This document provides information on rheumatic fever, including its:
1) Historical background, epidemiology, pathogenesis, pathology, and clinical features such as polyarthritis, pancarditis, chorea, and subcutaneous nodules.
2) Methods of diagnosis including evidence of preceding streptococcal infection through throat culture or elevated antibody titers, as well as detecting rheumatic activity with elevated inflammatory markers.
3) The role of echocardiography in diagnosis and its increased sensitivity over clinical exam alone.
8. India
S Padmavati
Director, National Heart Institute, New Delhi,
India
In 2000, in a school survey involving 3963
children from the district of Kanpur, the
prevalence of RHD was 4.54 per 1000
(Urban 2.56 and Rural 7.42).
The prevalence of RF was 0.75 per 1000
(Rural 1.20, Urban 0.42)
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9. EPIDEMIOLOGY
2000 - 2004
HOSPITAL BASED SURVEYS :
Agarwal et al (varanasi) : Decreasing
(8.4% - RHD & 1.1% RF)
Despande et al (Mumbai): No change
Mishra et al (cuttack) : No change
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10. EPIDEMIOLOGY
PREVELANCE :
2 million at present
INCIDENCE :
50 000 new cases every year
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11. PATHOGENESI
S
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12. STRUCTURE OF Group –A
Beta Hemolytic Streptococcus
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13. Group - A Streptococcus
Two highly conserved epitopes within M protein
divide GAS immunologically into
Class I (throat) Class II (skin) strains.
All RF strains fall clearly into Class I throat
strains
The site of infection must be pharyngeal .
Regardless of how virulent an invasive strain may be,
ARF does not result when it is introduced extra-
pharyngeally, e.g. through skin lesions or wound
infections
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15. CO-PATHOGENS
Burch et al & Pongpanich et al :
(1970) (1976)
Serological evidence of Cox B viruses
in patients with rheumatic fever
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16. GENETIC
PREDISPOSITION
Specific B - cell alloantigen
HLA DR 3 - Indians
Moari races in New Zealand &
Samoans in Hawaii
High concordance in twins
Increased risk in families with H/O RF
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17. ENVIRONMENT
Low socio-economic group
Urban slums
Poor accesibility to health care
Over crowding
Unclean environment
Mostly seen in developing
countries
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23. ASCHOFF BODY
Granuloma
Central fibrinoid
necrosis
Surrounded by
lymphocytes,
Antischkow cells
and Plasma cells
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30. ORDER OF VALVE
INVOLVEMENT
Mitral
Aortic
Tricuspid
Pulmonary
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31. INTERNATIONAL SERIES BY
BONOW
PURE MS : 25 %
PURE MR : 10 %
MS / MR : 25 %
AORTIC : 8%
ALL VALVES : 7%
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32. CLINICAL FEATURES
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34. LATENCY
From onset of sore throat to onset of
initial attack of rheumatic fever is
1 – 5 weeks
for recurrent attacks
Median of 19 days & shorter
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35. LATENCY
Joint manifestations are first to occur
- heralding onset of disease
Carditis occurs within 2 weeks
- is apparent when patient is first seen
Subcutaneous nodules appear 4 weeks or more
after onset of symptoms
Chorea may appear 2 to 6 months later
Erythema marginatum occurs both early & later
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36. MODE OF ONSET
Variable
Abrupt onset
with fever & acute polyarthritis
Insidious or sub clinical
in mild indolent carditis
May present with CCF
May present atypically with acute abdomen due
to peritoneal inflammation
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37. POLYARTHRITIS
Most common & Least specific
severe in adults
Large joints ; asymetrical
Flitting- involves joints after joints
Fleeting - Lasting for short time
3 days - 1 week
No residual damage
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38. POLYARTHRITIS
Responds dramatically to aspirin
Severity inversely related to carditis
(Feinstein & Spagnuola et al – 1962)
JACCOUDS ARTHRITIS :
Smalljoints
Produces residual damage
Seems to be related to RF
