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Rheumatic fever - all you need to know

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Teaching presentation on Rheumatic fever, including etiology, clinical features, diagnosis and treatment. Evidence based approach in management.

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Rheumatic fever - all you need to know

  1. 1. Dr.Sid Kaithakkoden MD MBBS,DCH,DNB,MD,MRCPCH,FCPS alavisaid@aol.com
  2. 2. 2 ARF Immunologically mediated inflammatory response Delayed sequel to GABH Strept. throat infection Genetically susceptible individuals Developed world - dramatic decline in incidence Developing world – still a major problem – 20 million new cases/year Introduction
  3. 3. 3 ARF - Aetiopathogenesis  Definite aetiology ?? Antigenic mimicry between streptococcal M-protein epitopes & human tissues (heart valves, myosin, synovium & basal ganglia) Autoimmunity in genetically susceptible individuals Constant association with HLA class II antigens (HLA B5)  Age – 5 -18 yrs  Incidence: Developed world - 0.05/1000 population Developing world - 24/ 1000 population
  4. 4. 4 Making the diagnosis of streptococcal pharyngitis Streptococcal pharyngitis (Group A beta- hemolytic pharyngitis) Only 10-15% incidence in adults with pharyngitis But a 40% incidence in children with pharyngitis
  5. 5. 5 Making the diagnosis of streptococcal pharyngitis  Scoring system for risk of strep pharyngitis: 1. Temperature > 37.8 degrees C 2. Tonsillar exudate 3. Anterior cervical lymphadenopathy  Three factors present = 40-50% risk of strep pharyngitis  Only two factors present = 15% risk  Consider increased risk for known exposure or community outbreak
  6. 6. 6 Making the diagnosis of streptococcal pharyngitis Clinical diagnosis Fever and sore throat are always present Rarely seen are rhinitis, conjunctivitis, bronchitis, laryngitis or diarrhea Must have pharyngeal edema or exudate Must have cervical lymphadenopathy
  7. 7. 7 Diagnosis of ARF No “gold standard” No specific clinical/lab. test to establish diagnosis Diagnosis based on revised (updated) Jones criteria 1944 T. Duckett Jones Final revision 1992 – by committee on Rheumatic Fever, Endocarditis, Kawasaki Disease of the AHA
  8. 8. 8 Updated Jones Criteria: (need 2 major or 1 major and 2 minor criteria AND evidence of infection):  Major manifestations  Carditis  Erythema marginatum  Polyarthritis  Subcutaneous nodules  Chorea  Minor manifestations  Clinical findings: arthalgia and fever  Lab findings: ↑ESR, ↑C-reactive protein, ↑acute-phase reactants, prolonged PR interval  Supporting evidence of antecedent streptococcal infection  Positive throat culture or rapid streptococcal antigen test  Elevated or rising streptococcal antibody titers  Exception :  Chorea  Indolent carditis
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  10. 10. 10 Rheumatic Aortic Valve
  11. 11. 11 Erythema marginatum
  12. 12. 12
  13. 13. 13 Clinical findings in ARF Carditis  may have an insidious or subclinical onset:  40-50% incidence with first attack of ARF  More common in younger children  Decreased risk with increasing degree of polyarthritis  Is frequently a pancarditis, may be asymptomatic.  Usually appears in the first 3 weeks of an ARF attack.  Suggested by presence of :  Pericarditis, cardiomegaly, CHF, new heart murmur(s)  Less specific findings:  ECG changes: PR interval (>0.04), P wave contour change, inverted T waves  Resting tachycardia – even during sleep  Arrythmias
  14. 14. 14 Carditis Onset of new heart murmur(s): Mitral regurgitation/insufficiency – high pitched blowing holosystolic apical murmur, grade 2 or higher that radiates to axilla Aortic regurgitation – high pitched decrescendo murmur at aortic area  Mitral stenosis and aortic stenosis are classic findings of chronic rheumatic heart disease.  25% go on to develop mitral stenosis  40% will develop mitral insufficiency
  15. 15. 15 Polyarthritis  Classically is a migratory polyarthritis:  Affects large joints sequentially (knees, elbows, ankles and wrists usually) with multiple joints involved at the same time.  Diagnosis based on joint pain along with heat, swelling, redness and tenderness.  May have arthralgias –-- pain without associated findings.  Adolescent children are more likely to have only one arthritic joint  50% have 6 or more joints involved (↑arthritis = ↓carditis).  Usually lasts < 4 weeks without residual damage
  16. 16. 16 Erythema Marginatum  The rash specific for ARF. 10% incidence  Described as a macular or raised erythematous rash in rings or crescent shapes with clear centers. Nonpruritic and nonpainful  Lesions come and go in minutes to hours. May occur intermittently for weeks to months  Primarily seen on trunk and proximal extremities.
