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Rheumatic fever

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Mohammed Alsheikh
Mohammed Alsheikh

Rheumatic fever is an inflammatory disease that can occur following a streptococcal throat infection. It commonly affects children ages 5-15 and is more prevalent in developing countries. The disease involves the heart, joints, skin, and brain. Diagnosis is based on the modified Jones criteria of major and minor manifestations along with evidence of a prior streptococcal infection. Treatment involves eradicating streptococci, using anti-inflammatory drugs, supportive care, and long-term preventative antibiotics to avoid recurrent attacks. Prognosis depends on factors like age and whether carditis was present initially.

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Rheumatic Fever
Etiology Acute rheumatic fever is a systemic disease of childhood,often recurrent that follows group A beta hemolytic streptococcal infection It is a delayed non-suppurative sequelae to URTI with GABH streptococci. It is a diffuse inflammatory disease of connective tissue,primarily involving heart,blood vessels,joints, subcut.tissue and CNS 2
Epidemiology Ages 5-15 yrs are most susceptible Rare <3 yrs Girls>boys Common in 3rd world countries Environmental factors-- over crowding, poor sanitation, poverty, Incidence more during fall ,winter & early spring 3
Pathogenesis Delayed immune response to infection with group.A beta hemolytic streptococci. After a latent period of 1-3 weeks, antibody induced immunological damage occur to heart valves,joints, subcutaneous tissue & basal ganglia of brain 4
Group A Beta Hemolytic Streptococcus Strains that produces rheumatic fever - M types l, 3, 5, 6,18 & 24 Pharyngitis- produced by GABHS can lead to- acute rheumatic fever , rheumatic heart disease & post strept. Glomerulonepritis Skin infection- produced by GABHS leads to post streptococcal glomerulo nephritis only. It will not result in Rh.Fever or carditis 5
Clinical Features 1.Arthritis Migratory polyarthritis, involving major joints Commonly involved joints-knee,ankle,elbow & wrist Occur in 80%,involved joints are exquisitely tender In children below 5 yrs arthritis usually mild but carditis more prominent Arthritis do not progress to chronic disease 6
Clinical Features (Contd) 2.Carditis Manifest as pancarditis(endocarditis, myocarditis and pericarditis),occur in 40-50% of cases Carditis is the only manifestation of rheumatic fever that leaves a sequelae & permanent damage to the organ Valvulitis occur in acute phase Chronic phase- fibrosis,calcification & stenosis of heart valves. 7
Clinical Features (Contd) 3.Sydenham Chorea Occur in 5-10% of cases Mainly in girls of 1-15 yrs age May appear even 6 months after the attack of rheumatic fever Clinically manifest as-clumsiness, deterioration of handwriting,emotional lability or grimacing of face 8
Clinical Features (Contd) 4.Erythema Marginatum Occur in <5%. Unique, transient lesions of 1-2 inches in size Pale center with red irregular margin More on trunks & limbs & non-itchy Worsens with application of heat Often associated with chronic carditis 9
Clinical Features (Contd) 5.Subcutaneous nodules Occur in 10% Painless,pea-sized,palpable nodules Mainly over extensor surfaces of joints,spine,scapulae & scalp Associated with strong seropositivity Always associated with severe carditis 10
Clinical Features (Contd) Other features (Minor features) Fever Low grade Arthralgia Pallor Anorexia Loss of weight 11
