ACUTE RHEUMATIC FEVER
RHEUMATIC FEVER is an inflammatory disease that follows pharyngeal
infection by specific strains of group A beta haemolytic streptococci (serotypes
1,3,5,6,18 etc). The major organs and tissues affected are the heart, joints, skin
and central nervous system. In the heart, this inflammation tends to cause
permanent damage. In the Indian sub-continent, rheumatic fever remains
significant health problem and the most important cause of valvular heart
disease, whereas in the developed world the incidence of rheumatic fever and
therefore rheumatic valvular heart disease has declined progressively to fairly
low levels. Contributory factors to this decline have been better socio economic
conditions, early access to medical care, use of antibiotics and decreased
ETIOLOGY: Following pharyngitis due to group A beta haemolytic
streptococci , about 1-3 % of untreated cases develop rheumatic fever 3 to 4
weeks later. The attack rate may be higher in epidemics of streptococcal
pharyngitis. The precise pathogenic role of the streptococcus is uncertain but is
likely to be an immunological process as evidenced by latency, demonstration
of gamma globulins in myocardial sarcolemma and circulating heart reactive
anti bodies in cases with rheumatic carditis. It is postulated that an antibody to
the carbohydrate call wall cross reacts with protein in valve tissue (antigenic
mimicry) and may cause permanent damage to the heart valves. Similar cross
reactivity with connective tissue in joints and with caudate nucleus may lead to
arthritis and chorea respectively.
EPIDEMIOLOGY: In developed countries the prevalence of rheumatic fever is
less than one per thousand rising to 1% of school going children in the
developing world. Most initial attacks occur between the ages of 5 to 15 years,
but about 20% of cases occur in adults. Female to male ratio is 1:1.
Contributory factors include overcrowding, poor socioeconomic status – these
encourage the spread of streptococcal infection –malnutrition and poor access to
health care. Host susceptibility also appears to play a role as there is a high
incidence of recurrence of rheumatic fever. Recurrent attacks may occur even in
patients in the age group 40-50 years. The disease may tend to occur in families
suggesting a genetic predisposition in a few patients.
Pancarditis. All 3 layers of the heart are affected.
Pericarditis is usually transient and may produce a fibrinous effusion but
cardiac tamponade and constriction are uncommon.
Myocarditis is characterised by presence of Aschoff bodies which are
perivascular foci of eousinophilic interstitial collagen surrounded by
lymphocytes, plasma cells, macrophages and fibroblasts. These persist long
after the acute illness has subsided.
Endocarditis is marked by inflammation and edema of the valve cusps
associated with verrucous nodules which develop along the lines of valve
closure. Acutely, this may cause severe valve damage but more commonly the
valve function remains little affected during this phase of illness. During healing
however, progressive scarring of the valve apparatus with retraction and fusion
can occur. If adhesion and fusion are predominant, stenosis of the valve results.
If fibrosis and retraction (shrinkage) take place, regurgitation occurs. A
combination of the two processes may also occur. Endocarditis most commonly
affects the mitral valve – 60 to 70%, aortic valve in about 25% and less
commonly the tricuspid valve in less than 10%. The pulmonary valve is rarely
affected by rheumatic fever.
Non cardiac tissues
Arthritis – there is an exudative synovitis but no long term damage to joints
Subcutaneous nodules – these are granulomatous lesions which disappear
following the acute illness.
Chorea – the cerebral pathology has not been identified but may involve the
CLINICAL MANIFESTATIONS: Rheumatic fever is a systemic illness which
occurs 2 to 3 weeks after an attack of acute pharyngitis ( ‘sore throat’) . The
onset is with fever, anorexia, lethargy, and joint pains. Abdominal pain and
epistaxis may sometimes occur. In some children fever and arthralgia may be
the only symptoms and maybe trivial enough to be forgotten by the patient and
Polyarthritis – a migratory polyarthritis is seen in approximately 75% of
patients. And is characterised by severe and tenderness in the affected joint. The
arthritis is usually asymmetrical involving the large joints of the extremities –
the ankle, the knee, the wrist, and elbow. Sometimes the spine, the temporo-
mandibular and costo-clavicular are affected. Involvement of the small joints of
hands or feet and of the hip is unusual.
The arthritis is characteristically ‘migratory’ or ‘flitting’ in which as one joint
improves another gets involved. Some may have only arthralgia. The duration
of arthritis is less than 4 weeks at each site. And there is no residual disability
(Rarely, recurrent attacks of rheumatic fever may result in a permanent
disability involving ulnar deviation of the fingers and lateral deviation of the
toes. This is termed Jaccoud’s arthritis.)
Carditis – this is the most important manifestation of rheumatic fever affecting
about 50% of all patients with first attack of rheumatic fever. Pancarditis occurs
characterised by :
Percarditis with central chest pain - A pericardial rub may be heard . Pericardial
effusion may occur. The ECG may show concave upwards ( saddle –shaped) ST
Myocarditis - clinical features include breathlessness, tachycardia, cardiac
enlargement, soft S1 and presence of S3 and frank evidence of cardiac failure.
ECG may show conduction defects (prolonged PR interval or second degree
heart block), chest x-ray shows pulmonary congestion.
Endocarditis – causes valvulitis which may result in cardiac murmurs. The
following murmurs should be looked for:
Pansystolic murmur at the apex
Mid diastolic murmur at the apex (Carey Coombs murmur)
Early diastolic murmur at aortic area or third left interspace parasternally.
