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Acute rheumatic fever


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briefly on acute rheumatic fever

Published in: Health & Medicine
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Acute rheumatic fever

  3. 3. INTRODUCTION o Rheumatic fever is an immunologically mediated inflammatory disorder, which occurs as a following to group A streptococcal pharyngeal infection. o Multisystem disease affecting connective tissue particularly of the heart, joints, brain, cutaneous and subcutaneous tissues o The illness is so named because of its similarity in presentation to rheumatism.
  4. 4. Rheumatic fever Rheumatic heart disease • It’s a crippling disease • Preventable disease • More common in developing countries.
  5. 5. INCIDENCE • It accounts for 12-65% of hospital admissions related to CVD in developing countries. • There has been marked decrease in cases of RF and RHD in places that have implemented preventive programs. IN INDIA: • RHD is prevalent in range of 5-7/1000 in 5-15 age groups.(IAP 2008) • Streptococcal infections common in children living in under privileged conditions and RF accounts for 1-3% of the cases.
  7. 7. PATHOGENESIS • MOST common strains- M types-1,3,5,6,18,29. • Immune mediated- antigenicity of several GAS cellular and extracellular epitopes and their immunologic cross reactivity with cardiac antigenic epitopes(molecular mimicry) • M proteins- M1,M5,M6 and M19.
  8. 8. STRETOCOCCAL SORE THROAT • Tender lymph nodes • Close contact with infected person • Scarlet fever rash • Excoriated nares( crusted lesions) in infants • Tonsillar exudates in older children Abdominal pain • Latent PERIOD- 2-4 weeks. • GOLD STANDARD-POSITIVE THROAT CULTURE • Sudden onset of fever, pallor, malaise, fatigue.
  9. 9. MODIFIED JONES CRITERIA MAJOR  Carditis  Polyarthritis  Erythema marginatum  Subcutaneous nodules  Chorea MINOR  Arthralgia  Fever  Lab findings a) ↑ ESR b) ↑ CRP c) Prolonged PR interval Supportive evidence of preceding streptococcal infection(essential except for diagnosis of chorea) Anti streptolysin O ASO titre>333units-children, >250 units-adults Anti deoxyribonucleade B History of (within previous 45 days) Streptococcal sore throat Scarlet fever Positive throat culture Positive rapid streptococcal antigen detection test
  10. 10. DIAGNOSTIC CATEGORIES CRITERIA Primary episode of RF 2 major or 1 major and 2 minor manifestation + evidence of presiding group A streptococcal infection Recurrence in a patient without established heart disease 2 major or 1 major and 2 minor manifestation + evidence of presiding group A streptococcal infection Recurrence in a patient with established heart disease 2 minor and supportive evidence of previous streptococcal throat infection Rheumatic chorea Insidious onset rheumatic carditis Other major manifestations or evidence of group A streptococcal infection not required Chronic valve lesions of RHD Do not require any other criteria to be diagnosed as having RHD
  11. 11. ARTHRITIS Most common feature: • Present in 90% of patients. • Painful, migratory, short duration, asymmetrical. • Rheumatic joints- red, hot, swollen, tender • Usually >5 joints affected and mainly large joints Knees, ankles, wrists, elbows, shoulders • Small joints and cervical spine less commonly involved
  12. 12. • Excellent response of salicylates and NSAIDS • Pain and swelling come on quickly and subsides within 5-7 days • In children below 5 yrs arthritis usually mild but carditis more prominent • Arthritis do not progress to chronic disease
  13. 13. • JACCOUD’S ARTHRITIS: Chronic post rheumatic fever arthropathy Deforms the fingers and toes Deformity – ulnar deviation of fingers, flexion of MCP joints and hyper extension of PIP joints No inflammatory changes Occurs after prolonged and multiple attacks. Prognosis – good.
  14. 14. CARDITIS • Early and most serious manifestation • Manifest as pancarditis • Occur in 60-70% of cases • Carditis is the only manifestation of rheumatic fever that leaves a sequelae & permanent damage to the organ • Valvular damage is the hallmark of RF • Chronic phase- fibrosis, calcification & stenosis of heart valves(fishmouth valves) Any cardiac tissue may be affected Valvular lesion most common: mitral and aortic Seldom see isolated pericarditis or myocarditis
  15. 15. • Myocarditis and pericarditis without endocarditis is never rheumatic carditis- ruleout other causes. • ACUTE RF- present as tachycardia and cardiac murmur. • Moderator and severe- cardiomegaly, heart failure with hepatomegaly, peripheral and pulmonary edema. • Indicators of recurrence of RF in established heart disease: 1.New murmur/change in pre existing murmur. 2.Pericardial rub(other evidence of pericarditis) 3.Unexplained congestive heart failure(CHF), including cardiomegaly.
