2. What is Acute Rheumatic Fever?
It is a multisystem disorder which occurs as a
sequel to infections caused by group A
streptococci.
It principally involves heart, joints , skin and
subcutaneous tissues.
This is the commonest cause of acquired heart
disease in childhood and adolescence.
3. Epidemiology
Most commonly affects children between age
groups of 5 to 15 years.
Recent studies have shown that 33.4M people
worldwide are suffering from ARF. Apprx 2-3M
people die because of ARF.
The incidences are very much high in developing
countries.
It is a disease of poverty, poor hygiene and
overcrowding.
No gender association. Both sex are equally
affected
4. Etiopathogenesis
• Group A streptococcus causing strep throat and scarlet fever. It usually takes
1-5 weeks for rheumatic fever to develop.
• The condition is triggered by an immune – mediated delayed response to
infection with specific strains of group A which have antigens that may cross
react with cardiac myosin and sarcolemmal membrane protein.
• The antibodies produced against streptococcal antigens cause inflammation
in endocardium, myocardium and pericardium as well as joints and skin.
5. Molecular Mimicry – Antibodies cross react with Ag present in
heart. Because of Ag-Ab reaction ,there is activation of adhesive
molecule VCAM – I (Vascular Cell Adhesion Molecule – I) resulting
in activation of leucocytes and also lysis of endothelial cell.
This results in release of proteins Laminin, Keratin and tropomyosin
resulting in activation of T-cells.
6. Clinical Features
Presents with fever, anorexia, lethargy and joint pain 2
to 3 weeks after an episode of streptococcal pharyngitis.
The diagnosis is made using Jones Criteria
7. Carditis
There is involvement of pericardium, endocardium and
myocardium.
Occurs in 40-60% of patients with ARF.
Manifests as breathlessness, palpitations, chest pain,
tachycardia, cardiac enlargement, mid-diastolic murmur(Carey
Coombs’ murmur).
The mitral valve is most commonly involved and the aortic
valve is next most affected.
8. Arthritis
Most common and major manifestation of ARF occurring
in 75% patients.
Tends to occur early when streptococcal antibody titers
are high.
There is painful inflammatory involvement of large
joints(knee, elbow, ankles) which are red, swollen and
tender.
Polyarthritis
– when more
than 4 joints
are involved
9. Chorea (Sydenham’s chorea)
Late neurological manifestations when all other signs have
disappeared.
It is characterized by involuntary purposeless movements of
hands, feet and face.
More common in females
Apprx one- quarter of affected patients develop chronic
rheumatic valve disease.
10. Subcutaneous Nodules
Occurs in less than 10% cases of ARF.
Nodules are 0.5 – 2 cm, painless, firm found over
extensor surface of joints.
Its presence signifies underlying rheumatic heart disease.
11. Erythema Marginatum
Occurs in less than 10% of patients.
Lesions starts as red macules that fade in centre but
remain red at edges and occur mainly on trunk and
proximal extremities.
12. Investigations
• Blood examination reveals leukocytosis, raised ESR and CRP.
• Throat swab culture (obtained in 10-25% patients).
• Anti – deoxyribonuclease B and anti hyaluronidase may be positive.
• ASO titer is raised to >200 units in adults or >300 units in children.
• Chest X rays – cardiomegaly, pulmonary congestion.
• ECG – features of pericarditis, first degree AV block, T-wave inversion.
• Echocardiography – cardiac dilatation and valve abnormalities.
Cardiomegaly
13. Treatment
1. Benzyl Penicillin (1.2M units) or oral
phenoxymethylpenicillin to eliminate any residual
streptococcal infection.
2. Penicillin – allergic – Erythromycin and cephalosporin
3. Bed Rest is important as it lessens joint pain and reduce
cardiac overload.
4. For arthritis, Aspirin (60mg/kg) is given to relieve the
symptoms.
5. Patients of sever carditis or severe arthritis are given
corticosteroids – Prednisolone (1-2mg/kg).
14. Secondary Prevention
It is used to prevent subsequent pharyngeal
infection and long term prophylaxis with
Benzathine Penicillin (1.2M units) is given.
Sulfadiazine and Erythromycin are given if
patient is allergic to penicillin.
Secondary prophylaxis is given life-long in
cases of Aortic and Mitral valve replacement.
