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Basic information regarding Rheumatic Fever, clinical features, investigation and Treatment.


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RHEUMATIC FEVER Dr.Sayeed Uddin Helal MS (Neurosurgery), MPH, MBBS, CCD(Diabetology) Ex-Registrar, SCI Unit-Medical Services, Centre for the Rehabilitation of the Paralysed (CRP)


INCIDENCE Acute rheumatic fever (ARF) usually affects children (5-15 years) or young adult. It remains endemic in parts of Asia, Africa and South America. Incidence : 100 per 100,000. Most common cause of acquired heart disease in childhood and adolescence.


PATHOGENESIS The condition is triggered by an immune-mediated delayed response to infection with specific strains of group A streptococci that possess antigens which may cross-react with cardiac myosin and sacrolemmal membrane protein. Histologically , fibrinoid degeneration is seen in the collagen of connective tissues.


CLINICAL FEATURE Multisystem disorder. Follows an episode of streptococcal pharyngitis (2-3 weeks later). Fever, anorexia, lethargy and joint pain. Arthritis in 75% cases. Other features: Rash, carditis, neurological change.


REVISED JONES CRITERIA Major manifestations: 1. Carditis 3. Erythema marginatum 5. Chorea 2. Polyarthritis 4. Subcutaneous nodules Minor manifestations: 1. Fever 4. Raised ESR or CRP 2. Arthralgia 5. Leucocytosis 3, Previous rheumatic fever 6. First degree AV block PLUS Supporting evidence of preceding streptococcal infection: recent scarlet fever, raised ASO titre or other streptococcal antibody titre, positive throat culture. N.B. Evidence of recent streptococcal infection is particularly important if there is only one major manifestation.


DIAGNOSIS According to the revised Jones criteria: Two or more major manifestations OR One major and two or more minor manifestations with Evidence of proceeding streptococcal infection


KEY POINTS FOR Dx Only 25% will have a positive culture for group A streptococcus at the time of diagnosis. Isolated serological evidence of recent streptococcal infection or raised ASO titre has no significance in diagnosis. In case of isolated Chorea or Pancarditis, ARF can be diagnosed without any evidence of preceding streptococcal infection.


INVESTIGATION Evidence of systemic illness (non-specific) Leucocytosis, raised ESR, raised CRP Evidence of preceding streptococcal infection ( specific) Throat swab culture: group A β - haemolytic streptococcai (also from family memebers and contacts) Antistreptolysin O ntibodies (ASO titres ) : Rising titres , or levels of >200 U(adults) or >300 U (children) Evidence of carditis CXR : cardiomegaly , pulmonary congestion ECG : first-(rarely second-) degree heart block; features of pericarditis ; T-wave inversion; reduction in QRS voltages. Echocardiography : cardiac dilatation and valve abnormalities.


KEY POINTS FOR INVESTIGATION ESR & CRP: non-specific markers for systemic inflammation, useful for monitoring progress of the disease. Positive throat swab culture: only in 10-25 % cases of ARF. ASO titre: normal in about one-fifth of adult cases and most cases of chorea. Echocardiography: typically shows mitral regurgitation with dilatation of the mitral annulus and prolapsed anterior mitral leaflet. Aortic regurgitation and pericardial effusion is common.


TREATMENT Bed rest : reduces joint pain and cardiac workload. Duration guided by symptoms and markers of inflammation. 2. Aspirin: helps to confirm diagnosis. Starting dose is 60mg/kg body weight per day, divided into six doses….continue until the ESR has fallen and then gradually tailed off.


TREATMENT…CONTINUE 3. For acute attack after diagnosis: A single dose of Benzyle Penicilline 1.2 million U IM or orally or Phenoxymethylpenicillin 250 mg 6 hourly for 10 days….it eliminate any residual streptococcal infection. 4. Treatment is then directed towards limiting cardiac damage and relieving symptoms.


TREATMENT…CONTINUE 5. Corticosteroids: No evidence that long term steroid are beneficial. Indicated for case with carditis or severe arthritis. Prednisolone , 1.0- 2.0 mg/kg per day in divided dose….continue until ESR is normal…then tailed off.


SECONDARY PREVENTION For prophylaxis: Benzylpenicillin 1.2 million U IM monthly or orally Phenoxymethylpenicillin 250 mg 12 hourly….. For long term…5 years after attack. Further attack of RF is unusual after the age of 21. Patient lives in high prevalence area or has an occupation with high risk of exposure streptococcal infection…continue .