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39. PANCARDITIS
More severe in the young
Sub clinical to fulminant
ENDOCARDITIS :
AR : 20 %
MR : 75 %
: due to - Valvulitis
- MVP (anterior leaflet)
- Annular dysfunction
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40. ENDOCARDITIS
Clinical
Evidence of
Endocaritis :
Apical holosystolic murmur
Carey coomb’s murmur
Early diastolic murmur
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41. MYOCARDITIS
Clinical evidence of Myocarditis :
Cardiomegaly
Clinical features of CHF
Gallop rhythm
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42. PERICARDITIS
Clinical evidence of Pericarditis :
Pericardial rub
Associated with endocarditis
Indicates severe carditis
(High rheumatic activity)
No residual constriction
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43. CHOREA
Occurs 3 months later than other RF features
- spontaneous resolution
Duration : variable ( upto 6 months)
Often in prepuberal girls
Neuropsychiatric disorder
Seen in 5 - 15 % cases
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44. CHOREA
ST. VITUS DANCE
25 - 30 % develop RHD particularly MS
(Bland et al – 20 years follow up)
Multiple purposeless movements of legs and
hands
(also involves face)
on exertion & absent during sleep
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45. DD FOR CHOREA
HABITUAL SPASMS
WILSONS DISEASE
POST ENCEPHALITIS
HYSTERESIS
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46. SUBCUTANEOUS
NODULE
FIRM
PAINLESS
0.5 – 3 cm IN SIZE
IN CROPS ( OVER EXTENSORS)
DISAPPEAR IN 12 WEEKS
ALWAYS ASSOCIATED WITH CARDITIS
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49. ERYTHEMA
MARGINATUM
Rare (< 1 %)
Bikini distribution
Evanescent
vanishing
Non pruritic
50. OTHER MANIFESTATIONS
EPISTAXIS
ABDOMINAL PAIN
- Occurs in 5% cases
- Clinical importance
Often appear hours or days before major
manifestations
Acute abdomen [ appendicitis ] to be excluded
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51. FEVER
Relatively common But nonspecific
Low grade; subside without treatment in 1-2wk
Associated with constitutional symptoms
Lab indices are high even after fever subsides
Remission does not exclude rheumatic activity
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52. ECG CHANGES
Seen in 2/5th patients [ Disciascio(1980)]
PR interval ;
QT interval ;
AV blocks
Does not correlate with organic murmurs,
prognosis or residual heart disease
Nonspecific & occur in many other infection
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53. LAB INVESTIGATIONS
Monitoring the Detecting the
antecedent
inflammatory activity infection with
streptococcus
There is no single diagnostic test
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54. EVIDENCE OF STREPTOCOCAL
INFECTION
TH ROAT SWAB CULTURE :
Only in Minority of cases
ASO TITRE :
elevated from 7 - 10 days
rise and fall rapidly
>240 todd units (adults)
>330 todd units (children)
Antibiotics/steroids/liver disease
affect the titre
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55. EVIDENCE OF STREPTOCOCAL
INFECTION
ANTI-DNAase B TEST :
# > 120 todd units (adults)
# > 240 todd units (children)
# used when ASO titre is not conclusive
# remains elevated for long time
STREPTOZYME TEST :
Detects antibodies against streptococcal
antigen
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56. RHEUMATIC ACTIVITY
DETECTION
Activity considered ended only when both ESR
& CRP become normal
and remain so for 2 weeks after stopping drugs
Fever & tachycardia subside long before lab
reactants decline
Joint symptoms & active carditis do not occur
after ESR & CRP decline
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57. RHEUMATIC ACTIVITY
DETECTION
CRP more specific than ESR
Usually lasts for 3 months
Longer in patients with valvular involvement
In 5% cases rheumatic activity persist longer
than 6 months
termed CHRONIC RHEUMATIC FEVER
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58. ECHOCARDIOGRAM
Abernathy et al:
echo allowed earlier diagnosis of carditis
Veasy et al :
echo increased the sensitivity of detecting
carditis from 72% to 91%
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59. ECHOCARDIOGRAM
Differentiates between innocent murmur and
Rheumatic MR
Detects MVP due to Rheumatic fever
(Wu et al – JACC 1994)
- AML
- Elongated chordae
- No myxomatous thickening
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60. ECHOCARDIOGRAM