Laboratory Findings High ESR Anemia, leucocytosis Elevated C-reactive protien ASO titre >200. (Peak value attained at 3 weeks,then comes down to normal by 6 weeks) Anti-DNAse B test Throat culture-GABHstreptococci 12
Laboratory Findings (Contd) ECG- prolonged PR interval Echo - valve edema,mitral regurgitation, LA & LV dilatation,pericardial effusion,decreased contractility 13
Diagnosis Rheumatic fever is mainly a clinical diagnosis No single diagnostic sign or specific laboratory test available for diagnosis Diagnosis based on MODIFIED JONES CRITERIA 14
Jones Criteria (Revised) for Guidance in the Diagnosis of Rheumatic Fever* Major Manifestation Minor Supporting Evidence Manifestations of Streptococal Infection Carditis Clinical Laboratory Polyarthritis Previous Acute phase Chorea rheumatic reactants: Increased Titer of Anti- Erythema Marginatum fever or Erythrocyte Streptococcal Antibodies ASO Subcutaneous Nodules rheumatic sedimentation (anti-streptolysin O), heart disease rate, others Arthralgia C-reactive Positive Throat Culture Fever protein, for Group A Streptococcus leukocytosis Recent Scarlet Fever Prolonged P- R interval *The presence of two major criteria, or of one major and two minor criteria, indicates a high probability of acute rheumatic fever, if supported by evidence of Group A streptococcal nfection. Recommendations of the American Heart Association 15
Treatment Step I - primary prevention (eradication of streptococci) Step II - anti inflammatory treatment (aspirin,steroids) Step III- supportive management & management of complications Step IV- secondary prevention (prevention of recurrent attacks) 16
STEP I: Primary Prevention of Rheumatic Fever (Treatment of Streptococcal Tonsillopharyngitis) Agent Dose Mode Duration Benzathine penicillin G 600 000 U for patients Intramuscular Once 27 kg (60 lb) 1 200 000 U for patients >27 kg or Penicillin V Children: 250 mg 2-3 times daily Oral 10 d (phenoxymethyl penicillin) Adolescents and adults: 500 mg 2-3 times daily For individuals allergic to penicillin Erythromycin: 20-40 mg/kg/d 2-4 times daily Oral 10 d Estolate (maximum 1 g/d) or Ethylsuccinate 40 mg/kg/d 2-4 times daily Oral 10 d (maximum 1 g/d) Recommendations of American Heart Association 17
Step II: Anti inflammatory treatment Clinical condition Drugs Arthritis only Aspirin 75-100 mg/kg/day,give as 4 divided doses for 6 weeks (Attain a blood level 20- 30 mg/dl) Carditis Prednisolone 2-2.5 mg/kg/day, give as two divided doses for 2 weeks Taper over 2 weeks & while tapering add Aspirin 75 mg/kg/day for 2 weeks. Continue aspirin alone 100 mg/kg/day for another 4 weeks 18
3.Step III: Supportive management & management of complications Bed rest Treatment of congestive cardiac failure: -digitalis,diuretics Treatment of chorea: -diazepam or haloperidol Rest to joints & supportive splinting 19
STEP IV : Secondary Prevention of Rheumatic Fever (Prevention of Recurrent Attacks) Agent Dose Mode Benzathine penicillin G 1 200 000 U every 4 weeks* Intramuscular or Penicillin V 250 mg twice daily Oral or Sulfadiazine 0.5 g once daily for patients 27 kg (60 lb Oral 1.0 g once daily for patients >27 kg (60 lb) For individuals allergic to penicillin and sulfadiazine Erythromycin 250 mg twice daily Oral *In high-risk situations, administration every 3 weeks is justified and recommended Recommendations of American Heart Association 20
Prognosis Rheumatic fever can recur whenever the individual experience new GABH streptococcal infection,if not on prophylactic medicines Good prognosis for older age group & if no carditis during the initial attack Bad prognosis for younger children & those with carditis with valvar lesions 21

