2. ACUTE RHEUMATIC FEVER
INCIDENCE
-Children (most common b/w 5&15 years)
-Young adults
-Geographical distribution : very RARE in Western Europe &
Northern America
-Remains ENDEMIC in parts of Asia, Africa & South America
-Annual Incidence : >100 per 100,000
-Most common cause of ACQUIRED heart disease in childhood &
adolescence
3. ACUTE RHEUMATIC FEVER
PATHOGENESIS
immune-mediated delayed response to infection with specific
strain of group A Streptococci (have Ags that may cross-react
with CARDIAC MYOSIN & SARCOLEMMAL MEMBRANE PROTEIN)
Abs produced against the streptococcal Ags
Inflammation in the endocardium, myocardium, pericardium,
joints & skin
5. ACUTE RHEUMATIC FEVER
HISTOLOGY
-FIBRINOID degeneration in the COLLAGEN of connective tissue
-ASCHOFF nodules are pathognomonic, occur only in the HEART
(multinucleated giant cells surrounded by macrophages & T
lymphocytes, seen in sub acute & chronic phases of rheumatic
carditis)
6.
7.
8. ACUTE RHEUMATIC FEVER
CLINICAL FEATURES
-fever, anorexia, lethargy, joint pain, 2-3 weeks after an episode
of streptococcal pharyngitis
-Diagnosis is made using the Revised Jones criteria
9. ACUTE RHEUMATIC FEVER
JONES CRITERIA for diagnosis of Rheumatic Fever
MAJOR manifestations
1.Carditis
2.Polyarthritis
3.Chorea
4.Erythema marginatum
5.Subcutaneous nodules
10. ACUTE RHEUMATIC FEVER
JONES CRITERIA for diagnosis of Rheumatic Fever
MINOR manifestations
1.Fever
2.Arthralgia
3.Previous RF
4.Raised ESR or CRP
5.Leukocytosis
6.First-degree AV block
PLUS
Supporting evidence of preceding streptococcal infection;
1.Recent scarlet fever
2.Raised ASO or other strep Ab titre
3.Positive throat culture
11. ACUTE RHEUMATIC FEVER
DIAGNOSTIC CRITERIA
1.2 or more major manifestations
2.1 major or 2 or more minor manifestations
1&2 along with evidence of preceding streptococcal infection
PRESUMPTIVE DIAGNOSIS
-Isolated chorea or pancarditis, if other causes for these hv been
excluded
ESTABLISHED RHD or PRIOR RF
-Diagnosis of acute RF can be made based only on the presence of
MULTIPLE MINOR criteria & evidence of PRECEDING group A
streptococcal pharyngitis.
16. ACUTE RHEUMATIC FEVER
ARTHRITIS
-Most common major manifestation
-Occurs early when streptococcal Ab titres are high
-Acute PAINFUL
-ASYMMETRICAL
-MIGRATORY
-Inflammation of LARGE joints (knees, ankles, elbows, wrists)
-QUICK succession
-RED, SWOLLEN, TENDER
-Between 1 day to 4 weeks
-RESPONDS to ASPIRIN
17.
18. ACUTE RHEUMATIC FEVER
SKIN LESIONS
1.ERYTHEMA MARGINATUM
-<5% pts
-Red macules (blotches)
-Fades in centre, RED at the EDGES
-TRUNK, PROXIMAL extremities
-NOT the face
-Red rings or margins may coalesce or overlap
19.
20. ACUTE RHEUMATIC FEVER
SKIN LESIONS
2. SUBCUTANEOUS NODULES
-5-7% pts
-Small (0.5-2.0 cm), firm, PAINLESS
-EXTENSOR surfaces of bone or tendons
-Appear >3 weeks AFTER onset of other manifestations
21.
22.
23. ACUTE RHEUMATIC FEVER
SYDENHAM’S CHOREA ( ST. VITUS DANCE )
-Appears at least 3 months AFTER episode of acute RF
-1/3 of cases
-> females
-1st
feature; EMOTIONAL LIABILITY
-Purposeless involuntary choreiform movements of HANDS<
FEET or FACE
-EXPLOSIVE or HALTING speech
-Spontaneous recovery within few months
-Approx. ¼ develop chronic rheumatic valve disease
25. ACUTE RHEUMATIC FEVER
INVESTIGATIONS
1.Evidence of a systemic illness ( non-specific )
-Leukocytosis
-Raised ESR & CRP (monitor disease progression)
2. Evidence of preceding streptococcal infection ( specific )
-Throat swab culture ; GABHS ( family members, contacts )
•+ve in only 10-25% cases
-ASO titres ; RISING titres OR >200 U (adults), >300 U (children)
•Normal in 1/5 of adult cases of RF & most cases of chorea
26. ACUTE RHEUMATIC FEVER
3. Evidence of carditis
-CXR ; cardiomegaly, pulmonary congestion
-ECG ; 1st
& rarely 2nd
degree heart block, features of pericarditis,
T-wave insertion, reduction in QRS voltages
-ECHO ; cardiac dilatation, valve abnormalities
•ECHO typically shows
i)MITRAL regurgitation with dilatation of mitral annulus &
prolapse of ant. mitral leaflet
ii)Aortic regurgitation
iii)Pericardial effusion
27. ACUTE RHEUMATIC FEVER
MANAGEMENT OF ACUTE ATTACK
1.SINGLE DOSE of BENZYL penicillin 1.2 million U IM or
2.PHENOXYMETHYLpenicillin 250mg 6-hourly for 10 days
Should be given ON DIAGNOSIS, to eliminate any residual
streptococcal infection
•Erythromycin or cephalosporin in penicillin-allergic pts
28. ACUTE RHEUMATIC FEVER
BED REST & SUPPORTIVE THERAPY
Bed rest – lessens joint pain, reduces cardiac workload
Valve replacement – heart failure not responding to medical Rx
29. ACUTE RHEUMATIC FEVER
ASPIRIN
Rapid relief of arthritis within 24 hours, confirm the Dx
Starting dose – 60mg/kg/day divided into 6 doses
Adults – 100mg/kg/day may be needed up to limits of tolerance
OR max of 8grams/day
Continued until ESR has fallen & tapered gradually
Toxic effects; nausea, tinnitus, deafness, vomiting, tachypnoea,
acidosis
30. ACUTE RHEUMATIC FEVER
CORTICOSTEROIDS
More rapid relief
Indications; cases with carditis, severe arthritis
Prednisolone 1.0-2.0 mg/kg/day in divided doses, until ESR is
normal & tapered.
31. ACUTE RHEUMATIC FEVER
SECONDARY PREVENTION
1.Benzathine penicillin 1.2 MU IM monthly or
2.Oral phenoxymethylpenicillin 250mg 12-hourly
•Sulfadiazine or erythromycin (pts allergic to penicillin)
•Sulfonamides prevent infection, but not eradication of GAS
-Further attacks of RF, unusual after 21 yrs, Rx may be stopped
-Extension of Rx
•i) if attack occurred in last 5 yrs
•ii) Pts live in area of high prevalence, occupational exposure
•iii) Residual heart disease,
•Prophylaxis until 10 yrs after last episode OR until 40 yrs of age,
whichever longer