  16. 16. PATHOLOGICAL MITRAL REGURGITATION PATHOLOGIC AORTIC REGURGITATION Seen in at least 2 views Seen in at least 2 views Jet length ≥ 2 cm in at least 1 view Jet length ≥ 1cm in at least 1 view Peak velocity > 3 meters/second Peak velocity > 3 meters/second Pan systolic jet in at least 1 envelope Pan diastolic jet in at least 1 envelope ECHO FINDINGS:
  17. 17. CHOREA • Occur in 5-10% of cases • Mainly in girls of 1-15 yrs age • Late manifestation of RF -months after infection • Involuntary , jerky choreiform movements, • Speech affected, fidgety • Choreiform movements particularly affect the head(darting movement of tongue)and upper limbs.
  18. 18. • Clinical maneuvers: 1. MILKMAID SIGN- irregular contractions and relaxation of muscle of fingers while squeezing the examiners finger. 2. Spooning and pronation of hands when patient’s arm extended. 3. Wormian darting movements of tongue upon pronation 4. Examination of hand writing to evaluate fine motor movements.
  19. 19. • First sign: difficulty walking, talking, writing Occurs in 30% of patients with ARF • Usually benign and resolves in 2 - 3 months • Disappears leaving no residual damage.
  20. 20. ERYTHEMA MARGINATUM • Occur in <7%. • Unique, transient, serpiginous-looking lesions of 1-2 inches in size • Pink macules - Clear centrally ,serpiginous spreading edge . • More on trunks & limbs & non-itchy • Almost never on face Worsens with application of heat Often associated with chronic carditis
  21. 21. SUBCUTANEOUS NODULE: • Small, painless, mobile hard lumps beneath skin. • Most common along - extensor surfaces of joint- Knees, elbows, wrists • Also: on bony prominences, tendons, dorsi of feet, occiput or cervical spine • Appears 4 weeks after onset of RF • Delayed manifestation, disappears –leaves no residual damage. • Occur in 9 - 20% of cases • Often associated with carditis
  22. 22. MINOR CRITERIA • Fever- LR-38.5◦F,MR/ HR- 38◦F • Arthralgia –LR-Polyarthralgia,MR/HR-Mono • High ESR • Anaemia, leucocytosis • Elevated C-reactive protein • Prolonged P-R interval(normal range- 3-12yrs=0.16secs, 12-14yrs=0.18secs, >17yrs=0.20secs)
  23. 23. Exceptions to Jones Criteria • Chorea alone, if other causes have been excluded • Insidious or late-onset carditis with no other explanation
  24. 24. TREATMENT • PHARYNGITIS AND SECONDARY PROPHYLAXIS: DRUGS DOSE PHARYNGITIS SECONDARY PROPHYLACTICS Benzathine penicillin G(deep IM injection) 1.2 million units>27kgs 0.6 million units <27kgs Single dose 21 days 15 days Penicillin V oral Children:250mg QID 10 DAYS Twice a day Azithromycin(oral) 12.5 mg/kg/day Once daily 5 days Not recommended Cephalexin (oral) 15-20mg/kg/day QID 10 days Not recommrnded Erythromycin (oral) 20mg/kg/dose max 500mg Not recommended Twice a day
  25. 25. DURATION OF SECONDARY PROPHYLAXIS • No carditis : 5 years / 18 years of age, whichever is longer • Mild to moderate carditis and healed carditis: 10 years / 25 years of age, whichever is longer • Severe disease or post intervention patients: Lifelong. One may opt for secondary prophylaxis up to the age of 40 years
  26. 26. • Inflammation in acute rheumatic fever: 1. Mild carditis+ arthritis Regime 1- aspirin 100mg/kg/day for 2-3 weeks divide in 4-5 doses. Taper to 60- 70mg/kg/day Regime 2- 50-60mg/kg/day total 12 weeks. 2. Moderate to Severe carditis Regime 1- Prednisolone 2mg/kg/day till normal ESR. Taper over 2-4 weeks reduce dose by 2.5-5 mg every 3 rd day. Start aspirin simultaneously to complete total 12 weeks. Regime 2- Prednisolone – 3-4 weeks taper slowly to cover total period of 10-12 weeks.
  27. 27. • Chorea – mild – silent environment and sedatives like oral phenobarbitone or diazepam. Haloperidol- (0.25-0.5mg/kg/day) Sodium valproate (15mg/kg/day). Carbamazepine (7-20mg/kg/day) • Congestive heart failure- digoxin, diuretics, inotropes.
  28. 28. • Recurrence – New episode of rheumatic fever following another GABHS infection occuring after 8 weeks following stopping treatment. • Rebound – RF occuring within 4-6 weeks of stopping treatment or while tappering the dose. • Relapse – Worsening of RF while under treatment and often with carditis • Sub-clinical carditis – Clinical examination normal, ECHO – abnormal • Indolent carditis – Present with persistent features of CHF, murmur and cardiomegaly. No or very few features of carditis.
  29. 29. 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 • Rheumatic heart disease is the only truly preventable chronic heart condition