  17. 17. 17 Subcutaneous Nodules  10% incidence in ARF More likely to be present with carditis  Are only present for days to a couple of weeks May be recurrent however  Description: Firm, painless, < 2cm nodules found over bony prominences or tendons Common on elbows, knees, wrists, ankles and Achilles tendon Usually one to a few dozen nodules Indistinguishable from rheumatoid nodules There is no treatment
  18. 18. 18 Sydenham’s Chorea  Involuntary movements of the hands, face and feet: 5-15% incidence May also involve muscular weakness and emotional lability  Often there is a long latent period between antecedent streptococcal pharyngitis and the onset of chorea. Movements are suppressible with sedation Females affected more often than males  Attacks often last for several months
  19. 19. 19 Laboratory Findings  No definitive tests  1. If there is no recent documented streptococcal pharyngitis, then you need to check a rapid streptococcal antigen test following by throat culture if antigen test negative  2. Acute phase reactants : ESR, CRP,  3. Serum titer of antistreptococcal antibodies (ASO) 80% will have a positive titer within 2 mths of ARF onset
  20. 20. 20 Treatment Prevention of initial attack of RF (primary prevention) eradication of streptococci Anti inflammatory treatment aspirin, steroids Prevention of recurrence (secondary prevention) antibiotic prophylaxis
  21. 21. 21 Treatment of ARF with Medications: 1. Antibiotics – Benzathine penicillin G (aka bicillin LA) 1.2 million units IM for positive throat culture to prevent spread of ARF-causing streptococcal strain. Alternatives:Alternatives:  Penicillin V 250mg BID po for 10days  Erythromycin 250mg QID x 10day for penicillin allergic patients
  22. 22. 22 Treatment of ARF with Medications: 2. Salicylates – for fever and joint pain/swelling 100mg/kg/d of aspirin for children Should see prompt response in joints Treat arthralgias with analgesics NSAIDs ok for aspirin allergic/intolerant but not studied.
  23. 23. 23 Treatment of ARF with Medications: 3. Corticosteroids – use when salicylates fail and whenever carditis is present. No proof of cardiac damage prevention. 2mg/kg mg oral prednisone 2-3 week course with taper for arthritis and fever. Up to 6 week course with 2 week taper for carditis. Continue aspirin for one month after stopping steroid
  24. 24. 24 Treatment of Carditis/Heart Failure All carditis patients receive corticosteroids. Strict bed rest for at least 4 weeks Conventional therapies are used to treat specific symptoms such as heart failure.
  25. 25. 25 Treatment of Sydenham’s Chorea Mainstay of treatment is: Quiet environment (symptoms disappear during sleep and are are less frequent with less environmental stimulation). Sedation: Benzodiazepines Haloperidol for more severe cases
  26. 26. 26 Prevention of ARF recurrences: High risk for ARF recurrence with repeat episodes of streptococcal pharyngitis. Recurrences ↓with ↑age and with the number of years since last attack Recurrences are more common in those with a history of ARF carditis and in children. Children have a 20% risk of recurrence in 1st five years.
