DIAGNOSIS & MANAGEMENT OF RHEUMATIC FEVER DR.SANDEEP R SR
CARDIO 87 slides
Slide 2
Introduction Rheumatic fever( RF) - a delayed autoimmune
reaction in genetically predisposed individuals to group A,
-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 2
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MAJOR MANIFESTATIONS 3
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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 4
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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 5
Slide 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. 6
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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 7
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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 8
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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 9
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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 10
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POLYARTHRITIS Synovial fluid in ARF usually has 10,000-100,000
WBC/mm 3 Exudative with normal glucose & neutrophil
predominance Self limiting & normalizes by 2 4 wks
Polyarthritis & sydenhams chorea never occurs simultaneously
Inverse relationship b/w the severity of arthritis & cardiac
involvement 11
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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 12
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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 13
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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
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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 15
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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 16
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CHOREA Long latent period Clinical maneuvers to elicit features
of chorea include (1) demonstration of milkmaids grip (irregular
contractions of the muscles of the hands while squeezing the
examiners fingers) (2) spooning & pronation of the hands when
the patients 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
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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 18
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DIFFERENTIAL DIAGNOSIS 19
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MINOR MANIFESTATIONS Arthralgia 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 >100.40 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
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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 21
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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) 22
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?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 23
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JACCOUDS ARTHRITIS 24
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JACCOUDS 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 4 th and 5 th fingers & hyperextn of PIP Initially
the deformity is correctable & not assoc with bone destruction
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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). 26
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EVOLUTION OF JONES CRITERIA ORGINAL JONES CRITERIA 1944 MAJOR
MANIFESTATIONS MINOR MANIFESTATION 1.CARDITIS 2.ARTHRALGIA 3.CHOREA
4.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 27
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MODIFIED JONES CRITERIA 1956 MAJOR MANIFESTATIONMINOR
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 28
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REVISED JONES CRITERIA 1965 MAJOR MANIFESTATIONMINOR
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 PRECEEDING STREPTOCOCCAL
INFECTION,H/O RECENT SCARLET FEVER;POSITIVE THROAT C/S FOR GROUP A
STREPTOCOCCUS; INCREASED ASO TITRE 29
Slide 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 30
Slide 31
JONES CRITERIA UPDATE 1992 MAJOR MANIFESTATIONMINOR
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 + SUPPORTING EVIDENCE OF PRECEEDING STREPTOCOCCAL INFECTION
POSITIVE THROAT C/S FOR GROUP A STREPTOCOCCUS OR RAPID ANTIGEN TEST
ELEVATED OR RISING STREPTOCOCCAL ANTIBODY TITRE 31
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EVOLUTION OF JONES CRITERIA 32
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WHO 2002 2003 33
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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 36
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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) 37
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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 38
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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.
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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) -80-85% Multiple antibody test-(ASO +ANTIDNase B)-
95-100% 40
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LAB INVESTIGATION 1) B CELL MARKER D8/17 monoclonal antibody -
90 to 100% of all patients with RF Mode of inheritence - Autosomal
recessive 2) CRP, ESR 41
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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 20- 30% 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 42
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ECHOCARDIOGRAPHY CHARACTERISTIC CHANGES IN RHEUMATIC MITRAL
VALVULITIS CHORDAL ELONGATION ANNULAR DILATION AML PROLAPSE
POSTEROLATERAL JET OF MR 43
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ECHOCARDIOGRAPHY SUBCLINICALCARDITIS/ ECHOCARDITIS Patients
with suspected acute rheumatic carditis have no clinical murmurs
but have documented regurgitation on echocardiography Prevalence 0
to 53% 44
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ECHOCARDIOGRAPHY ADVANTAGES 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
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WHO ECHO CRITERIA FOR CLINICAL CARDITIS 0: Nil, including
physiological or trivial regurgitant jet