The document provides current recommendations for treating rheumatic fever in children based on consensus guidelines from experts in India. It discusses the diagnosis and management of acute rheumatic fever and rheumatic heart disease. Key points include guidelines for diagnosing and treating streptococcal throat infections, diagnosing acute rheumatic fever, and treatment approaches for complications including infective endocarditis prophylaxis and secondary prophylaxis.

1. CARDIOLOGY UPDATE 2009
CURRENT RECOMMENDATIONS
on
t r e a t in g
RHEUMATIC FEVER
M. Zulfikar Ahamed
Professor & HOD , Pediatric Cardiology
GMC, Thiruvananthapuram

2. CONSENSUS GUIDELINES
on PEDIATRIC ACUTE RHEUMATIC
FEVER & RHEUMATIC HEART DISEASE
Working Group on Pediatric RF and
Cardiology Chapter of IAP
WRITING COMMITTEE
Anitha Saxena
R.Krishnakumar
S.Radhakrishnan
Rani Gera
Smitha Mishra
M.Zulfikar Ahamed
INDIAN PEDIATRICS, JULY 2008

3. GUIDELINES ON
• Diagnosis & Treatment of
Streptococcal Throat infection
• Diagnosis & treatment of RF
• Treatment of Complications
• Issues like IE Prophylaxis
• Secondary Prophylaxis

4. RF in INDIA
6.4/1000
3.4/1000
3/1000
1.4/1000
0.9/1000
0.5/1000
Shimla Agra Rajasthan UP rural Chandigarh Kochi

5. 2 Decades , RF - SATH
100 103
96
87
64
1985 1993 1998 2003 2008

6. ACQUIRED HEART ILLNESS IN
CHILDREN
SATH, Tvpm ( 2006 )
RF 60%
KD 19%
DCM / Myocarditis 11%
Others 10%

7. EPIDEMIOLOGY of
RF / RHD
it bites & maims
RHD
RF
0 <5 5-14 15-24 25-34 > 34 yrs

8. DIAGNOSIS of RF
Mature
clinical Laboratory Echo
judgment support cardiography
FRAME WORK - DJC ’92 / WHO 2OO4

9. MODIFIED DJC 1992
Major Carditis
Polyarthritis
Chorea
Subcutaneous Nodule
Minor Erythema Marginatum
Clinical
fever
polyarthralgia
Laboratory
Elevated Acute Phase reactants
ESR
CRP
Prolonged PR
Supporting evidence Positive throat culture / Rapid antigen /
of GABS infection Elevated streptococcal antibody titer

10. WHO 2002-2003 DIAGNOSIS OF RF / RHD
DIAGNOSIS How ?
Primary RF AHA Recommendations.
2 Major or 1 Major + 2 Minor +
antecedent strep infection
Recurrence RF 2 Major or 2 Minor + 1 Major +
No Valve Disease
antecedent strep infection
Recurrence RF
RHD 2 Minor + antecedent strep
Chorea infection
Insidious Carditis Other Major criteria not required

11. INCIDENCE OF MAJOR
CRITERIA
(JONES)

12. MAJOR MANIFESTATIONS- RF
Then and now
1985 – Kerala - 2003
77
72
61 60
13
10
5 2
0 0
PA RC CH SCN EM

13. ARTHROPATHIES
RA Infectious
SLE
Reactive
Vasculitis Malignancies

14. POLYARTHRALGIA
Downgraded to ‘minor’ in 1956
An important finding in high RF incidence area
More useful in rheumatic recurrence/ rheumatic
carditis

15. POST STREPTOCOCCAL
REACTIVE ARTHRITIS
• Short latent period
• Sluggish response to Aspirin
• Involves older children, young adults
• Can persist longer
Is it Benign ?

16. CARDITIS
Child With A Recently Acquired
Cardiac Problem
Rheumatic Carditis
Myocarditis Inf. Endocarditis

17. DIAGNOSTIC DILEMMAS IN
RHEUMATIC CARDITIS, MYOCARDITIS
& INF ENDOCARDITIS
 Resolved by :-
History
Physical examination
Lab results
ECG
ECHO

18. TERMINOLOGY
1. Recurrence : A new episode of RF following
another GAS infection; occurring
> 6 - 8 wks following stopping treatment
2. Rebound : Manifestations of RF occurring within
4-6 wks of stopping treatment or
while tapering drugs
3. Relapse : Worsening of RF while under
treatment and often with Carditis

19. RF - LAB DIAGNOSIS
APR ESR CRP
Preceding Strep ASO ( Throat swab)
ADNA se B
Rapid Ag
Blood Counts Hb .TC , DC. Smear
Cardiac status CXR .ECG
Others ANA. Blood C & S. Etc
Echocardiography

