Rheumatic fever clinical features and diagnosis

RHEUMATIC
FEVER
Clinical features
and
diagnosis
DR . SUJIT SAHU
http://cardiologysearch.blogspot.in/

 INTRODUCTION
 HISTORICAL BACKROUND
 EPIDEMIOLOGY
 PATHOGENESIS
 PATHOLOGY
 CLINICAL FEATURES
 DIAGNOSIS


INTRODUCTION

Clinical syndrome

 Acute , non-suppurative inflammatory disease
following Group A Beta Hemolytic
Streptococcal sore throat

 Classified as Connective tissue disease or
collagen vascular disease
affecting the Joints, heart , brain , skin and
subcutaneous tissue

HISTORICAL
BACKROUND
 1604 – Guilleaume (France)
Thomas Syndenham (Eng)
-Polyarthritis
 1605 - Sydenham - St. Vitus Dance
 1761 - Morgani (Italy) – Heart valves
 1813 - W.C.wells – Subcutaneous Nodules
 1818 - Laennec - RHD (clinical)


HISTORICAL
BACKROUND
 1886 - Cheadle - Full syndrome
 1904 - Aschoff - Aschoff Nodule
 1931 - Coburn - Streptococcal assoc.
 1944 - Jones - Criteria
 1951 - Wannamaker (penicillin prophylaxis)


EPIDEMIOLOGY


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)

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

EPIDEMIOLOGY

PREVELANCE :
2 million at present

INCIDENCE :
50 000 new cases every year


PATHOGENESI
S


STRUCTURE OF Group –A
Beta Hemolytic Streptococcus


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

CROSS REACTIVITY


CO-PATHOGENS

 Burch et al & Pongpanich et al :
(1970) (1976)

Serological evidence of Cox B viruses
in patients with rheumatic fever


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


ENVIRONMENT
 Low socio-economic group

 Urban slums

 Poor accesibility to health care

 Over crowding

 Unclean environment

Mostly seen in developing
countries

INTERACTION


PATHOLOGY


INFLAMMATORY RESPONSE

Edematous change

Cellular infiltrate

Fibrinoid necrosis

Aschoff body
(seen only in heart)

 Joints : serositis
Pericardium

 Skin (S/C nodule) : Fibrinoid
Heart degeneration

 Erythema Marginatum : Vasculitis

 Chorea : Vasculitis

ASCHOFF BODY
 Granuloma
 Central fibrinoid
necrosis
 Surrounded by
lymphocytes,
Antischkow cells
and Plasma cells


Initial edema

Hyaline degeneration

Verrucae formation at the edge of leaflets

Prevents approximation Regurgitation

Fibrosis & calcification Stenosis

ORDER OF VALVE
INVOLVEMENT
 Mitral

 Aortic

 Tricuspid

 Pulmonary


INTERNATIONAL SERIES BY
BONOW
 PURE MS : 25 %

 PURE MR : 10 %

 MS / MR : 25 %

 AORTIC : 8%

 ALL VALVES : 7%


 CLINICAL FEATURES


PERCENTAGE
INVLOVEMENT
(Indian Scenario)
 ARTHRITIS : 70 %

 ARTHALGIA : 90 %
 CARDITIS : 70 %
 CHOREA : 08 %
 S/C NODULE : 02
%
 ERYHTEMA MARGINATUM : 01

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


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


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


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


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


PANCARDITIS
 More severe in the young

 Sub clinical to fulminant

 ENDOCARDITIS :
 AR : 20 %

 MR : 75 %
: due to - Valvulitis
- MVP (anterior leaflet)
- Annular dysfunction


ENDOCARDITIS

Clinical
Evidence of
Endocaritis :

 Apical holosystolic murmur
 Carey coomb’s murmur
 Early diastolic murmur


MYOCARDITIS

 Clinical evidence of Myocarditis :

Cardiomegaly
Clinical features of CHF
Gallop rhythm


PERICARDITIS

 Clinical evidence of Pericarditis :
Pericardial rub

 Associated with endocarditis

 Indicates severe carditis
(High rheumatic activity)

 No residual constriction


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


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

DD FOR CHOREA

 HABITUAL SPASMS

 WILSONS DISEASE

 POST ENCEPHALITIS

 HYSTERESIS


SUBCUTANEOUS
NODULE
 FIRM
 PAINLESS
 0.5 – 3 cm IN SIZE
 IN CROPS ( OVER EXTENSORS)
 DISAPPEAR IN 12 WEEKS

ALWAYS ASSOCIATED WITH CARDITIS


SUBCUTANEOUS
NODULE


ERYTHEMA
MARGINATUM
 Rare (< 1 %)

 Bikini distribution

 Evanescent
vanishing

 Non pruritic

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

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


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


LAB INVESTIGATIONS

Monitoring the Detecting the
antecedent
inflammatory activity infection with
streptococcus

There is no single diagnostic test

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

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


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


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

ECHOCARDIOGRAM
 Abernathy et al:
echo allowed earlier diagnosis of carditis

 Veasy et al :
echo increased the sensitivity of detecting
carditis from 72% to 91%


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

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


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

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


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

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


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


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



 Rheumatic chorea
 Insidious onset rheumatic carditis

Other major manifestations or evidence of
Group-A streptococcal infection not required


 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

DUCKETT JONES
CRITERIA

 Specificity – 97 %

 Sensitivity – 77 %


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


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


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


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

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


RHEUMATIC FEVER

 Licks the Joint and Bites the Heart


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