1. DIAGNOSIS & MANAGEMENT OF RHEUMATIC FEVER
DR.SANDEEP R
SR CARDIO
87 slides

2. Introduction
Rheumatic fever( RF) - a delayed autoimmune reaction in genetically predisposed individuals to group A, β-hemolytic, streptococcal (GABHS) pharyngitis characterized by inflammation of several tissues that gives rise to typical clinical characteristics including
1)Carditis/ valvulitis
2)Arthritis
3)Chorea
4)Erythema marginatum
5)Subcutaneous nodules
Residual damage only in the heart
Latent period of 3 weeks(1 – 5 wks) b/w GABHS infection & ARF
3%-6% of any population

3. MAJOR MANIFESTATIONS

4. CARDITIS Incidence varies from 50%-60%
The clinical diagnosis of carditis in an index attack of RF is based on
1) Presence of significant murmurs (MR/AR)
2)Pericardial rub
3) Unexplained cardiomegaly with CHF.
Common in young
80% of patients develop it within first 2 weeks of RF

5. ENDOCARDITIS/VALVULITIS
Almost always associated with a murmur of valvulitis
An universal finding in rheumatic carditis, whereas the presence of pericarditis or myocarditis is variable.
Valve Involvments-
92 – 95% mitral valve involvement ( 70 – 75 % isolated MV)
20 – 25% aortic valve involvement( 5-8% isolated AV)
MR – PSM in apex radiating to axilla > with grade 2 (MC FINDING IN CARDITIS)
AR in the absence of MR is uncommon

6. ENDOCARDITIS/VALVULITIS
First attack of RF- apical holosystolic murmur of mitral regurgitation (with or without apical MDM, Carey Coombs), or basal EDM
Pt. with previous RHD- a definite change in the character of any of these murmurs or the appearance of a new significant murmur
Severe MR
Associated with worst prognosis - fatal HF
Incidence of chronic RHD 90%.
Linear relationship between the severity of MR during the first episode of RF and subsequent RHD.

7. ENDOCARDITIS/VALVULITIS
Pathogenesis of severe MR
Valvulitis
Mitral annular dilatation
Leaflet prolapse with or without chordal elongation
Chordal rupture
Carey Coombs murmur
MDM without presystolic accentuation
Associated with severe MR
Due to increased flow through diseased mitral valve

8. MYOCARDITIS No definite evidence of myocarditis!!
Myocarditis is always associated with valvulitis
New onset CMGLY and recent change in cardiac size - most specific sign
No definite evidence of myocarditis!!
- No consistent elevation of cardiac biomarkers
- No evidence of systolic dysfunction
- CHF does not occur without significant valvular lesions
- Radionuclide studies failed to demonstrate significant myocardial staining
- Biopsy in acute RF failed to show cellular necrosis -inflammation was subepicardial, subendocardial and perivascular
- Surgical valve replacement during RF and AHF reverted features of HF
- Aschoff nodules do not contain myocardial cells

9. PERICARDITIS 6 -15 % OF RF Diagnosed by typical pain & friction rub
Always associated with rheumatic valvulitis
May be associated with normal ecg
May be associated with effusion but rarely causes constriction and tamponade
Its presence denote severe carditis

10. POLYARTHRITIS 66-75% of patients MC & most earliest manifestation
Typically involves larger joints – knee, ankle, wrist, & elbow
Involved joints - hot, red, swollen, and tender
Migratory in nature
Not deforming
A dramatic response to small doses of salicylates
MIGRATORY POLYARTHRITIS Arthritis occurs in about 75% of patients with acute rheumatic fever and typically involves larger joints, particularly the knees, ankles, wrists, and elbows. Involvement of the spine, small joints of the hands and feet, or hips is uncommon. Rheumatic joints are generally hot, red, swollen, and exquisitely tender; even the friction of bedclothes is uncomfortable. The pain can precede and can appear to be disproportionate to the other findings. The joint involvement is characteristically migratory in nature; a severely inflamed joint can become normal within 1-3 days without treatment, as 1 or more other large joints become involved. Severe arthritis can persist for several weeks in untreated patients. Monoarticular arthritis is unusual unless antiinflammatory therapy is initiated prematurely, aborting the progression of the migratory polyarthritis. If a child with fever and arthritis is suspected of having acute rheumatic fever, it frequently is useful to withhold salicylates and observe for migratory progression. A dramatic response to even small doses of salicylates is another characteristic feature of the arthritis, and the absence of such a response should suggest an alternative diagnosis. for example, found severe cardiac involvement in 10% of those with
arthritis, 33% of those with arthralgia, and 50% of those with no joint
symptoms