20. ACUTE PHASE REACTANTS
• Elevated ESR - > 30 mm/hr
• Positive CRP - > 8 mg /dl
• Polymorphonuclear Leukocytosis
- less specific

21. IDENTIFICATION OF
STREP INFECTION
• Throat swab 10 -25 %
• Rapid Antigen test
• Antibodies
ASO
Anti DNAse B
Anti Hyaluronidase

22. ASO
‘Positive’ ASO
ASO is > 320 units in Child
> 240 units in Adult
Tvpm Experience
Rheumatic Carditis 1995 - 70%
All Rheumatic fever 2003 - 73%
Chorea Alone 2001 - 30%

23. ASO - TWIN SITUATIONS
“The Case of Persisting ASO positivity”
“The Case of Isolated elevation of ASO”
Un riddling ?
Know the natural history of ASO
Know that ASO + vity = Previous throat Inf

24. 10 AV BLOCK in RF
Present in 30%
Minor diagnostic laboratory criteria
No correlation to Carditis ?
No correlation to future RHD

25. ECHO IN Ac RF
1. Severity of Carditis ? Bi valvar involvement ?
2.. Sub clinical Carditis ?
3. Occasionally in confirming the diagnosis
of RF

26. ECHO in RF
Ejection fraction - Normal
Mitral Valve involvement 100
90
80
Aortic Valve involvement 70
60
50
40
30
20
10
0
MR AR AR alone

27. A Pixel is worth Thousand of words

32. TREATMENT
RF
DAMAGE
to
HEART
GABS
DAMAGE CONTROL MEASURES

33. RF - TREATMENT
Past to Present
Venesection , Opium, Purgatives 19th century
Sanatoria treatment Early 20th Century
Aspirin Late 19th century
Steroids, Penicillin 20 th Century

34. TREATMENT STRATEGY
• Eradicate GAS
• Anti inflammatory drug therapy
• General Medical Measures
Rest. Diet. CHF . Surgery ?

35. ERADICATION OF GAS
choice
Penicillin V 250 mg 3 - 4 times x 10 days
BPG 1.2 / 0.6 mega units IM once
Erythromycin 40 mg /kg/day divided x 10 days

36. TREATMENT OF SORE THROAT
BPG 0.6 / 1.2 Million units SD
Penicillin V 250 mg q i d x IO days
500 mg q i d x 10 days
Azithromycin 12.5 mgm / kg / OD x 5 days
Cephalexin 50 mgm /kg /day BD x 10 days

37. COST OF
TREATING STREP THROAT
Per course (INR)
• Oral Penicillin (10 D) - 135
• Erythromycin (10 D) - 135
• Azithromycin (5 D) - 65
• Cephalexin (10 D) - 220

38. WHEN TO TREAT RF
With Anti Inflammatory Drugs ?
Occasion Treat
Typical Polyarthritis +
Carditis +
SC Nodules ( + Carditis) +
Chorea; CRP / ESR + ve +?
Chorea; CRP / ESR - ve -

39. TREATMENT OF RF
CHOICE OF THERAPY ( Anti inflammatory)
Aspirin vs Steroids
Aspirin vs Steroids vs IVIG ?

40. TREATMENT OF RF
DURATION OF THERAPY
Empirical
4 wks - 12 wks
Mild Polyarthritis Severe Carditis

41. ASPIRIN vs STEROIDS
To Give or Not to Give ?
TRIALS
Non Randomized Randomized
119 11
117 +
Meta analysis
(5)
?

42. STEROIDS and Rheumatic Carditis
in defense of steroids
• Beneficial role in proliferative stage
• Enhanced role in T- cell mediated Valve injury
• Definitively prevents death

43. TREATMENT
When Do We Administer
Aspirin/Steroids in Rheumatic Fever ?
SAT protocol RF
No Carditis Carditis
( Clinical + ( Clinical / Echo)
Echo)
Significant / Severe
Mild
Aspirin
Aspirin Steroids

44. POLYARTHRITIS
Aspirin 100 mgm / kg /day
3 -4 divided doses x
2 weeks
Followed by 75 mgm/ kg x 2-4 weeks

45. MILD CARDITIS
Aspirin 100 mgm /kg /day 4 divided doses
(4 -6 gm)
x 2 -3 weeks
75 mgm / kg/ day
x 6 -8 weeks

46. WHEN TO SWITCH ?
Aspirin to Steroid
1. No response within 3 -4 days *
2. New Cardiac findings develop
3. Child develops CHF/ CE
4. Child intolerant to Aspirin ??
( Can We Try NSAID ? )

