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Rheumatic heart disease: Acute Rheumatic Fever

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Rheumatic heart disease: Acute Rheumatic Fever

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Rheumatic heart disease: Acute Rheumatic Fever

  1. 1. Case summary
  2. 2. Rheumatic heart disease: Acute rheumatic fever Pratap Sagar Tiwari
  3. 3. Introduction :ARF • RF is an acute, immunologically mediated, multisystem inflammatory disease involving heart, joints, CNS, skin and other tissues that occurs a few wks (2-4 Wks) after an episode of group A β-hemolytic streptococcal pharyngitis. • RHD is the cardiac manifestation of RF and is a/w inflammation of the valves, myocardium, or pericardium. • ARF usually affects children (MC betwn 5-15 yrs)
  4. 4. Pathophysiology • The condition is triggered by an immune-mediated response to infection with specific strains of group A streptococci, which have antigens that may cross-react with cardiac myosin and sarcolemmal membrane protein. • Antibodies produced against the streptococcal antigens cause inflammation in the endocardium, myocardium and pericardium, as well as the joints and skin.
  5. 5. Histology • Aschoff nodules are pathognomonic and occur only in the heart. • They are composed of multinucleated giant cells surrounded by macrophages and T lymphocytes. • Are not seen until the subacute or chronic phases of rheumatic carditis. • Anitschkow cells are enlarged macrophages found within granulomas (called Aschoff bodies).
  6. 6. The Jones Criteria Major Minor Migratory arthritis (predominantly involving the large joints) Carditis and valvulitis (eg, pancarditis) Sydenham chorea Erythema marginatum Subcutaneous nodules • Fever, Arthralgia • Elevated acute phase reactants [(ESR), (CRP)] • Prolonged PR interval The probability of acute rheumatic fever is high in the setting of group A streptococcal infection followed by two major manifestations or one major and two minor manifestations .
  7. 7. Evidence of Streptococcal pharyngitis • Positive throat culture for group A beta-hemolytic streptococci • Positive rapid streptococcal antigen test • Elevated or rising antistreptolysin O antibody titer.
  8. 8. There are 3 circumstances in which a presumptive DX of ARF can be made without strict adherence to the above criteria : • Chorea as the only manifestation. • Indolent carditis . • Recurrent rheumatic fever in patients with a history of rheumatic fever or rheumatic heart disease.
  9. 9. Pharyngitis: GAS infection vs Viral Infection F s/o GAS infection F s/o Viral Infection Patient 5 to 15 years of age Conjunctivitis Fever, Headache Coryza Sudden onset of sore throat Cough Beefy, swollen, red uvula Diarrhea Pain with swallowing Hoarseness Nausea, vomiting, abdominal pain Tender, enlarged anterior cervical nodes Scarlet fever rash Soft palate petechiae (doughnut lesions”) Tonsillopharyngeal erythema,exudates Adapted from Gerber MA, Baltimore RS, Eaton CB, et al. endorsed by the American Academy of Pediatrics. Circulation. 2009;119(11):1543.
  10. 10. Carditis :50-60% • A 'pancarditis' involves the endocardium, myocardium and pericardium to varying degrees. • May manifest as breathlessness (due to HF or p.effusion), palpitations or chest pain (usually due to pericarditis or pancarditis). • Other: tachycardia, cardiac enlargement and new or changed cardiac murmurs. • A soft systolic murmur due to MR is very common. A MDM (the Carey Coombs murmur) is typically due to valvulitis, with nodules forming on the MV leaflets. • AR occurs in about 50% but TV and PV are rarely involved. • Pericarditis may cause chest pain, a pericardial friction rub.
  11. 11. Arthritis :60-75% • MC major manifestation and tends to occur early when streptococcal antibody titres are high. • An acute painful asymmetric and migratory inflammation of the large joints typically affects the knees, ankles, elbows and wrists. • The joints are involved in quick succession and are usually red, swollen & tender for btn a day and 4 wks. • The pain characteristically responds to aspirin; if not, the diagnosis is in doubt.
  12. 12. Skin lesions: <5 % • Erythema marginatum :The lesions start as red macules (blotches) that fade in the centre but remain red at the edges and occur mainly on the trunk and proximal extremities but not the face. • Subcutaneous nodules :They are small (0.5-2.0 cm), firm and painless, non pruritic and are best felt over extensor surfaces of bone or tendons. They typically appear more than 3 weeks after the onset of other manifestations .
  13. 13. Erythema marginatum / Subcutanous nodules http://www.hxbenefit.com/erythema-marginatum.htm http://www.doctortipster.com/1789-rheumatic-fever.html l
  14. 14. Sydenham's chorea 2-30 % • This is a late neurological manifestation that appears at least 3-8 months after the episode of ARF, when all the other signs may have disappeared. • It occurs in up to 1/3rd of cases and is more common in females. • Emotional lability may be the first feature and is typically followed by purposeless involuntary choreiform movements of the hands, feet or face. Speech may be explosive and halting. • Spontaneous recovery usually occurs within a few months. • Approximately one-quarter of affected patients will go on to develop chronic rheumatic valve disease.
  15. 15. Primary Prevention of Rheumatic Fever (Treatment of Streptococcal Tonsillopharyngitis)
  16. 16. AHA Recommendations for Duration of Secondary Prophylaxis Category of Patient Duration of Prophylaxis RF without carditis For 5 years after the last attack or 21 years of age (whichever is longer) RF with carditis but no residual valvular disease For 10 years after the last attack, or 21 years of age (whichever is longer) RF with persistent valvular disease, evident clinically or on echocardiography For 10 years after the last attack, or 40 years of age (whichever is longer). Sometimes lifelong prophylaxis.
  17. 17. Chronic rheumatic heart disease • Chronic VHD develops in at least half of those affected by rheumatic fever with carditis. Two-thirds of cases occur in women. • The mitral valve is affected in more than 90% of cases; the aortic valve is the next most frequently affected, followed by the tricuspid and then the pulmonary valve. • Isolated MS accounts for about 25% and an additional 40% have mixed MS/MR.
  18. 18. Poststreptococcal reactive arthritis • The latent period between the antecedent streptococcal infection -migratory arthritis is shorter (1-2wks) than the 2-3 wks usually seen in ARF. • The response of arthritis to aspirin is poor • Evidence of carditis is not seen, and the severity of the arthritis is quite marked. • Extraarticular manifestations ie tenosynovitis & renal abnormalities often are seen . • Acute phase reactants (ESR, CRP) tend to be lower than of ARF.
  19. 19. End of slides Ref: • 1st slide pic :www.pathguy.com/lectures/heart.htm • Harrison’s Principles of Internal medicine • Davidson 21st ed • Uptodate 20.3 • Medscape

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