11. POLYARTHRITIS Synovial fluid in ARF usually has 10,000-100,000 WBC/mm3
Exudative with normal glucose & neutrophil predominance
Self limiting & normalizes by 2 – 4 wks
Polyarthritis & sydenham’s chorea never occurs simultaneously
Inverse relationship b/w the severity of arthritis & cardiac involvement

12. SUBCUTANEOUS NODULES Rare 2-20% Freely mobile,painless 0.5 - 2 cm
Occur in crops over bony prominences or extensor tendons
Common locations - elbow,wrist knee,ankle & achilles tendon

13. SUBCUTANEOUS NODULES Self limiting –days to 1 month
Nodules may occur in SLE,RA but they tend to be larger
There is a correlation between its presence & carditis
PATHOLOGY
Fully developed nodules consist of a central zone of fibrinoid necrosis surrounded by a peripheral cellular reaction consisting of histiocytes and fibroblasts
Do not exhibit the pallisading pattern of RA
Behera surveyed 336 casesof RF in 5 yr period in hospital to ascertain frequency of occurrence and clinical evolution of subcut nodules.12.5% incidence,mean no. of nodules 18,12% it lasted more than 8 weeks

14. ERYTHEMA MARGINATUM 3-15%
Erythematous, serpiginous, macular lesions with pale centers that are not pruritic
Multiple lesions primarily on the trunk or proximal extremities,rarely on distal extremities & never on face
It occurs early in course of RF

15. ERYTHEMA MARGINATUM Nonpainful , nonpruritic, blanches on pressure
Accentuated by warming the skin.
Not influenced by antiinflammatory therapy
It is associated with carditis
Nodules & marginatum can occur simultaneously
It is also seen in sepsis,drugrn.,glomerulonephritis

16. CHOREA Sydenham chorea , St.Vitus dance 5 - 36% of ARF
Mc in females, rare > 20 yrs
Isolated, frequently subtle, neurologic behavior disorder
Emotional lability, incoordination, poor school performance, uncontrollable movements, and facial grimacing
Exacerbated by stress and disappears with sleep
Seen occasionally unilateral

17. CHOREA Long latent period
Clinical maneuvers to elicit features of chorea include
(1) demonstration of milkmaid’s grip (irregular contractions of the muscles of the hands while squeezing the examiner’s fingers)
(2) spooning & pronation of the hands when the patient’s arms are extended
(3) wormian darting movements of the tongue upon protrusion
(4) examination of handwriting to evaluate fine motor movements
Do not cause permanent neurologic sequelae

18. CHOREA Rheumatic chorea –marker of future carditis
23% pure rheumatic chorea dvpd MS in 20 yr follow up & 27% in 30 yr period
Chorea is rarely associated with polyarthritis
Inflammatory markers & ASO titres may be normal

19. DIFFERENTIAL DIAGNOSIS

20. MINOR MANIFESTATIONS Arthralgia Fever Epistaxis seen in 4% of cases
constitutes pain in one or more joints without evidence of inflammation, tenderness to touch, or limitation of motion.
Arthralgia + monoarticular arthritis – suggestive of RF
Fever
Temperature > F rectally-diurnal variations are seen
Children with mild carditis and pateints with chorea are afebrile
Epistaxis seen in 4% of cases
Abdominal pain
5% 0f cases - occurs before the appearance of major maniftn
Pain usually epigastric or periumblical & may mimic appendicitis

21. MIMETIC FEATURE OF RHEUMATIC FEVER
Those patients who develop extracardiac manifestation in the initial attack ,there is a less chance for carditis during recurrence whereas if the initial attack is carditis there is a high chance of recurrent carditis

