Acute rheumatic fever

1. Acute Rheumatic Fever
Dr S V Ramanamurty
MD, FIACM, FICP, FACP (USA)

2. Rheumatic Fever
• Definition
• A multisystem inflammatory disorder occurring
as a delayed sequel to pharyngeal infection with
Group A Beta Hemolytic streptococci
• It primarily involves heart, joints, CNS, skin &
subcutaneous tissues

3. Epidemiology
• Rheumatic fever is world wide
• It is a major cause of death and disability in
children & adolescents in socioeconomically
deprived areas
• Over-crowding and substandard housing
predispose to rheumatic fever

4. Epidemiology
• Age of incidence : 5-15 years
• Males and females are equally affected
• People with HLA DR-3 are more prone to
develop

5. Pathogenesis
• Molecular mimicry appears to play an important
role in the pathogenesis of Rheumatic Fever
• Rheumatic fever occurs as an immunological
sequel
• Latent period : 2-15 weeks after streptococcal
sore throat
• Streptococcus induced autoimmunity is believed
to be the mechanism in rheumatic process

6. Pathogenesis
• Several streptococcal antigens have been
demonstrated to have cross reactivity with
cardiac and other tissues (Molecular mimicry)
• Super antigens
• There are shared epitopes between
streptococcal M protein and cardiac myosin
• This leads to cross-reactivity between human
heart & streptococci

7. Pathology
• General
• Acute rheumatic fever is characterized by
• Exudative & Proliferative lesions
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• Exudative lesions are seen in acute lesions (in joints)
• They heal completely
• Proliferative lesions are seen in prolonged & chronic
lesions usually in heart valves

8. Aschoff Body
Hallmark lesion of rheumatic fever
Characterized by localized
Areas of
Fibrinoid degeneration
Surrounded by
o Plasma cells
o Lymphocytes,
o Mononuclear and
o Basophilic giant cells

9. Pathology
• ARF involves heart, joints, CNS, skin, and subcutaneous
tissues
• Carditis: all three layers are involved
• Myocarditis, pericarditis & endocarditis
• Myocarditis:
– Fragmentation of fibroblasts
– Infiltration of lymphocytes
– Presence of Aschoff bodies

10. Pathology
• Pericarditis
– Characterized by deposition of sero-fibrinous exudates giving a
naked eye appearance of
– Bread and butter appearance
– Occasionally pericardial effusion
• Endocarditis
– Characterized by verrucous valvulitis,
– formation of small rheumatic nodules, on the atrial surface of
the mitral valve
– The mitral valve is most commonly affected, next aortic, &
tricuspid valves very rarely pulmonary valve

11. Pathology
• Joints: involvement with effusion (Poly Arthritis)
• Usually heal without residual lesions
• Subcutaneous nodules
• Show histological features of Aschoff bodies
• Pleural effusion: Fibrinous pleurisy
• Pulmonary lesions: Rheumatic pneumonitis
• Brain parenchyma: Nonspecific lesions

12. Clinical features
• General
• After a latent period of 1-5 weeks of sore throat
– Fever
– Anorrhexia
– Arthralgia
– Palpitations
– Lethargy
– Night sweats

13. Clinical features of rheumatic fever

14. Clinical features
• Major features
– Carditis
– Arthritis
– Subcutaneous nodules
– Erythema marginatum
– Chorea

15. Clinical features
• Carditis occurs earlier within 3 weeks of sore throat
• It includes Myocarditis, pericarditis, endocarditis
• Myocarditis
– Tachycardia disproportionate to fever
– Dropped beats
– Tic-tac rhythm / fetal rhythm
– Arrhythmias – prolonged PR interval
– S3, S4, or Summation gallop
– Congestive heart failure

16. Clinical features
• Pericarditis
– Pericardial friction rub,
– pericardial effusion with increased area of dullness,
– Ewart sign (bronchial breathing near inferior angle of left
scapula
• Endocarditis
– Mitral area:
– MDM (carey-combs murmur) due to rheumatic nodules
– Pan-systolic murmur due to Mitral Regurgitation
– Aortic area
– Early Diastolic Murmur due to Aortic Regurgitation

