Rheumatic Fever by Adnan Bhutto

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Rheumatic Fever

Rheumatic Fever

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  • Rheumatic Fever

    Rheumatic fever is inflammation of the body’s organ systems, mainly joints, skin, brain and the heart.The fever mostly develops two or three weeks after the streptococcal infection of the throat. It is so named because of its similarities with Rheumatic disorder (medical conditions affecting the joints and connective tissue).

    How does it affect the heart?

    The heart is considerably affected by this infection. The valves and membranes of the heart are damaged by rheumatic fever, which results in improper opening and closing of the valves. Although most of the patients recover completely from the fever, a small percentage of people damage their heart permanently. When the damage is permanent it is called rheumatic heart disease.It can effect any valve , especially mitral valve.The valves may become aberrantly narrow (mitral valve stenosis), leaky (mitral valve regurgitation) or both. These damages to the valves may ultimately cause heart failure and atrial fibrillation later in life of the patient.
    http://heart-consult.com/articles/132/rheumatic-fever
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  • 1. RHEUMATIC FEVER PRESENTED BY ADNAN AHMED BHUTTO Hamdard University Karachi, Pakistan 05/22/10
  • 2. OBJECTIVES
    • ETIOLOGY
    • EPIDEMIOLOGY
    • PATHOGENESIS
    • CLINICAL FEATURES
    • INVESTIGATIONS
    • DIAGNOSIS
    • TREATMENT
    05/22/10
  • 3. ETIOLOGY
    • Acute Rheumatic Fever is an inflammatory disease with devastating sequalae.
    • It resulting from infection of upper respiratory tract by group A beta hemolytic streptococcal infection.
    • It is a diffuse inflammatory disease of connective tissue, primarily involving heart, blood vessels, joints, subcutaneous tissue and brain.
    05/22/10
  • 4. EPIDEMIOLOGY
    • Important cause of chronic heart disease and death in developing countries.
    • More common in third world countries.
    • Commonly occurs in 5-15 years of age.
    • Male and Female are equally effected.
    • Overcrowding, poverty, lack of access to medical care contributes to transmission.
    • Incidence is more in cold months
    05/22/10
  • 5. 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.
    • Antibodies made against group A strep cross react with human tissue.
    05/22/10
  • 6. 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 as skin lipid cholesterol inhibit antigenicity
    05/22/10
  • 7. 05/22/10 Diagrammatic structure of the group A beta hemolytic streptococcus Cytoplasm Cyto.membrane Peptidoglycan Group carbohydrate Protein antigens Cell wall Capsule Antigen of outer protein cell wall of GABHS induces antibody response in victim which result in autoimmune damage to heart valves, sub cutaneous tissue,tendons, joints & basal ganglia of brain
  • 8. Pathologic lesions
    • Fibrinoid degeneration of connective tissue,inflammatory edema, inflammatory cell infiltration & proliferation of specific cells resulting in formation of Ashcoff nodules , resulting in-
    • - Pancarditis in the heart
    • - Arthritis in the joints
    • - Ashcoff nodules in the
    • subcutaneous tissue
    • - Basal gangliar lesions resulting in chorea
    05/22/10
  • 9. Rheumatic Carditis Histology 05/22/10
  • 10. Histology of Myocardium in Rheumatic Carditis 05/22/10
  • 11. CLINICAL FEATURES
    • ARTHIRITIS
    • Most common feature occurs in 80% of patients.
    • Flitting & fleeting migratory polyarthritis, involving major joints
    • Commonly involved joints knee, ankle, shoulders, elbow & wrist
    • Involved joints becomes tender.
    • In children below 5 yrs arthritis usually mild but Carditis more prominent
    • Arthritis do not progress to chronic disease
    05/22/10
  • 12. 05/22/10 Showing ARTHIRITIS In knee joint