22. POST STREPTOCOCCAL REACTIVE ARTHRITIS
Relatively shorter latent period ( 7 to 10) days
May be persistent or relapsing
Slower response to aspirin
Not associated with other major manifestations
Symmetric invlnt. of large , small joints & axial skeleton
Occ . causation by non GABHS
Secondary prophylaxis for up to 1 year after the onset of their symptoms (Class IIb,LOE C)

23. ?RHEUMATIC PNEUMONIA
An acute inflammatory pneumonitis has been described in patients with RF
Presents as sudden onset respiaratory distress
Associated with carditis
CXR shows a hilar or patchy distributionn
Difficult to differentiate clinically with CHF
Responds to steroids
Uncertainity of its frequency and its existence as a distinct entity

24. JACCOUD’S ARTHRITIS

25. JACCOUD’S ARTHRITIS Chronic postrheumatic fever arthritis
Seen in patients with severe RHD & not associated with evidence of RF
Recovery delayed & assoc. with stiffness of metacarpophalyngeal joints
Characteristic deformity due to periarticular , fascial and tendon fibrosis
Joint disease is inactive with normal ESR & negative RA factor
Deformity characterized by flexion at the metcarpophalangeal joint with ulnar deviation of 4th and 5th fingers & hyperextn of PIP
Initially the deformity is correctable & not assoc with bone destruction

26. PANDAS
Pediatric autoimmune neuropsychiatric disorders associated with streptococcal infections
Autoimmune responses that cross-react with brain tissue in response to a GAS infection
Obsessive-compulsive & tic disorders
No need of secondary prophylaxis (Class III, LOE B).

27. EVOLUTION OF JONES CRITERIA
ORGINAL JONES CRITERIA 1944
MAJOR MANIFESTATIONS
MINOR
MANIFESTATION
CARDITIS
ARTHRALGIA
CHOREA
SUBCUTANEOUS
NODULES
5.H/O OF PREVIOUS DEFINITIVE RF OR RHD
1.FEVER
2.ABDOMINAL PAIN
3.PRECORDIAL PAIN
4.RASHES( ERYTHEMA MARGINATUM)
5.EPISTAXIS
6.PULMONARY FINDINGS
7.LAB FINDINGS
A.ECG
B.MICROCYTIC ANAEMIA
C.ELEVATED TLC
D.RAISED ESR
Dr .jones made no distinction between arthralgia and arthritis
He added past h/o rf as major and EM as minor
Rule that two major or one major + two minor has been preserved in all revisions

28. MODIFIED JONES CRITERIA 1956
MAJOR MANIFESTATION
MINOR MANIFESTATION
1.CARDITIS
2.POLYARTHRITIS
3. CHOREA
4.SUBCUTANEOUS NODULES
5.ERYTHEMA MARGINATUM
1.FEVER
2.ARTHRALGIA
3.PROLONGED PR INTERVAL
4.INCREASED ESR,CRP OR LEUKOCYTOSIS
5.PREVIOUS H/O OF RF OR RHD
6.EVIDENCE OF PRECEEDING BETA HEMOLYTIC STREPTOCOCCAL INFECTION
POLYARTHRITIS WAS ADDED AS MAJOR CRITERIA & ARTHRALGIA ADDED TO MINOR
MINOR SYMPTOMS LIKE ABDOMINAL PAIN & EPISTAXIS WERE EXCLUDED
Prolongation of pr was only ecg criteria
Addition of evidence of streptococcal infn to minor criteria

29. REVISED JONES CRITERIA 1965
MAJOR MANIFESTATION
MINOR MANIFESTATION
1.CARDITIS
2.POLYARTHRITIS
3. CHOREA
4.SUBCUTANEOUS NODULES
5.ERYTHEMA MARGINATUM
1.FEVER
2.ARTHRALGIA
3.PROLONGED PR INTERVAL
4.INCREASED ESR,CRP OR LEUKOCYTOSIS
5.PREVIOUS H/O OF RF OR RHD
SUPPORTING EVIDENCE OF STREPTOCOCCAL INFECTION MADE AN ABSOLUTE CRITERIA
DONE TO REDUCE MISDIAGNOSIS
EXCEPTION TO JONES – RHEUMATIC CHOREA & LOW GRADE CARDITIS
ASO WAS MOST RELIABLE BEST STANDARDIZED ANTIBODY TEST > 333 > 5YR DIAGNOSTIC +
SUPPORTING EVIDENCE OF PRECEEDING STREPTOCOCCAL INFECTION
,H/O RECENT SCARLET FEVER;POSITIVE THROAT C/S FOR GROUP A STREPTOCOCCUS;
INCREASED ASO TITRE

