Rheumatic fever is an autoimmune disorder caused by a prior streptococcal infection of the throat or skin. It is characterized by inflammation of the heart (carditis), joints (arthritis), brain (chorea), or subcutaneous tissue (erythema marginatum or subcutaneous nodules). Diagnosis is based on the Jones criteria of symptoms occurring 1-5 weeks after strep infection, plus evidence of a recent strep infection. Treatment involves antibiotics to prevent recurrent strep infections, along with aspirin or steroids for arthritis and carditis. Long-term antibiotic prophylaxis is also needed to prevent future episodes of rheumatic fever.

1. Rheumatic fever
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2. Content
Disease overall view
Precipitating factors
Diagnosis
Management
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3. Disease overall view
Rheumatic fever is a systemic autoimmune
disorder related to prior streptococcal infection
The development of rheumatic fever is preceded by the
upper respiratory tract infection (tonsillitis or pharyngitis)
with Group A β-hemolytic streptococci.
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4. Disease overall view
Autoimmune mediated by: Antigenic similarity: Abs
formed against streptococcal Ags react with native
human tissue antigens(autoimmune tissue
damage)
The latent period between streptococcal infection
& onset of RF is 1-5 wks.
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5. Precipitating factors
Age: between 5-15 yrs.
Sex: equal except for rheumatic chorea (common
in females).
Socio-economic factors: common in developing
countries &low social classes (overcrowding &bad
hygiene).
 Recurrent and severe recent strept. infections
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6. Diagnosis
Jone’s criteria
Polyarthritis: w/ low grade fever, large joints,
migratory - often 1 at a time, No permanent
dysfunction. (>75%)
Carditis, pericarditis, cardiomegaly, or
valvulitis (the most serious manifestation &
often results in permanent damage).
(~50%)
plus
Evidence of a recent group A Strep infection
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7. Diagnosis
Jone’s criteria
Chorea: late occurrence, 1- 6 months after
the infection, self-limiting, resolves in 1- 3
months. (~30%)
plus
Evidence of a recent group A Strep infection
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8. Diagnosis
Jone’s criteria
Erythema Marginatum:
“classic” truncal rash, migratory - appears &
disappears within hours.
 (pink rash – irregular red edges serpiginous
borders– clear center) (~10%)
plus
Evidence of a recent group A Strep infection
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9. Diagnosis
Jone’s criteria
Subcutaneous Nodules:
Late occurrence ( months after infection)
 Painless small nodules over bony
prominences:
Elbows
Knees
spine. (1- 2%)plus
Evidence of a recent group A Strep infection
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10. Diagnosis
Jone’s criteria
Fever
Arthralgia (mild pain without objective
findings): Can only be considered without
finding of arthritis
Elevated acute-phase reactants: ESR, C-
reactive protein.
plus
Evidence of a recent group A Strep infection
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11. Diagnosis
Anti streptococcal Abs are investigated:
ASO
Anti-deoxy-ribonuclease (DNAse) B
Anti-hyaluronidase
Heart reactive antibodies
Tropomyosin is elevated in acute rheumatic
fever.
plus
Evidence of a recent group A Strep infection
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12. Diagnosis
Diagnosis with rheumatic fever if you meet two
major criteria, or one major and two minor criteria,
and have signs that you've had a previous strep
infection.
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Work ups