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Rheumatic fever

  • 2. Etiology Acute rheumatic fever is a systemic disease of childhood,often recurrent that follows group A beta hemolytic streptococcal infection It is a delayed non-suppurative sequelae to URTI with GABH streptococci. It is a diffuse inflammatory disease of connective tissue,primarily involving heart,blood vessels,joints, subcut.tissue and CNS 2
  • 3. Epidemiology Ages 5-15 yrs are most susceptible Rare <3 yrs Girls>boys Common in 3rd world countries Environmental factors-- over crowding, poor sanitation, poverty, Incidence more during fall ,winter & early spring 3
  • 4. Pathogenesis Delayed immune response to infection with group.A beta hemolytic streptococci. After a latent period of 1-3 weeks, antibody induced immunological damage occur to heart valves,joints, subcutaneous tissue & basal ganglia of brain 4
  • 5. Group A Beta Hemolytic Streptococcus Strains that produces rheumatic fever - M types l, 3, 5, 6,18 & 24 Pharyngitis- produced by GABHS can lead to- acute rheumatic fever , rheumatic heart disease & post strept. Glomerulonepritis Skin infection- produced by GABHS leads to post streptococcal glomerulo nephritis only. It will not result in Rh.Fever or carditis 5
  • 6. Clinical Features 1.Arthritis Migratory polyarthritis, involving major joints Commonly involved joints-knee,ankle,elbow & wrist Occur in 80%,involved joints are exquisitely tender In children below 5 yrs arthritis usually mild but carditis more prominent Arthritis do not progress to chronic disease 6
  • 7. Clinical Features (Contd) 2.Carditis Manifest as pancarditis(endocarditis, myocarditis and pericarditis),occur in 40-50% of cases Carditis is the only manifestation of rheumatic fever that leaves a sequelae & permanent damage to the organ Valvulitis occur in acute phase Chronic phase- fibrosis,calcification & stenosis of heart valves. 7
  • 8. Clinical Features (Contd) 3.Sydenham Chorea Occur in 5-10% of cases Mainly in girls of 1-15 yrs age May appear even 6 months after the attack of rheumatic fever Clinically manifest as-clumsiness, deterioration of handwriting,emotional lability or grimacing of face 8
  • 9. Clinical Features (Contd) 4.Erythema Marginatum Occur in <5%. Unique, transient lesions of 1-2 inches in size Pale center with red irregular margin More on trunks & limbs & non-itchy Worsens with application of heat Often associated with chronic carditis 9
  • 10. Clinical Features (Contd) 5.Subcutaneous nodules Occur in 10% Painless,pea-sized,palpable nodules Mainly over extensor surfaces of joints,spine,scapulae & scalp Associated with strong seropositivity Always associated with severe carditis 10
  • 11. Clinical Features (Contd) Other features (Minor features) Fever Low grade Arthralgia Pallor Anorexia Loss of weight 11
  • 12. Laboratory Findings High ESR Anemia, leucocytosis Elevated C-reactive protien ASO titre >200. (Peak value attained at 3 weeks,then comes down to normal by 6 weeks) Anti-DNAse B test Throat culture-GABHstreptococci 12
  • 13. Laboratory Findings (Contd) ECG- prolonged PR interval Echo - valve edema,mitral regurgitation, LA & LV dilatation,pericardial effusion,decreased contractility 13
  • 14. Diagnosis Rheumatic fever is mainly a clinical diagnosis No single diagnostic sign or specific laboratory test available for diagnosis Diagnosis based on MODIFIED JONES CRITERIA 14
  • 15. Jones Criteria (Revised) for Guidance in the Diagnosis of Rheumatic Fever* Major Manifestation Minor Supporting Evidence Manifestations of Streptococal Infection Carditis Clinical Laboratory Polyarthritis Previous Acute phase Chorea rheumatic reactants: Increased Titer of Anti- Erythema Marginatum fever or Erythrocyte Streptococcal Antibodies ASO Subcutaneous Nodules rheumatic sedimentation (anti-streptolysin O), heart disease rate, others Arthralgia C-reactive Positive Throat Culture Fever protein, for Group A Streptococcus leukocytosis Recent Scarlet Fever Prolonged P- R interval *The presence of two major criteria, or of one major and two minor criteria, indicates a high probability of acute rheumatic fever, if supported by evidence of Group A streptococcal nfection. Recommendations of the American Heart Association 15
  • 16. Treatment Step I - primary prevention (eradication of streptococci) Step II - anti inflammatory treatment (aspirin,steroids) Step III- supportive management & management of complications Step IV- secondary prevention (prevention of recurrent attacks) 16
  • 17. STEP I: Primary Prevention of Rheumatic Fever (Treatment of Streptococcal Tonsillopharyngitis) Agent Dose Mode Duration Benzathine penicillin G 600 000 U for patients Intramuscular Once 27 kg (60 lb) 1 200 000 U for patients >27 kg or Penicillin V Children: 250 mg 2-3 times daily Oral 10 d (phenoxymethyl penicillin) Adolescents and adults: 500 mg 2-3 times daily For individuals allergic to penicillin Erythromycin: 20-40 mg/kg/d 2-4 times daily Oral 10 d Estolate (maximum 1 g/d) or Ethylsuccinate 40 mg/kg/d 2-4 times daily Oral 10 d (maximum 1 g/d) Recommendations of American Heart Association 17
  • 18. Step II: Anti inflammatory treatment Clinical condition Drugs Arthritis only Aspirin 75-100 mg/kg/day,give as 4 divided doses for 6 weeks (Attain a blood level 20- 30 mg/dl) Carditis Prednisolone 2-2.5 mg/kg/day, give as two divided doses for 2 weeks Taper over 2 weeks & while tapering add Aspirin 75 mg/kg/day for 2 weeks. Continue aspirin alone 100 mg/kg/day for another 4 weeks 18
  • 19. 3.Step III: Supportive management & management of complications Bed rest Treatment of congestive cardiac failure: -digitalis,diuretics Treatment of chorea: -diazepam or haloperidol Rest to joints & supportive splinting 19
  • 20. STEP IV : Secondary Prevention of Rheumatic Fever (Prevention of Recurrent Attacks) Agent Dose Mode Benzathine penicillin G 1 200 000 U every 4 weeks* Intramuscular or Penicillin V 250 mg twice daily Oral or Sulfadiazine 0.5 g once daily for patients 27 kg (60 lb Oral 1.0 g once daily for patients >27 kg (60 lb) For individuals allergic to penicillin and sulfadiazine Erythromycin 250 mg twice daily Oral *In high-risk situations, administration every 3 weeks is justified and recommended Recommendations of American Heart Association 20
  • 21. Prognosis Rheumatic fever can recur whenever the individual experience new GABH streptococcal infection,if not on prophylactic medicines Good prognosis for older age group & if no carditis during the initial attack Bad prognosis for younger children & those with carditis with valvar lesions 21