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Dr. Deepak K. Gupta
ACUTE RHEUMATIC FEVER
Acute rheumatic fever
• Usually affects children (most commonly between 5
and 15 years) or young adults
• endemic in parts of Asia, Africa and
• Triggered by an immune-mediated delayed response
to infection with specific strains of group A
– Antigens that may cross-react with cardiac myosin and
sarcolemmal membrane protein
• Antibodies produced against the streptococcal
– inflammation in the endocardium, myocardium and
pericardium, as well as the joints and skin.
• Multisystem disorder that usually presents with
– Joint pain
• Latent period: 2–3 weeks after an episode of
• Diagnosis is made using the revised Duckett Jones
– 2 or more major manifestations,
– Or 1 major and 2 or more minor manifestations,
– along with evidence of preceding streptococcal infection
• Involves the endocardium, myocardium and
pericardium to varying degrees
• Incidence declines with increasing age - ranging from
90% at 3 years to around 30% in adolescence.
• It manifest as
– Breathlessness - heart failure or pericardial effusion
– Palpitations or chest pain - pericarditis or pancarditis
– Other features include tachycardia, cardiac enlargement
and new or changed cardiac murmurs.
– Aortic regurgitation - 50% of cases but the tricuspid and
pulmonary valves are rarely involved.
– Syncope: Conduction defects
• ECG changes: ST and T wave changes
• Most common and early
• Acute painful asymmetric
and migratory inflammation
of the large joints
• Typically affects the knees,
ankles, elbows and wrists.
• Pain characteristically
responds to aspirin
• If not, the diagnosis is in
• Erythema marginatum
– occurs in < 5% of patients
– lesions start as red macules (blotches)
that fade in the
– but remain red at the edges
– occur mainly on the trunk and proximal
extremities but not the face
• Subcutaneous nodules
– occur in 5–7% of patients
– Small (0.5–2.0 cm), firm and painless
– Best felt over extensor surfaces of bone
– typically appear more than 3 weeks
after - help to confirm rather than make
(St Vitus dance)
• Late neurological manifestation
• Appears at least 3 months after the
episode of ARF
• all the other signs may have
• Occurs in up to 1/3rd of cases and is
more common in females
• Emotional breakdown or changes may
be the first feature.
• Typically followed by purposeless
involuntary choreiform movements of
the hands, feet or face.
• Speech may be explosive and halting.
• Spontaneous recovery usually occurs
within a few months
• ESR and CRP: monitoring progress of the
• Positive throat swab cultures are obtained in
only 10–25% of case.
– Mitral regurgitation with dilatation of the mitral
– Prolapse of the anterior mitral leaflet
– May also show aortic regurgitation and pericardial
CXR: cardiomegaly due to
• Treatment strategies can be divided into management
– acute attack,
– management of the current infection
– prevention of further infection and attacks.
• Management of the acute attack
– Single dose of benzyl penicillin 1.2 million U i.m.
– Oral phenoxymethylpenicillin 250 mg 6-hourly for 10 days
– Penicillin-allergic: erythromycin or a cephalosporin
– Analgesia: optimally achieved with high doses of
• Treatment is then directed towards limiting cardiac
damage and relieving symptoms.
• Bed rest and supportive therapy
– Lessens joint pain and reduces cardiac workload.
– Duration should be guided by symptoms along
with temperature, leucocyte count and ESR
– Should be continued until these have settled.
– Return to normal physical activity but strenuous
exercise should be avoided in those who have
• Cardiac failure
• Mild heart failure usually responds to rest and
• Severe carditis: Digoxin, but its use should be monitored
closely - AV block
• Vasodilators and diuretics also may be used
• If heart failure in these cases does not respond
to medical treatment, valve replacement may be
• Protracted Sydenham chorea has responded
• It requires long-term antimicrobial
prophylaxis, even if no other manifestations of
rheumatic fever evolve.
• Complete physical and mental rest is essential
because the manifestations of chorea may be
exaggerated by emotional trauma.
– relieve the symptoms of arthritis rapidly and a response
within 24 hours helps to confirm
– Reasonable starting dose is 60 mg/kg body weight/day,
divided into six doses.
– In adults, 100 mg/kg per day may be needed up to the
limits of tolerance or a maximum of 8 g per day.
– should be continued until the ESR has fallen and then
gradually tailed off.
– more rapid symptomatic relief than aspirin and are
indicated in cases with carditis or severe arthritis.
– Prednisolone, 1.0–2.0 mg/kg per day in divided doses,
should be continued until the ESR is normal then tailed off.
PRIMARY-10 days course
of penicillin therapy;
about 30% of patients with
acute rheumatic fever do
not recall a preceding
episode of pharyngitis
prevention is directed at
preventing acute GABHS
pharyngitis in patients at
substantial risk of
recurrent acute rheumatic
• It involves eradication of Streptococcus from
• Administering a single i.m. benzathine
• Patients are susceptible to further attacks if another
streptococcal infection occurs.
• Long term prophylaxis with penicillin should be given
– Benzathine penicillin 1.2 million U i.m. monthly
– Oral phenoxymethylpenicillin 250 mg 12-hourly
• Sulphonamides prevent infection but are not effective
in the eradication of group A streptococci.
• Long-term antibiotic prophylaxis prevents another
attack of ARF but does not protect against infective
Secondary Prevention: Duration
Rheumatic fever without carditis At least for 5 yr or until age
21 year, whichever is longer
Rheumatic fever with carditis but
without residual heart disease (no
At least for 10 yr or well
into adulthood, whichever is
Rheumatic fever with carditis &
residual heart disease (persistent
At least 10 yr since last
episode & at least until age
40 yr; sometime lifelong