Cost effectiveness and the additional
workload have to be validated
Vasan et al (Circ . 1994 ):
showed no
additional detection of carditis by echo
than by clinical detection
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61. OTHER INVESTIGTIONS
Endomyocardial biopsy – to establish
the myocarditis
not likely to provide additional informations
Radionuclide imaging-
- Gallium-67 imaging has better diagnostic
characteristics than antimyosin scintigraphy
- the results confirm that rheumatic carditis is
infiltrative rather than degenerative in nature
- not suitable for routine investigation
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62. DUCKETT JONES
CRITERIA
ORIGINAL (JAMA 1944)
MAJOR MINOR
Carditis erythema mariginatum
Chorea fever / epistaxis /
Arthralgia abdominal pain
S/C Nodule WBC / ESR / CRP
Preexisting RF
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63. DUCKETT JONES
MODIFIED
CRITERIA
:1956 - AHA
Arthritis : Included as – major
criteria
Erythema marginatum: Included as – major criteria
REVISED : 1965 /84 - AHA
Recent streptococcal infection is included as essential
criteria
WHO : 1988
UPDATED : 1992 - AHA
WHO CRITERIA : 2003
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64. DUCKETT JONES
CRITERIA
WHO CRITERIA FOR RF AND RHD- 2003
MAJOR MINOR
Carditis Clinical
Polyarthritis - Fever
Chorea - Arthralgia
S/C Nodules Laboratory
Ery. Marginatum - Leucocytosis
- Elevated : ESR /CRP
ECG - Increased
PR interval
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65. DUCKETT JONES
CRITERIA
Supporting evidence of antecedent
streptococcal infection Within the last 45
days
- positive Throat culture
- Rapid streptococcal antigen test
- Elevated or Rising ASO Titer
- Recent scarlet fever
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66. Diagnostic categories: WHO 2003
PRIMARY RF :
2 major or 1 major and 2 minor + evidence of preceding Gr-A
streptococcal infection
RECURRENT ATTACK OF RF WITHOUT
ESTABLISHED RHD
2 major or 1 major and 2 minor + evidence of preceding Gr-A
streptococcal infection
RECURRENT ATTACK OF RF WITH
ESTABLISHED RHD
2 minor + evidence of preceding Gr-A streptococcal infection
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67. Diagnostic categories: WHO 2003
Rheumatic chorea
Insidious onset rheumatic carditis
Other major manifestations or evidence of
Group-A streptococcal infection not required
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68. Diagnostic categories: WHO 2003
Chronic valve lesions of RHD
Patients presenting first time with pure MS
or mixed mitral valve disease and /or
aortic valve disease
Do not require any other criteria for
diagnosis as having RHD
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70. BEYOND JONES
CRITERIA
Not a substitute for clinical judgment
Not meant to predict course or severity
Useful for initial diagnosis only
Exceptions :
- Chorea
- Isolated indolent carditis
- Recurrence with RHD
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71. APPLYING JONES CRITERIA
2 major criteria is stronger than
One major and 2 minor
Arthalgia cannot be used as minor criteria when
arthritis is present
Prolonged PR cannot be used as a minor criteria
when clinical carditis is present
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72. APPLYING JONES
CRITERIA
Absence of evidence of an antecedent
Group-A Beta-hemolyticus Streptococci is a
warning that RF is unlikely
Possibility of early suppression of full clinical
manifestations by drugs should be sought
during history taking
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73. RECURRENCE
Cardiac status deteriorates with each new attack
Younger the patient - higher recurrence rate
Recurrence decreases with passage of time –
. - 50% within first year
- only 10% after 5 years
Recurrence more in those with valvular lesion
Increase antibody response associated with high
recurrence rate
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74. RECURRENCE
Clinical manifestations in recurrence tend to
mimic those in preceding attack
Recurrence distinguished from rebound or
exacerbation if interval of 3 months freedom
of rheumatic activity
Valve stenosis at diagnosis indicates
recurrence
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75. RHEUMATIC FEVER
Licks the Joint and Bites the Heart
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