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

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

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

MAJOR MANIFESTATIONS 3

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

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

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

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

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

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

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

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

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

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

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 14

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

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

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 17

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

DIFFERENTIAL DIAGNOSIS 19

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 20

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

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

?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

JACCOUDS ARTHRITIS 24

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 25

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

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

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

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

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

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

EVOLUTION OF JONES CRITERIA 32

WHO 2002 2003 33

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

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

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

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. 39

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

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

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

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

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

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 45

WHO ECHO CRITERIA FOR CLINICAL CARDITIS 0: Nil, including physiological or trivial regurgitant jet

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

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