Chorea (Sydenhams chorea or St. Vitus’dance) – this is a late manifestation of
rheumatic fever and may occur up to 1 to 6 months after a n attack of
pharyngitis. It may be seen in 10 to 30% of patients with rheumatic fever and is
commoner in girls. It may be followed by chronic cardiac disease. The child
may show emotional lability, depression, uncharacteristic behaviour,
restlessness and decreased school performance. The chorea may manifest
uniaterally or bilaterally and consist of irregular, jerky, non repetitive, brief,
semi- purposive movements of the extremities, face or trunk. Facial grimacing
may occur. Chorea may last for 3 to 4 months and then subside. Occasionally it
may last longer.
The inability to sustain a given posture leads to the demonstration of certain
Pronator sign – if the hands are raised above the shoulder level, they tend
to assume an attitude of pronation.
Milkmaid’s grip – on squeezing the examiner’s fingers, irregular
contractions result in a milking movement.
Jack-in-the-box tongue – rapid protusion and withdrawal of the tongue.
Piano-playing sign - on extending the arms irregular movements of the
fingers are observed.
Spooning sign- on extension of the fore arm and hand, hyperextension of
the fingers is observed.
Subcutaneous nodules – these are small (less than 0.5cms), firm, painless,
mobile nodules seen in about 2 to 10% of cases and associated with more severe
carditis. They may be found over the extensor tendons of the hands, feet, knees,
elbows, spinal column, sub-occipital region and tendo Achilles. They usually
subside within 1 to 2 weeks.
Erythema marginatum – this is a evanescent, red, macular rash with a
serpiginous outline and a clear centre usually seen over the trunk, thighs and the
arms. It tends to fade and reappear. It is reported in 10 -20% of cases but
observed in less than 5% of patients in our population.
Other manifestations- these include fever upto 38degrees C, pleurisy, pleural
Diagnosis of acute rheumatic fever - there is no diagnostic test for rheumatic
fever which tends to be over diagnosed in children with fever, arthralgia and
soft ejection murmurs, all of which may occur in a viral fever. This can lead to
expensive investigations, needless prophylaxis and prolonged follow up.
Duckett Jones laid down the criteria for diagnosis of rheumatic fever in 1994.
These have been subsequently modified by AHA in 1992 and revised in2001.
The modified Jones criteria are:
Major Manifestations Minor Manifestations
Sub cutaneous nodules
Polyarthralgia (if polyarthritis is
not a major criteria)
Raised ESR or C reactive
Prolonged PR interval on
ECG(if carditis is not a major
Essential Criteria : Supporting evidence of preceding recent streptococcal
infection ie positive throat culture or raised anti streptococcal antibody titres –
ASO, AHT, and ADN ase B.
Diagnosis of rheumatic fever requires :
Two major manifestations plus or essential criteria
One major and two minor manifestations plus essential criteria
Jones criteria have a specificity of about 97% and sensitivity of 77%.
MANAGEMENT OF ACUTE RHEUMATIC FEVER
There is no specific treatment which cures or changes the course of illness.
Treatment is aimed at limiting cardiac damage, relieving symptoms and
eliminating any residual streptococcal infection.
Bed rest and supportive therapy- bed rest is advised during the acute
phase of the illness or during active carditis and is continued till the
temperature, leucocytosis, ESR and CRP come down. If carditis is
present, bed rest is continued for 2 – 6 weeks after ESR and temperature
have returned to normal. In such cases the total period of bed rest may be
upto 3 months.
Salicylates – these are highly effective for fever and arthritis. Aspirin is
given in a dose of 60 – 70 mg per kg body weight per day in 4-6 divided
doses. Toxic effects include head ache, tinnitus, deafness, vomiting,
hyperventilation and metabolic acidosis. Where possible, salicylate level
should be monitored. Salicylates are continued till ESR and CRP have
fallen to normal – usually 4 – 6 weeks – and then tapered gradually to
prevent a rebound. When carditis is present, aspirin may be required for
a longer duration.
Corticosteroids – in patients who cannot tolerate salicylates, or in moderate
to severe active carditis with congestive heart failure, corticosteroids are
recommended. But there is no evidence that they are more beneficial than
salicylates nor do they have a major impact on sequelae. Prednisilone is
given in dose 1-2 mg per kg body weight daily till ESR is normal and then
gradually tailed off. Salicylates are added while tapering steroids to prevent
Antistreptococcal therapy – this is given to eradicate streptococci and to
prevent recurrence (secondary prophylaxis). Inj Benzathine Penicillin – 1.2
million units is administered intramuscularly on diagnosis followed by oral
Phenoxymethyl Penicillin 500mg tid for 10 days. Subsequently, inj
Benzathine Penicillin is given once in 3 weeks. In patients allergic to
Penicillin, Erythromycin may be used.
Chorea – this self limiting condition may respond to Diazepam, oral
Haloperidol or Valproate.
Myocarditis with cardiac failure – responds to bed rest with salt restriction,
diuretics and ACE inhibitors.
Acute rheumatic fever subsides within 6 weeks to 3 months. Carditis occurs
within 2 weeks of onset of arthritis and is more common in children. There is a
1 to 10% mortality in the acute phase. Recurrent attacks of rheumatic fever can
occur.Overall, 50% of patients develop chronic valvular heart disease.
To prevent recurrences, prophylactic antibiotic therapy should be continued for
the period indicated below:
Rheumatic fever without carditis - for 5 years or till the age of 21,
whichever is later.
Rheumatic fever with carditis but no residual valvular lesion – for 10
years or till the age of 25 years , whichever is later.
Rheumatic fever with carditis and residual valvular lesion – life-long
prophylaxis is indicated. However, in most patients, prophylaxis is
discontinued by age of 40years, since the chance of recurrence is low
after that age.