47. SEVERE CARDITIS
Prednisolone
2 mgm /kg /day 2-4 divided doses
(max 60mgm /day )
x 2-3 weeks [ ESR < 30mm / 1hr ]
Taper off 5 mg / 3 days
Add - Aspirin
75 mgm / kg / day x 8 -10 weeks

48. WHAT IS SIGNIFICANT / SEVERE
CARDITIS ?
1 . CHF
2. Cardiomegaly Clinically or
CXR
Clinical
3. Bi valvar Involvement Echo
4. Pericardial Rub

49. RF IN ADULT
• Polyarthritis is the predominant event
• Carditis - lower incidence
• Chorea ; SC Nodes rare
• Other arthropathies may meet Jones Criteria
• PSRA is a well defined entity

50. RF IN ADULT
Polyarthritis ; No Carditis /
Mild Carditis :
Aspirin 4-6 gm / day divided
doses x 2 wks
Taper to 50 mg / kg / or
75 % x 4 -6 wks

51. RF IN ADULT
Carditis –Significant
Prednisolone 2 mg /kg / day
max: 80 mg /day divided
x 2 wks
Taper
Add aspirin 50 mgm/ kg /day

52. ADULT DOSES
• Aspirin 6 gm /day / 4 divided doses
tapering dose
75 % initial dose or 50mg/kg/day
• Prednisolone 2 mgm /kg/ day
max: 80 mg /day

53. Can We Use other NSAIDS ?
Possible in Polyarthritis
Naproxen
Ibuprofen

54. NSAID / OTHES
• Naproxen 10 – 20 mg/ kg/ day BD
• Methyl Prednisolone
30 mgm / kg / daily x 3
doses

55. CHOREA - DRUGS
1. Haloperidol
0.25 – 0.5 mgm / kg /day
2. Diazepam
0.25 – 0.5 mgm/ kg / day
3. Valproate
15 mgm / kg / day

56. REST
Guidelines
Clinical Rest Ambulation Schooling
Polyarthritis 2 wks > 2 wks 6 wks
Carditis - No CHF /CE 4 wks > 4 wks 6-12 wks
Carditis -CHF0 /CE + 6 wks > 6 wks 12 wks
Carditis CHF + /CE+ 6 wks > 6 wks 4 wks - post
stopping

57. TREATMENT OF CHF IN RF
Issues
• Use of Digoxin - Needed ?
- Modification of dosage
• Use of ACEI - Standard practice
• Use of Diuretic - in pulmonary congestion
• Use of Dobutamine / Nitroprusside
- in refractory CHF

58. Ac RF CARDITIS
- Severe / Refractory CHF ?
• Inotrope IV (Dobutamine)
• NTP infusion - especially in Chordal rupture
• IVIG ??
• Methyl Prednisolone ?
• Emergency Surgery Repair
Replacement

59. NEW MODES OF TREATMENT
? IVIG
?? Valproate for chorea
? Anti- cytokines - adjuvants
Methyl Prednisolone
1.Intolerant to oral steroids
2. Fulminant Carditis

60. PREVENTION IN RF
Primordial Preventing Strep Throat - Vaccine ?
Primary Treating Strep Throat infection
Secondary Preventing Rheumatic recurrence
by chemoprophylaxis
Tertiary Treating RHD

61. SECONDARY PROPHYLAXIS
Continuous chemoprophylaxis to prevent
recurrence in a patient who had an initial
attack of RF
STRATEGY
Chemoprophylaxis Treat breakthrough Infection

62. SECONDARY PROPHYLAXIS
-What to Give ?
BPG 1.2 million U IM 3 weekly
Penicillin V 250 mg BD PO daily
Erythromycin 250 mg BD PO daily
Sulfadiazine 500 mg OD PO daily
1000 mg

63. COST OF
SECONDARY PROPHYLAXIS
Per month ( INR)
1. Benzathine Penicillin - 35
2. Oral penicillin - 210
3. Erythromycin - 200

64. HOW LONG TO GIVE ?
RF; No Carditis 5 years from last Episode
or till 18 / 21 years
RF, Carditis ;
No residual RHD 10 years from last episode ;
or till 25 years
RF, Carditis; RHD 10 years from last episode ;
or till 40 years
/ lifelong

65. RHEUMATIC CHOREA
NO ACCESS TO ECHO ACCESS TO ECHO
NO MURMUR
TILL 25 YEARS
No Valve involv Valve involv
Till 18/ 21 years 25 - 40 yrs

66. Post Strep Reactive Arthritis
What to be done ?
ECHO
Normal Abnormal
Secondary Prophylaxis Secondary Prophylaxis
as for RF
1 year (LP) 5 years ? (HP)

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