1. Rheumatic Fever
Dr.M.H.Jokar

2. Objectives Etiology Epidemiology Pathogenesis
Clinical manifestations & Laboratory findings
Diagnosis & Differential diagnosis
Treatment & Prevention
Prognosis

3. DEFINITION
Acute rheumatic fever (ARF) is a systemic autoimmune disease triggered by cross-reactive immune responses between group A β-hemolytic streptococci (GAS) and host tissue epitopes.

4. “The most important consequence of rheumatic fever is chronic rheumatic heart disease”

5. Epidemiology Incidence more during fall ,winter & early spring
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

6. Epidemiology
3% of individuals with untreated group A streptococcal pharyngitis will develop rheumatic fever

7. Epidemiology

9. Pathophysiology

10. Etiology Follows group A beta hemolytic streptococcal pharyngitis
It is a delayed non-suppurative sequelae to URTI with GABH streptococci.

11. Diagrammatic structure of the group A beta hemolytic streptococcus
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
Capsule
Cell wall
Protein antigens
Group carbohydrate
Peptidoglycan
Cyto.membrane
Cytoplasm
…………………………………………………...

12. 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

13. Tonsillitis

14. Pathogenesis
Abnormal immune response to one or more as yet unidentified somatic or extracellular antigens produced by all (or perhaps only by some) group A streptococci.

15. 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

16. Pathogenesis
Antigenic mimicry
Predisposing genetic influence

19. Arthritis
Migratory polyarthritis, involving large 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

20. It usually does not affect the small joints of the hands or feet and seldom involves the hip joints

22. Carditis

23. Sydenham Chorea Occur in 5-10% of cases
Mainly in girls of 5-15 yrs age
May appear even 6/12 month after the attack of rheumatic fever
Clinically manifest as deterioration of handwriting, emotional lability or grimacing of face
Clinical signs- milking sign of hands

25. 4.Erythema Marginatum Occur in <5%.
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

27. Subcutaneous nodules Occur in 5-10%
Painless,pea-sized,palpable nodules
Mainly over extensor surfaces of joints,spine,scapulae & scalp
Always associated
with severe carditis

28. SUBCUTANEOUS NODULES

29. Other features (Minor features)
Fever
Arthralgia
Pallor
Anorexia
Loss of weight

30. Laboratory Findings High ESR Anemia, leucocytosis
Elevated C-reactive protien
ASO titre >200 Todd units.80% (Peak value attained at 3 weeks,then comes down to normal by 6 weeks)
Anti-DNAse
Throat culture-GABHstreptococci 20-40%

31. Pathology
Aschoff nodule (body)

32. مبحث: تب روماتيسمي
درس فيزيوپاتولوژی

33. Diagnosis Rheumatic fever is mainly a clinical diagnosis
No single diagnostic sign or specific laboratory test available for diagnosis

34. Major & Minor manifestations
Carditis
Polyarthritis
Chorea
Erythema marginatum,
Subcutaneous nodules.
Minor
Fever
Polyarthralgia
Acute-phase reactants (erythrocyte sedimentation rate or leukocyte count).

35. Supporting evidence for a preceding GAS
Prolonged PR interval
Elevated or rising aso or antibody
Positive throat culture
Positive rapid antigen test
Recent scarlet fever.

37. DIFFERENTIAL DIAGNOSIS

38. Treatment Step I - primary prevention (eradication of streptococci)
Step II - anti inflammatory treatment (aspirin,steroids)
Step III- secondary prevention (prevention of recurrent attacks)

39. ANTI-INFLAMMATORY AGENTS
Aspirin 4-8grams/day for adults
Continue anti-inflammatory therapy until ESR or CRP are normal
May need steroids if there is cardiac involvement to help prevent sequelae such as mitral stenosis
Corticosteroids, if indicated, are given at prednisone 2mg/kg/day for 2 weeks and then tapered

41. 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