30. REVISED CRITERIA OF 1965 WAS REVIEWED AND PUBLISHED AGAIN IN 1984
INFERENCES MADE
1) Premature administration of antiinflammatory drugs may modify the clinical picture
2) Usefulness of echo in distinguishing pt with MVP & BICUSPID VALVE from RHD
3) PR prolongation- not an indication of carditis nor does it corelate with development of RHD
JONES CRITERIA WAS NOT DIAGNOSTIC-INDOLENTCARDITIS,RECURRENT RF,CHOREA

31. JONES CRITERIA UPDATE 1992 + MAJOR MANIFESTATION MINOR MANIFESTATION
1.CARDITIS
2.POLYARTHRITIS
3. CHOREA
4.SUBCUTANEOUS NODULES
5.ERYTHEMA MARGINATUM
1.FEVER
2.ARTHRALGIA
3.PROLONGED PR INTERVAL
4.LAB FINDINGS
ELEVATED ACUTE PHASE REACTANTS,INCREASED ESR,CRP
Jones was only for initial attack and exception for jones are reccurent RF,chorea,indolent carditis
Signs & symptoms of recurrent attack may not fit into jones
Past h/o rf excluded
Rapid antigen test added
Role of echo was seen
In 1992 update noted that arthralgia may be the only clinical manifestation of recurrent attackof RF SO WITH EVIDENCE OF RECENT STREPTOCOCCAL INFN. RECURRENCE CAN BE DIAGNOSED +
SUPPORTING EVIDENCE OF PRECEEDING STREPTOCOCCAL INFECTION
POSITIVE THROAT C/S FOR GROUP A STREPTOCOCCUS OR RAPID ANTIGEN TEST
ELEVATED OR RISING STREPTOCOCCAL ANTIBODY TITRE

32. EVOLUTION OF JONES CRITERIA

33. WHO 2002 – 2003

34. High-risk groups defined as those living in communities with high rates of ARF (incidence>30
per 100,000 per year in 5–14-year-olds) or RHD (all-age prevalence>2 per 1,000).

36. LAB DIAGNOSIS OF ARF No gold standard diagnostic technique
1)THROAT C/S
initially considered a gold standard for diagnosis of streptococcal infection
LIMITATIONS
1) Difficult to differentiate a carrier from active infection
2) 1/3 of RF has no H/O preceeding pharyngeal infn.
3) Delay in getting culture report

37. LAB DIAGNOSIS 2)RAPID ANTIGEN TEST FROM THROAT SWAB
Specificity is 95% & sensitivity 60 to 90%
3)STREPTOCOCCAL ANTIBODY TEST
Antistreptolysin O (ASO)
Anti DNAase B
Anti hyaluronidase (AH)
Streptozyme(SZ)

38. ASO TEST
Significant antibody response defined as a rise in titre > 2 dilution increments b/w acute phase and convalescent phase
Serum samples obtained 2 to 4 week intervals
ASO Titre of > 240 todd units in adults & > 320 todd units in children> 5 yrs is elevated
Appears 7 to 10 days after the infection with peak detection at 2 & 3 weeks after the onset of RF

39. ASO TEST
All cases of suspected ARF should have elevated serum streptococcal serology demonstrated.
If the initial titre is above ULN, there is no need to repeat serology.
If the initial titre is below the ULN for age, testing should be repeated 10–14 days later.