  • 13. 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 (fishmouth valves)
    05/22/10
  • 14.
    • Clinical Signs:
    • High Pulse rate
    • Murmurs
    • Cardiomegaly
    • Rhythm disturbances(Prolonged PR intervals
    • Pericardial Friction rubs
    • Cardiac Failure
    1.Carditis (Contd.) 05/22/10
  • 15. 05/22/10 Rheumatic heart disease . Abnormal mitral valve. Thick, fused chordae
  • 16. 05/22/10 Another view of thick and fused mitral valves in Rheumatic heart disease
  • 17. 05/22/10
  • 18. 3. Sydenham’s Chorea
    • Occur in 10-15% of patients with rheumatic fever.
    • Mainly in girls resulting from disorder of extra pyramidal system.
    • Fast, Clonic, involuntary movements (especially face and limbs)
    • Emotionally liability
    • Muscular Hypotonus
    • Clinically difficulty in Walking, Talking and Writing.
    • It is self limiting but may last from few weeks to six months and rarely up to 1-2 years.
    05/22/10
  • 19. 05/22/10
    • It is self limiting but may last from few weeks to six months and rarely up to 1-2 years.
    • Chances of reoccurrence are recorded. Shown in table.
    3. Sydenham’s Chorea
  • 20. 05/22/10
  • 21. 4.Subcutaneous Nodules
    • Occur in 5% of patients
    • Painless, pea-sized, palpable nodules.
    • Mainly over extensor surfaces of joints, spine, scapulae & scalp.
    • Associated with strong seropositivity
    • Always associated with severe Carditis
    05/22/10
  • 22. 5.Erythema Marginatum
    • Occur in 5%of children.
    • Unique, transient, serpiginous-looking 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
    05/22/10
  • 23. 05/22/10 OTHER CLINICAL FEATURES
    • Fever
    • Arthralgia
    • Epistaxis
    • Abdominal pain due to Peritonitis
    • Hematuria due to involvement of kidneys
    • Pneumonitis
    • Mild Pleuritis (5-10%)
    • Encephalitis (extremely rare)
  • 24. 05/22/10 LABORTARY INVESTIGATIONS
    • High ESR
    • Anemia, leucocytosis
    • Elevated C-reactive protien
    • ASO titre >200 Todd units.
    • (Peak value attained at 3 weeks, then comes down to normal by 6 weeks)
    • Anti-DNAse B test
    • Throat culture-GABHstreptococci
    • ECG- prolonged PR interval, 2nd or 3rd degree blocks,ST depression, T inversion
    • 2D Echo cardiography- valve edema, mitral regurgitation, LA & LV dilatation,pericardial effusion, decreased contractility
  • 25. 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
    05/22/10
  • 26. 05/22/10
  • 27. Exceptions to Jones Criteria
    • Chorea alone, if other causes have been excluded
    • Insidious or late-onset Carditis with no other explanation
    • Patients with documented RHD or prior rheumatic fever, one major criterion, or of fever, arthralgia or high CRP suggests recurrence
    05/22/10
  • 28. Differential diagnosis
    • Juvenile rheumatoid arthritis
    • Septic arthritis
    • Sickle-cell arthropathy
    • Kawasaki disease
    • Myocarditis
    • Scarlet fever
    • Leukemia
    05/22/10
  • 29. 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)
    05/22/10
  • 30. 05/22/10 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)
  • 31. Step II: Anti inflammatory treatment 05/22/10 Clinical condition Drugs
  • 32. 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
    05/22/10
  • 33. 05/22/10 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
  • 34. 05/22/10 Duration of Secondary Rheumatic Fever Prophylaxis Category Duration Rheumatic fever with carditis and At least 10 y since last residual heart disease episode and at least until (persistent valvar disease * ) age 40 y, sometimes lifelong prophylaxis Rheumatic fever with carditis 10 y or well into adulthood, but no residual heart disease whichever is longer (no valvar disease*) Rheumatic fever without carditis 5 y or until age 21 y, whichever is longer *Clinical or echocardiographic evidence.
  • 35. 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 valvular lesions
    05/22/10
  • 36. 05/22/10