13. Complications
Early complications:
( Heart failure, Arrhythmias)
Late complications:
(Rheumatic valvular lesions, rheumatic activity)
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14. Management
Acute statePrevent further attacks
2ry1ry
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15. Management
Prevention
1ry prevention by treating Strep. Pharyngitis:
The penicillin is the drug of choice because:
Narrow spectrum of activity
Long standing efficacy
Low cost
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16. Management
Prevention
IM benzathine penicillin:
1. 0.6 Millions Unit IM 1 Time (< 27 Kg)
2. 1.2 Millions units IM 1 Time (>27 Kg)
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17. Management
Prevention
PO - Phenoxymethyl Penicillin (Penicillin V)
1. Children (50mg/kg/day) 250 mg (2-3
times/day) x10 days
2. Adolescent /Adult 500 mg TID x10 days
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18. Management
Adverse effect of penicillins
Allergic reaction:
Anaphylaxis, Urticaria
Angioedema, rash.
GIT:
Diarrhea, vomiting & oral thrush
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19. Management
Adverse effect of penicillins
Renal:
Acute interstitial nephritis
CNS:
Convulsion & fever
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20. Management
Adverse effect of penicillins
Site of injection:
Pain with IM route, inflammation at IV-site
Blood:
Hemolytic anemia, thrombocytopenia,
neutropenia
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21. Management
In case of penicillin allergy use:
Oral erythromycin:
(20-40 mg/kg/day)(2-4 times/day)(up to
1gm/day) for 10 days.
Newer macrolides
Oral cephalosporins
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22. Management22

23. Management
Antibiotics “NOT” Recommended for Strep.
Pharyngitis:
Sulfonamides, Trimethoprim, Sulfamethoxazole (not
eradicate GAS).
Tetracyclines / Doxycycline / Minocycline.
(High prevalence of resistant strain)
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24. Management
2ry prevention starts after diagnosis of RF:
Rheumatic Fever requires “continuous” prophylactic
antibiotics due to:
1. Increased “susceptibility” to recurrences
2. Increased “severity” of recurrences
3. “Asymptomatic nature” of Strep. Infections.
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25. Management25
Or Whichever is longer

26. Management26
Treatment of:
Arthritis
Carditis
Chorea

27. Management27
Treatment of:
Arthritis
Relieves by aspirin within 24-48 hrs of pain
The diagnosis is questionable if aspirin isn’t
effective
Mild arthritis may respond to paracetamol
alone

28. Management28
Treatment of:
Arthritis
Aspirin dose for pediatrics:
90-130 mg/kg/day in equally divided doses
every 4-6 hrs, up to 5.5 gm/day.

29. Management29
Treatment of:
Arthritis
Salicylate serum levels (20-25 mg/mL)
After 1-2 wks, the dosage is decreased to 60-
70 mg/kg/day and given for 1-6 wks or longer
if necessary, then gradually withdrawn over 1-2
wks.

30. Management30
Treatment of:
Arthritis
Naproxen/ Ibuprofen/ paracetamol are
potential alternative

31. Management31
Treatment of:
Carditis
Avoid strenuous exercise
Treat symptoms of heart failure
Steroids are preferred over salicylates
Treatment is guided according to clinical
status of the patient

32. Management32
Treatment of:
Carditis === severe
case < use prednisolone
2 mg/kg /day 2-4 divided doses (max 60mgm /day )
2-3 wks [ ESR < 30mm / 1hr ]
Taper off 5 mg / 3 days
Add - Aspirin 75 mgm / kg / day x 8 -10 weeks

33. Management33
Clinical Severity
 Arthralgia or mild
arthritis; no carditis
 Mod./severe arthritis;
no carditis OR carditis
without failure
Treatment
 Analgesics only
 Aspirin 100 mg/kg/d
with meals for 3 wk
(or longer up to 6 wk);
then taper gradually
over 6 wk

34. Management34
Clinical Severity
 Carditis with failure;
with or without joint
manifestation
Treatment
 Prednisone 40-60
mg/d,  if necessary;
after 3 wk, slow
tapering over 3 wk,
continue aspirin for 4
wk after d/c of
prednisone.

35. Management35
Treatment of:
Rheumatic chorea
Haloperidol : initial dose 0.5 to 1 mg and 0.5
is added every 3 days for Maximal effect or
until a maximal dose of 5 mg/ day
Sodium valproate: (15 to 20 mg/kg per day)
is also effective.

36. Management36
Treatment of:
Rheumatic chorea
Diazipam 0.25-0.5 mg/Kg/day

37. Management37
Treatment of:
Rheumatic chorea
In resistant cases, plasmaphersis iv
Immunoglubulins.
Steroid therapy is generally not effective in
Sydenham’s chorea.

38. thanks
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