17. Areas of cardiac auscultation

18. Clinical features
• Note
• Carditis to be considered with
• A combination of
– Cardiomegaly
– Pericarditis
– Congestive heart failure

19. Clinical features
• Polyarthritis
– It is the most common manifestation of ARF 75 %,
usually in 4-6 weeks of sore throat
– Large joints, asymmetrically involved with fleeting
Joint pains (two or more joints involved)
– Red, tender, swollen
– Migratory in nature
– spine rarely involved
– Heal completely, without residual lesions

20. Clinical features
• Subcutaneous nodules
– Seen in 10% with severe
Carditis
– Nodules are small, pea
sized, painless & Movable,
– Present on dorsal aspect of
knees, ankles, elbows &
scalp
– Would be present for about
2-3 weeks

21. Clinical features
• Erythema marginatum :
– present in about 10% cases
– Erythmatous macules, with red rounded or
serpiginous margins and clear centers
– They are migratory, transient and evanescent, non-
pruritic, non-indurated
– Blanch with pressure and brought back with
application of heat
– Present usually over trunk or proximal parts of limbs

22. Erythema marginatum :

23. Chorea
– Develops usually late around
6-9 months after the initial
sore throat
– Usually seen in Female
children 7-14 yrs.
– Obsessions and compulsions
common
– Defined as sudden, jerky,
pleomorphic, non-repetitive,
quasi-purposive, involuntary
movements

24. Clinical features
• Other features
– Fever
– Arthralgia
– Epistaxis
– Pain abdomen

25. Duration of the attack
– The average duration of an untreated rheumatic
fever is around 3 months
– If it exceeds 6 months, it is called Chronic
rheumatic fever

26. Investigations
• Isolation of Group A Beta hemolytic streptococci
• By Culture of throat swab (only in a minority of cases)
• Streptococcal Antibody Tests (Serological tests)
• These tests confirm recent streptococcal infection
– Anti-streptolysin O (ASO test)
– Anti – D Nase B test
– Anti Hyaluronidase (AH test)
– Anti Streptozyme (ASTZ test)

27. Investigations
• Serological tests
• Single titers of ASO >250 todd units in adults &
333 todd units in children >5years taken as positive
• A rising titer is more significant
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• Anti Streptozyme (ASTZ test) is a very sensitive indicator of
recent streptococcal infection. Titers >250 units/ml are
considered positive
• If it is negative it rules out streptococcal infection

28. Investigations
• Acute phase reactants
• These tests indicate presence of an inflammatory
process
– ESR raised
– C-reactive protein is raised
• Note
• These two are normal in chorea

29. Investigations
• Other tests confirming an inflammatory
reaction
– Polymorphonuclear leucocytosis
– Increase in serum complements
– Increase in serum mucoproteins, alpha 2 & gamma
globulins
– Anemia due to suppression of erythropoiesis

30. Investigations
• ECG –may show prolonged PR interval
• Chest X Ray PA view – Cardiomegaly,
pulmonary congestion
• Echocardiography can detect
– Myocardial dysfunction
– Valve dysfunction
– Pericardial effusion

31. ECG & X-Ray chest
Prolonged PR interval
Cardiomegaly
Pulmonary congestion

32. Complications
• Arrhythmias
• Congestive heart failure
• Rheumatic heart disease
• Pericarditis
• Myocarditis

33. Differential diagnosis
• Infective endocarditis
• Rheumatoid arthritis
• Sickle cell anemia
• SLE
• Osteomyelitis
• Chronic meningococcemia

34. Diagnosis of acute rheumatic fever
Major criteria
• Carditis
• Polyarthritis
• Chorea
• Erythema marginatum
• Subcutaneous nodules
Minor criteria
• Fever
• Arthralgia
• Previous rheumatic fever
• Raised ESR
• Positive CRP
• Prolonged PR- interval (ECG)
Revised Jones criteria 1992

35. Diagnosis of acute rheumatic fever
• Essential criteria: Evidence of preceding streptococcal
infection
• Recent scarlet fever or
• Positive throat culture for group A Streptococci or
• Streptococcal antibodies in high titers
Confirmed diagnosis
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[Two major or One major + two minor] + { one essential criteria }

36. Revised Jones criteria 2015
• Low risk population: Cases of acute rheumatic fever
≤ 2per 1,00,000 population school-age children or
• prevalence of chronic rheumatic carditis in any age
group lower than or equal to 1/1000 per year.
• High risk population
• Children from communities that exhibit levels above
these would have moderate-to-high risk for
acquiring the disease.