  27. 27. 27 Prevention of ARF recurrences Need continuous antibiotic prophylaxis for at least 5 years or until patient at least into their early 20s Primary recommendation: Benzathine penicillin G (Bicillin LA) – IM every 4 weeks May give every 3 weeks for those at highest risk Alternative: Sulfadiazine 500mg QD for < 27#, 1000mg QD for > 27# Erythromycin 250mg BID for PCN allergic
  28. 28. 28 Endocarditis Prophylaxis Patients with residual rheumatic valvular disease also need endocarditis prophylaxis Use a different antibiotic than that used for ARF recurrence prevention
  29. 29. 29 Prognosis  Initial mortality rate is 1-2%  Persistent carditis = poorer prognosis 30% mortality within 10 years for children  80% of children affected with ARF live to adulthood  Adults – 2/3 are affected with rheumatic valvular disease after 10 years
  30. 30. 30 Questions needing answer…..  Should we treat all sore throat with antibiotics to prevent rheumatic fever ?  What is the best anti inflammatory drug in carditis to prevent RHD? Aspirin? Steroid?  What is the best mode of administration of penicillin in secondary prophylaxis?  Should we use echocardiographic finding as a major/minor criterion in diagnosis of carditis in ARF ?
  31. 31. 31 Antibiotics for sore throat ?  Del Mar CB, Glasziou PP, Spinks AB. Antibiotics for sore throat. The Cochrane Database of Systematic Reviews 2010, Issue 2. Art. No.: CD000023 Objectives: To assess the benefits of antibiotics in the management of sore throat Search of the literature from 1945 to 2003 Selection: Trials of antibiotic against control with either suppurative complications & non-suppurative complications of sore throat Twenty-six studies
  32. 32. 32 Results & Conclusion:  Antibiotics confer relative benefits in the treatment of sore throat. However, the absolute benefits are modest  Protecting sore throat sufferers against suppurative and non-suppurative complications in modern Western society can be achieved only by treating with antibiotics many who will derive no benefit  In emerging economies where rates of acute rheumatic fever are high, the number needed to treat may be much lower
  33. 33. 33 Anti-inflammatory treatment for carditis in ARF  Cilliers AM, Manyemba J, Saloojee H. Anti- inflammatory treatment for carditis in acute rheumatic fever. The Cochrane Database of Systematic Reviews 2009, Issue 2. Art. No.: CD003176 Objectives: To assess the effects of anti-inflammatory agents (aspirin, corticosteroids & immunoglobulin) for preventing or reducing further heart valve damage in patients with ARF Literature search from1966 to 2005 Eight RCT
  34. 34. 34 Results & Conclusion: No significant difference in the risk of cardiac disease at one year between the corticosteroid-treated and aspirin-treated groups (relative risk 0.87, 95% confidence interval 0.66 to 1.15) Use of prednisone (relative risk 1.78, 95% CI 0.98 to 3.34) or intravenous immunoglobulins (relative risk 0.87, 95% CI 0.55 to 1.39) when compared to placebo did not reduce the risk of developing heart valve lesions at one year CONCLUSION: No benefit in using corticosteroids or intravenous immunoglobulin to reduce the risk of heart valve lesions in patients with ARF
  35. 35. 35 Penicillin for secondary prevention of ARF  Manyemba J, Mayosi BM. Penicillin for secondary prevention of rheumatic fever. The Cochrane Database of Systematic Reviews 2000, Issue 3. Art. No.: CD002227 Objectives: To assess the effects of penicillin compared to placebo and the effects of different penicillin regimens and formulations for preventing strept.infection and rheumatic fever recurrence Nine studies
  36. 36. 36  Four trials (n=1098) compared IM with oral penicillin and all showed that IM penicillin reduced RF recurrence and Strept. throat infections compared to oral penicillin  One trial (n= 249) showed 3-weekly IM penicillin inj. reduced strept. throat infections (RR 0.67, 95% CI 0.48 to 0.92) compared to 4-weekly dose  Conclusions: IM penicillin more effective than oral penicillin in preventing RF recurrence and strept. throat infections Two-weekly or 3-weekly injections appeared to be more effective than 4-weekly injections Results & Conclusion:
  37. 37. 37 Should Echocardiography used as a criterion in diagnosing rheumatic carditis?  Ferrieri P et al. Proceedings of the Jones Criteria workshop. AHA scientific statement. Circulation 2002;106:2521-2523 Echocardiography should only be used as an adjunctive technique to confirm clinical findings and to evaluate chamber sizes, ventricular function & valvar morphology It should not be used as a major/minor criterion for establishing the diagnosis of carditis of ARF in the absence of clinical findings
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