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ACUTE RHEUMATIC
FEVER
By – Angel Kanda
Roll Number - 5
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.
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
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.
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.
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
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.
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
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.
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.
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.
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
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).
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.
Acute rheumatic fever/ Rheumatic Fever

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Acute rheumatic fever/ Rheumatic Fever

  • 1. ACUTE RHEUMATIC FEVER By – Angel Kanda Roll Number - 5
  • 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.