40. ANTI DNASe B
Anti DNAse B titers begin to rise 1 to 2 weeks and peak 6 to 8 weeks after infection
Antidnase 1:60 in preschool, 1:480 in school age, 1:340 in adult( NORMAL TITRE)
Single antibody test-( only ASO) %
Multiple antibody test-(ASO +ANTIDNase B) %

41. LAB INVESTIGATION 1) B CELL MARKER 2) CRP, ESR
D8/17 monoclonal antibody - 90 to 100% of all patients with RF
Mode of inheritence - Autosomal recessive
2) CRP, ESR
The D8/17 monoclonal antibody appears to be a consistent marker of past rheumatic fever in many
populations. Intermediate levels are detected in first degree relatives, including relatives of patients with
Sydenham’s chorea, suggesting that the B cell marker is inherited.47 An autosomal recessive mode of
inheritance has been proposed.46 Expression is highest in patients with ARF, suggesting the alloantigen
may be upregulated during the disease process. monoclonal antibody designated D8/17 that is
present on the surface of B cells.44,45 This antigen does not appear to be associated with any of the
known HLA antigens. The monoclonal antibody identified the alloantigen on the surface of B cells of
almost all patients with current or past rheumatic fever and only 14% of controls. First degree relatives
had intermediate levels of expression.

42. ECG CHANGES IN ARF
1) Persistent sinus tachycardia & an elevated sleeping pulse rate are signs of carditis
2)Sinus bradycardia
3) PR prolongation
Seen in % of cases
Proposed theory – due to vagal overactivity,myocardial inflmn
No correlation with carditis and future dvpnt. Of RHD
4) High grade AV block ,CHB
5) pericarditis
6) QT prolongation

43. ECHOCARDIOGRAPHY CHARACTERISTIC CHANGES IN RHEUMATIC MITRAL VALVULITIS
CHORDAL ELONGATION
ANNULAR DILATION
AML PROLAPSE
POSTEROLATERAL JET OF MR

44. ECHOCARDIOGRAPHY SUBCLINICALCARDITIS/ ECHOCARDITIS
Patients with suspected acute rheumatic carditis have no clinical murmurs but have documented regurgitation on echocardiography
 Prevalence 0 to 53%

45. ECHOCARDIOGRAPHY ADVANTAGES DISADVANTAGES
1)Superior sensitivity in detecting rheumatic carditis
2) Avoids misdiagnosis
DISADVANTAGES
-Overdiagnosis of physiological valvular regurgitation as an organic dysfn.
-Echocardiographic facilities not widely available
-Ability to detect the recurrence of subclinical carditis not clear
The prevalence of physiological valvular
regurgitation in normal people varied by valve: mitral regurgitation
was present in 2.4–45% of normal individuals (7, 9), aortic regurgitation
in 0–33% (9, 12), tricuspid regurgitation in 6.3–95% (9, 13), and
pulmonary regurgitation in 21.9–92% of normal individuals

46. WHO ECHO CRITERIA FOR CLINICAL CARDITIS
0: Nil, including physiological or trivial regurgitant jet <1.0 cm, narrow, small, of short duration, early systolic at mitral valve or early diastolic at aortic valve.
0+: Very mild regurgitant jet, more than 1.0cm, wider, localized immediately above or below the valve, throughout systole at the mitral valve or diastole at the aortic valve (clinically, no murmur audible).
1+: Mild regurgitant jet.
2+: Moderate regurgitant jet, longer and at a wider area.
3+: Moderately severe regurgitant jet, reaching the entire left atrium (MR) or left ventricle (AR).
4+: Severe regurgitant jet, diffusely into the enlarged LA, with systolic backward flow into pulmonary veins (mitral valve); markedly enlarged LV filled with regurgitant jets (aortic valve).
MARIJON ET AL IN Independent investigators
reviewed all suspected RHD cases using a higher-resolution, nonportable ultrasound system. On-site echocardiography
identified 18 and 124 children with suspected RHD according to WHO and combined criteria, respectively.
After consensus review, 17 were finally considered to have definite RHD according to WHO criteria, and 66 had definite
RHD according to combined criteria, giving prevalence rates of 7.8 (95% confidence interval, 4.6 to 12.5) and 30.4 (95%
confidence interval, 23.6 to 38.5) per 1000 children, respectively (P0.0001, exact McNemar test).