37. Revised Jones criteria 2015
Low risk population High risk population
• Carditis
• Arthritis – only by polyarthritis
• Chorea
• Erythema marginatum
• Subcutaneous nodules
• Carditis (clinical or by Echo
• Arthritis – monoarthritis or Poly.
• Polyarthralgia
• Chorea
• Erythema marginatum
• Subcutaneous nodules
Major criteria

38. Revised Jones criteria 2015
Low risk population High risk population
Minor criteria
• Polyarthralgia
• Fever ≥ 38.5 0 C
• ESR ≥60 mm/ hour,
CRP 3.0mg/dL
• Prolonged PR interval
(if there is no carditis as major
criteria)
• Monoarthralgia
• Fever ≥ 38 0 C
• ESR ≥30 mm/ hour,
CRP 3.0mg/dL
• Prolonged PR interval (if thereis
no carditis as major criteria )

39. Management
• Bed rest
• For patients without Carditis – rest until
Temperature & ESR become normal
• For patients who developed Carditis – rest for
2-6 weeks after the ESR & temperature have
returned to normal

40. Management
• Anti – streptococcal therapy
• Single inj. of benzathine penicillin 1.2 million units IM
(or)
• Inj. Procaine Penicillin 6 lacks im daily for 10 days
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• For those who are allergic or sensitive to penicillin
• Oral erythromycin 20-40mg /kg /day in three divided
doses for 5 days (or)
• Oral Azithromycin 500mg/day for five days

41. Management
• Salicylates
• Aspirin is useful for symptomatic relief
• For children: Started at 60mg/kg/ day in six divided doses.
• For adults 100mg/kg , increased gradually up to 8 grams per day
until the drug produces side effects or clinical improvement
• Aspirin should be continued at this dose until ESR comes to
normal,
• Then dose is gradually tapered
• Side effects
• Mild toxicity : Nausea, tinitus, deafness, vomiting,
• Severe: Tachypnoea, acidosis

42. Management
• Corticosteroids
• Indications
• Patients who have severe Carditis, manifested by heart
failure not responding to aspirin
• Patients with severe arthritis not responding to aspirin
• Prednsolone is given at a dose of 60-120 mg /day in four
divided doses until ESR is normal
• It is then tapered off gradually over a period of 4-6
weeks

43. Management
• Supportive therapy
• Treatment of
– Heart failure
– Heart blocks
– Chorea

44. Prevention of rheumatic fever
• Primary prevention –
• Secondary prevention-

45. Prevention of rheumatic fever
• Primary prevention –
– Accurate diagnosis and treatment of pharyngitis with
Group A Beta Hemolytic streptococci with
– inj Procaine Penicillin 6 lacks IM daily for 5 days
– Sore throat in children in close communities where
rheumatic fever is endemic, all children 5-12 years to
be treated for sore throat

46. Prevention of rheumatic fever
• Secondary prevention
• Rheumatic fever prophylaxis to be offered to all patients
who have documented diagnosis of rheumatic fever
• Duration of prophylaxis
• If No carditis – for 5 years or until 18 years of age
• If Resolved carditis – for 10 years or until 25 years of age
• If Severe RHD – offered for 40years or lifelong

47. • Regimen offered
• Benzathine penicillin 12lacks Units IM once in 3 weeks
(or)
• Oral penicillin V 500mg twice a day
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• Erythromycin 250mg twice a day orally
• Azithromycin 500mg once day (for patients who are
allergic to penicillin)

48. What next

50. Thank you