Rheumatic Fever

Dr Sachin G Pai
Asst Professor
Dept of Medicine
Acute rheumatic fever (ARF)
 Most common cause of acquired heart
disease in childhood and adolescence.
 usually affects children (most commonly
between 5 and 15 years) or young adults.
 It is endemic in parts of Asia, Africa and
South America.
Definition
 Rheumatic = pertaining to metabolic
derangement of connective tissue
structures (especially joints and related
structures)
* Dorland’s Dictionary
 Rheumatic fever “licks the joint and bites
the heart”
Pathogenesis
 Triggered by an immune-mediated delayed
response to infection against specific strains of
group A streptococci
 Antigens of these strains may cross-react with
cardiac myosin and sarcolemmal membrane
protein.
 Antibodies produced against the streptococcal
antigens mediate inflammation in the
endocardium, myocardium and pericardium as
well as the joints and skin.
Pathology
 Fibrinoid degeneration is seen in the collagen of
connective tissues
 Aschoff nodules are pathognomonic and occur
only in the heart (composed of multinucleated
giant cells surrounded by macrophages and T
lymphocytes )
 Aschoff nodules are not seen until subacute and
chronic phase of rheumatic carditis emerges.
Clinical features – Jones Criteria

Major manifestations
 Carditis
 Polyarthritis
 Chorea
 Erythema marginatum
 Subcutaneous nodules
Clinical features – Jones Criteria

Minor manifestations
 Fever
 Arthralgia
 Previous rheumatic fever
 Raised ESR or CRP
 Leucocytosis
 First-degree AV block
Clinical features – Jones Criteria
 two or more major manifestations, or one major
and two or more minor manifestations is
required for diagnosis.
 plus
Supporting evidence of preceding streptococcal
infection:
 recent scarlet fever,
 raised antistreptolysin 0 or other streptococcal antibody titre,
 positive throat culture
Carditis
 'pancarditis' that involves the endocardium, myocardium
and pericardium.
 manifests as
 breathlessness (due to heart failure or pericardial effusion),
 palpitations
 chest pain (usually due to pericarditis or pancarditis).
 tachycardia, cardiac enlargement
 new or changed cardiac murmurs.
 soft systolic murmur due to mitral regurgitation
 soft mid-diastolic murmur (the Carey Coombs murmur) is typically
due to valvulitis,
carditis
 Pericarditis may cause chest pain, a pericardial
friction rub and precordial tenderness.
 Cardiac failure may be due to myocardial
dysfunction and/or mitral or aortic regurgitation.
 ECG changes are common and include ST and
T wave changes; conduction defects sometimes
occur and may cause syncope.
Arthritis
 Usually an early feature that tends to occur when
streptococcal antibody titres are high.
 most common major manifestation and is characterised
by acute, painful, asymmetric and migratory
inflammation of the large joints (typically the knees,
ankles, elbows and wrists).
 The joints are involved in quick succession and are
usually red, swollen and tender for between a day and
up to 4 weeks. The pain characteristically responds to
aspirin; if it does not, the diagnosis is in doubt.
Skin lesions
 Erythema marginatum occurs in less than
5% of patients.
 Lesions start as red macules (blotches)
which fade in the centre but remain red at
the edges and occur mainly on the trunk
and proximal extremities but not the face.
 The resulting red rings or 'margins' may
coalesce or overlap
Subcutaneous nodules
 Occur in 5-7% of patients.
 They are small (0.5-2.0 cm), firm and painless,
and are best felt over extensor surfaces of bone
or tendons.
 Nodules typically appear more than 3 weeks
after the onset of other manifestations and are
therefore a feature that helps to confirm rather
than make the diagnosis.
Sydenham's chorea (St Vitus
dance)
 late neurological manifestation
 appears at least 3 months after the episode of
ARF.
 Emotional lability may be the first feature and is
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.
Investigations
Evidence of a systemic illness
 Leucocytosis, raised ESR, raised CR

Evidence of preceding streptococcal infection

Evidence of carditis
 Chest X-ray: cardiomegaly; pulmonary congestion
 ECG: first- and rarely second-degree heart block; features of
pericarditis; T-wave inversion; reduction in QRS voltages
 Echocardiography: cardiac dilatation and valve abnormalities
Treatment of the acute attack
 A single dose of benzyl penicillin 1.2 million U
i.m. or oral phenoxymethylpenicillin 250 mg 6-
hourly for 10 days should be given on diagnosis
to eliminate any residual streptococcal infection.
If the patient is penicillin-allergic, erythromycin or
a cephalosporin can be used. Treatment is then
directed towards limiting cardiac damage and
relieving symptoms.
Supportive therapy
 Bed rest till symptoms and markers of
inflammation have settled.
 Cardiac failure should be treated as
necessary
 Valve replacement may be necessary if
severe regurgitant lesions
 Pacemaker insertion is rarely needed.
Aspirin
 prompt response to aspirin (within 24 hours)
helps to confirm the diagnosis
 Reasonable starting dose is 60 mg/kg body
weight per day.
 toxic effects include nausea, tinnitus and
deafness
 more serious ones are vomiting, tachypnoea
and acidosis
Corticosteroids
 Produce 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
 There is no evidence that long-term
steroids are beneficial
Secondary prevention
 Patients with history of rheumatic fever are
susceptible to additional attacks.
 Will proceed to chronic rheumatic heart
disease if it goes unchecked
 Severe valvular deformities and stenotic
and regurgitant lesions are caused.
Secondary prevention
 Benzyl penicillin 1.2 million U i.m. monthly
 oral phenoxymethylpenicillin 250 mg 12-
hourly
 Sulfadiazine or erythromycin may be used
if the patient is allergic to penicillin
Thank you