47. WHO CRITERIA FOR SUBCLINICAL CARDITIS
MARIJON ET AL IN 2009
Demonstrated that WHO criteria was inadequate
A significant difference in prevalence of RHD 7.8 (95% confidence interval, 4.6 to 12.5) and 30.4 (95% confidence interval, 23.6 to 38.5) –who vs combined criteria

48. ECHOCARDIOGRAPHY

49. OUTCOME OF SUBCLINICAL CARDITIS
35 PT.WITH CARDITIS

50. WORLD HEART FEDERATION CRITERIA 2012 FOR RHD

51. WHF CRITERIA

52. WHF CRITERIA
AMVL thickness should be measured during diastole at full excursion. Measurement should be taken at the thickest portion of the leaflet, including focal thickening, beading, and nodularity. Measurement should be performed on a frame with maximal separation of chordae from the leaflet tissue. Valve thickness can only be assessed if the images were acquired at optimal gain settings without harmonics and with a frequency ≥2.0 MHz.
‡Abnormal thickening of the AMVL is age-specific and defined as follows: ≥3 mm for individuals aged ≤20 years; ≥4 mm for individuals aged 21–40 years; ≥5 mm for individuals aged >40 years. Valve thickness measurements obtained using harmonic imaging should be cautiously interpreted and a thickness up to 4 mm should be considered normal in those aged ≤20 years.
§Restricted leaflet motion of either the anterior or the posterior MV leaflet is usually the result of chordal shortening or fusion, commissural fusion, or leaflet thickening.
||Excessive leaflet tip motion is the result of elongation of the primary chords, and is defined as displacement of the tip or edge of an involved leaflet towards the left atrium resulting in abnormal coaptation and regurgitation. Excessive leaflet tip motion does not need to meet the standard echocardiographic definition of MV prolapse disease, as that refers to a different disease process. This feature applies to only those aged <35 years. In the presence of a flail MV leaflet in the young (≤20 years), this single morphological feature is sufficient to meet the morphological criteria for RHD (that is, where the criteria state “at least two morphological features of RHD of the MV” a flail leaflet in a person aged ≤20 years is sufficient).
¶In the parasternal short axis view, the right and noncoronary aortic cusp closure line often appears echogenic (thickened) in healthy individuals and this should be considered as normal.

53. MORPHOLOGIC FEATURES OF RHD
Excessive leaflet tip motion is defined as displacement of the tip or edge of the involved leaflet towards the left atrium, resulting in abnormal coaptation and regurgitation .
Excessive MV leaflet tip motion of the AMVL or posterior MV leaflet is the result of elongation or rupture of the primary chords together with annular dilatation, is the predominant mechanism of mitral regurgitation in the setting of acute rheumatic carditis

54. ECHOCARDIOGRAPHY
MITRAL VALVE
THICKNESS
RESTRICTED MV
OPENING

55. ECHO POINTERS OF RF
BEADED APPEARENCE
POSTEROLATERAL JET

58. EXCESSIVE LEAFLET TIP MOTION
RHD
MVP
NORMAL

59. RESTRICTED MV LEAFLET MOTION
RHD
NORMAL

60. VIJAYAS ECHO CRITERIA FOR RF
81% sensitivity with 93% specificity.
Objectives:  The objective of our study, therefore, was to define the potential role of echocardiography in detecting carditis in the setting of acute rheumatic fever
Materials and methods: We performed echocardiography in 452 consecutive patients with acute rheumatic fever, clinically diagnosed by the strict Jones criterions, using the patients as part of a multi-centric and double blinded prospective study.
 Results: Of our 452 patients, 230 were males, and 222 were females. The youngest was aged 1 year 11 months, while the oldest was a 51-year-old female. Out of the 452 cases of acute rheumatic fever, 239 patients (52.8%) had arthritis. Out of 164 cases of clinically diagnosed carditis, only 141 cases had echocardiographic evidence of carditis (85.97%).
The remaining 23 patients (14%) had functional murmurs, tachycardia, or anaemia. Of the patients, 2 also had congenitally malformed hearts. Of 40 patients with rheumatic chorea, 28 (70%) had echocardiographic evidence of carditis or valvitis. Polyarthralgia was seen in 213 cases (47.12%), from which only 38 patients (17.8%) had carditis clinically, albeit that 88 patients (41.3%) showed echocardiographic evidence of subclinical carditis or valvitis. 
Conclusion: Echocardiography, when carried out in patients with acute rheumatic fever diagnosed strictly according to the Jones criterion, can avoid both overdiagnosis and underdiagnosis of carditis. A high incidence of carditis, or subclinical carditis, is detected by echocardiography when performed in patients with rheumatic chorea or arthralgia.

