet fever rash
• Excoriated nares( crusted lesions) in infants
• Tonsillar exudates in older children
• Latent PERIOD- 2-4 weeks.
• GOLD STANDARD-POSITIVE THROAT CULTURE
• Sudden onset of fever, pallor, malaise, fatigue.
MODIFIED JONES CRITERIA
a) ↑ ESR
b) ↑ CRP
c) Prolonged PR
Supportive evidence of preceding
streptococcal infection(essential except
for diagnosis of chorea)
Anti streptolysin O
ASO titre>333units-children, >250
Anti deoxyribonucleade B
History of (within previous 45 days)
Streptococcal sore throat
Positive throat culture
Positive rapid streptococcal antigen detection
DIAGNOSTIC CATEGORIES CRITERIA
Primary episode of RF 2 major or 1 major and 2 minor
manifestation + evidence of presiding
group A streptococcal infection
Recurrence in a patient without
established heart disease
2 major or 1 major and 2 minor
manifestation + evidence of presiding
group A streptococcal infection
Recurrence in a patient with established
2 minor and supportive evidence of
previous streptococcal throat infection
Insidious onset rheumatic carditis
Other major manifestations or evidence
of group A streptococcal infection not
Chronic valve lesions of RHD Do not require any other criteria to be
diagnosed as having RHD
Most common feature:
• Present in 90% of patients.
• Painful, migratory, short duration, asymmetrical.
• Rheumatic joints- red, hot, swollen, tender
• Usually >5 joints affected and mainly large joints
Knees, ankles, wrists, elbows, shoulders
• Small joints and cervical spine less commonly
• 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
• JACCOUD’S ARTHRITIS:
Chronic post rheumatic fever arthropathy
Deforms the fingers and toes
Deformity – ulnar deviation of fingers, flexion of
MCP joints and hyper extension of PIP joints
No inflammatory changes
Occurs after prolonged and multiple attacks.
Prognosis – good.
• 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
• Valvular damage is the hallmark of RF
• Chronic phase- fibrosis, calcification & stenosis of heart
Any cardiac tissue may be affected
Valvular lesion most common: mitral and aortic
Seldom see isolated pericarditis or myocarditis
• Myocarditis and pericarditis without endocarditis is
never rheumatic carditis- ruleout other causes.
• ACUTE RF- present as tachycardia and cardiac murmur.
• Moderator and severe- cardiomegaly, heart failure with
hepatomegaly, peripheral and pulmonary edema.
• Indicators of recurrence of RF in established heart
1.New murmur/change in pre existing murmur.
2.Pericardial rub(other evidence of pericarditis)
3.Unexplained congestive heart failure(CHF), including
Seen in at least 2 views Seen in at least 2 views
Jet length ≥ 2 cm in at least 1
Jet length ≥ 1cm in at least 1
Peak velocity > 3 meters/second Peak velocity > 3 meters/second
Pan systolic jet in at least 1
Pan diastolic jet in at least 1
• Occur in 5-10% of cases
• Mainly in girls of 1-15 yrs age
• Late manifestation of RF -months after
• Involuntary , jerky choreiform movements,
• Speech affected, fidgety
• Choreiform movements particularly affect the
head(darting movement of tongue)and upper
• Clinical maneuvers:
1. MILKMAID SIGN- irregular contractions and
relaxation of muscle of fingers while
squeezing the examiners finger.
2. Spooning and pronation of hands when
patient’s arm extended.
3. Wormian darting movements of tongue upon
4. Examination of hand writing to evaluate fine
• First sign: difficulty walking, talking, writing
Occurs in 30% of patients with ARF
• Usually benign and resolves in 2 - 3 months
• Disappears leaving no residual damage.
• 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
• 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 - 20% of cases
• Often associated with carditis
• Fever- LR-38.5◦F,MR/ HR- 38◦F
• Arthralgia –LR-Polyarthralgia,MR/HR-Mono
• High ESR
• Anaemia, leucocytosis
• Elevated C-reactive protein
• Prolonged P-R interval(normal range-
Exceptions to Jones Criteria
• Chorea alone, if other causes have been
• Insidious or late-onset carditis with no other
• PHARYNGITIS AND SECONDARY PROPHYLAXIS:
DRUGS DOSE PHARYNGITIS SECONDARY
G(deep IM injection)
0.6 million units
Single dose 21 days
Penicillin V oral Children:250mg QID 10 DAYS Twice a day
Azithromycin(oral) 12.5 mg/kg/day
5 days Not recommended
Cephalexin (oral) 15-20mg/kg/day
10 days Not recommrnded
Erythromycin (oral) 20mg/kg/dose max
Not recommended Twice a day
DURATION OF SECONDARY
• No carditis : 5 years / 18 years of age, whichever is
• Mild to moderate carditis and healed carditis: 10
years / 25 years of age, whichever is longer
• Severe disease or post intervention patients:
Lifelong. One may opt for secondary prophylaxis up
to the age of 40 years
• Inflammation in acute rheumatic fever:
1. Mild carditis+ arthritis
Regime 1- aspirin 100mg/kg/day for 2-3 weeks
divide in 4-5 doses.
Taper to 60- 70mg/kg/day
Regime 2- 50-60mg/kg/day total 12 weeks.
2. Moderate to Severe carditis
Regime 1- Prednisolone 2mg/kg/day till normal ESR. Taper over
2-4 weeks reduce dose by 2.5-5 mg every 3 rd day. Start
aspirin simultaneously to complete total 12 weeks.
Regime 2- Prednisolone – 3-4 weeks taper slowly to cover
total period of 10-12 weeks.
• Chorea – mild – silent environment and
sedatives like oral phenobarbitone or
diazepam. Haloperidol- (0.25-0.5mg/kg/day)
Sodium valproate (15mg/kg/day).
• Congestive heart failure- digoxin, diuretics,
• Recurrence – New episode of rheumatic fever
following another GABHS infection occuring after 8
weeks following stopping treatment.
• Rebound – RF occuring within 4-6 weeks of stopping
treatment or while tappering the dose.
• Relapse – Worsening of RF while under treatment
and often with carditis
• Sub-clinical carditis – Clinical examination normal,
ECHO – abnormal
• Indolent carditis – Present with persistent features
of CHF, murmur and cardiomegaly. No or very few
features of carditis.
• 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
• Rheumatic heart disease is the only truly
preventable chronic heart condition