61. ECHO AS A SCREENING TEST
N=2170, Simplified echo criteria vs reference echo criteria
When compared with the reference criteria, the simplified approach yields a
maximum sensitivity of 73% for case detection, with a positive predictive value of 92%.

62. ENDOMYOCARDIAL BIOPSY
Aschoff nodules or histiocytic aggregates considered diagnostic of rheumatic myocarditis
Sensitivity 43% ,specificity of 100%
Myocyte degeneration usually without lymphocytic infiltration and frequently without Aschoff nodules.
Does not provide additional diagnostic information
Not recommended

63. ASCHOFF NODULES

64. RADIONUCLIDE IMAGING GALLIUM-67 SCINTIGRAPHY
ANTIMYOSIN ANTIBODY IMAGING WITH INDIUM -111
Insufficient data to support the use of radionuclide scanning.
Gallium scintigraphy appears to be superior technique whereas the role of antimyosin appears to be equivocal
Inflammation is associated with increased capillary permeability & increased leaking of transferrin .Galium binds to transferrin results in localization at site of inflammation .can demonstrate pericardial,myocardial and endocardial uptake.86% sensitive 100% specific PPV of 100% npv of 90%.
Sarcolemmal desruption in inflammation causes leakage of intracellular molecules.so antimyosin antibody can detect myocardium which is inflammed

65. TREATMENT OF RF

66. TREATMENT OF RF 1)TREATMENT OF GABHS
1) ORAL PENCILLIN V 500MG B.D -10DAYS
2) INJ BENZATHINE PENCILLIN 12LAKH UNITS
3)ERYTHROMYCIN 250MG Q.I.D DAYS
2)TREATMENT OF ARTHRITIS
1)ASPIRIN ONCE DIAGNOSIS IS CONFIRMED WITH 100MG/KG FOR 2 WEEKS AND THEN GRADUALLY TAPERED TO MG/KG FOR ANOTHER 4 WKS
2) NAPROXEN ALTERNATIVE
3) TREATMENT OF CARDITIS
1)NO CHF- ONLY ASPIRIN
2) CHF- STEROIDS AT A DOSE 2MG/KG FOR 4 WEEKS
TO BE OVERLAPPED WITH ASPIRIN WHEN IT IS TAPERED
3) ANTIFAILURE eg. Diuretics ,Ace, beta blockers,digoxin

67. TREATMENT OF RF TREATMENT OF SKIN LESIONS NO SPECIFIC TREATMENT
TREATMENT OF CHOREA
REASSURANCE & SEDATION
NSAID & STEROID HAVE NO ROLE
HALOPERIDOL
CARBAMZEPINE & VALPROATE –REFRACTORY CASES
IVIG & PLASMAPHERESIS-NO BENEFIT
SURGERY
PT WITH REFRACTORY CARDITIS
IDEAL AFTER THE ACUTE INFLAMMATION SETTLES
VALVE REPLACEMENT BETTER THAN VALVE REPAIR
IF MORPHOLOGIC EVIDENCE OF INFLAMMATION
REPAIR MAY CAUSE INCREASED REOPERATION

69. PRIMORDIAL PREVENTION
(i) Improvement in socio-economic status
(ii) Prevention of overcrowding
(iii) Improving nutritional status
(iv) Availability of prompt medical care
(v) Public education regarding
the incidence of anaphylaxis was 1.2/ injections, and
the incidence of death was 0.31/ injections

70. PRIMARY PREVENTION

72. TREATMENT
Post treatment throat cultures 2 to 7 days after completion of therapy are indicated:
If patient remain symptomatic
Whose symptoms recur
Patients who have had RF and are therefore at unusually high risk for recurrence
TREATMENT FAILURE
A second course of therapy in asymptomatic individuals should be considered only for those with previous RF themselves or in members of their families.
Treatment reqd if symptomatic
May require other drugs – clindamycin,rifampicin,amoxclav

73. TREATMENT OF CARRIERS Carriers
Chronic streptococcal carriers (defined as individuals with positive throat cultures for GAS without clinical findings or immunologic response to GAS antigens) usually do not need
to be identified or treated with antibiotics

74. SECONDARY PROPHYLAXIS
Forty-eight consecutive patients (44% males, mean age 29.4 years) with established RHD were randomised into two groups-26 patients received AZT and 22 received oral penicillin. Patients were evaluated at randomisation, at 1 month, 3 months, and 6 months, clinically, serologically and by throat swab culture. End points were absence of streptococcal colonisation, infection or fever at the end of 6 months. During the study, 4 patients (15.4%) in the AZT group developed sore throat and fever, had positive throat culture and positive serology indicating streptococcal infection. None satisfied the criteria for rheumatic fever reactivation. None in the oral penicillin group developed streptococcal infection. In conclusion, weekly 500 mg of AZT is not effective in the prevention of streptococcal throat infection compared to oral penicillin therapy in adult patients with established RHD

75. SECONDARY PROPHYLAXIS
those with moderate or severe carditis, or a
history of valve surgery, who demonstrate
good adherence to less frequent injections;and those who have confirmed breakthroughARF, despite full adherence to 4-weeklyBPG

76. WHO 2002 CRITERIA FOR SECONDARY PROPHYLAXIS
PATIENT WITHOUT PROVEN CARDITIS
FOR 5 YEARS AFTER THE LAST ATTACK, OR UNTIL 18 YEARS
OF AGE WHICHEVER IS LONG
PATIENT WITH CARDITIS
(MILD MITRAL REGURGITATION,
HEALED CARDITIS)
FOR 10 YEARS AFTER THE LAST ATTACK, OR AT LEAST UNTIL 25 YEARS OF AGE WHICHEVER IS LONGER
MORE SEVERE VALVULAR DISEASE
LIFELONG
AFTER VALVE SURGERY. ..
Several factors can influence the risk of RF recurrence, including:
— the age of the patient
— the presence of RHD
— the time elapsed from the last attack
— the number of previous attacks
— the degree of crowding in the family
— a family history of RF/RHD
— the socioeconomic and educational status of the individual
— the risk of streptococcal infection in the are whether a patient is willing to receive injections the occupation and place of employment of the patient (school
teachers, physicians, employees in crowded areas).

79. COMPARISION OF GUIDELINES

80. Oral agents are more appropriate for patients at lower risk for rheumatic fever recurrence
Accordingly, some physicians may consider switching patients to oral prophylaxis when they have reached late adolescence or young adulthood and have remained free of rheumatic attacks for at least 5 years (Class IIb, LOE C).

81. RECURRENCE RISK OF ANTIBIOTICS

82. METAANALYSIS OF SECONDARY PREVENTION
(Wood
et al : RR 0.23, 95% CI: ; Feinstein : RR 0.09, 95% CI: ; Feinstein et al : RR 0.29, 95%
CI: ).

83. METAANALYSIS OF SECONDARY PREVENTION
Problems in the studies
No blinding
2) Many there were crossing over
3) Initial studies used different regimen of oral pencillin
4) Used pencillin g not pencillin V 5)

84. ANTISTREPTOCOCCAL VACCINE
Components of GAS used in vaccine development
1)M- protein
2)GAS C5a peptidase, a major surface virulence factor
3) Fibronectin binding protein sfb1
4) Chimeric peptide J8 from the conserved region of the M- protein
M protein vaccines-less likely to succeed
1) Heterogeneous distribution of strains- varies from place to place and keeps changing even within a closed community in a short period.
2) On the basis of emm typing of M-protein more than 250 strains of GAS can cause infection and provide only strain-specific immunity.
3) GAS has a strong tendency for mutation

85. ICMR PROJECTS

87. SUMMARY
Rheumatic fever is more of a clinical diagnosis but nowadays echo& doppler also has a role
No gold standard diagnostic test
No entity as rheumatic myocarditis
Duration of prophylaxis depends on the high risk factors
Injectable pencillin is better than oral pencillin
School & community based programme required for primary prophylaxis
Echocarditis may become a major criteria in the coming yrs

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