This document summarizes acute rheumatic fever, which is an autoimmune disease triggered by a streptococcal infection. It most commonly affects children ages 5-15. It can cause inflammation of the heart (carditis), joints (arthritis), skin (erythema marginatum), and brain (chorea). The pathogenesis involves molecular mimicry between antigens in streptococcus and human tissues. Treatment involves bed rest, antibiotics to treat strep, and aspirin or steroids for suppression. Prevention relies on prompt strep treatment and long-term antibiotic prophylaxis to prevent recurrence, especially for those with heart valve damage.

1. DR DEEPASHREE DAS
ASSAM MEDICAL COLLEGE,
PEDIATRICS,PGT
ACUTE RHEUMATIC
FEVER

2. INTRODUCTION
 Rheumatic fever is an immunological disorder
initiated by group A beta hemolytic
streptococci.
 Antibodies produced against selected
streptococcal cell wall proteins and sugars
react with the connective tissues of the body
as well as the heart and results into rheumatic
fever.

3. EPIDEMIOLOGY
 Most common form of acquired heart disease in
all age groups.
 Constitutes from 5%-50% of the cardiac patients
in indian hospital.
AGE AND SEX:
 Commonly affects between 5 and 15 years of
age; 1st episode rare before 3 yrs and after 30
years.
 Incidence of rheumatic fever following
streptococcal throat infection is 0.3%.
 1-3% in presence of epidemics of streptococcal
pharyngitis.

4.  Sexes nearly equally affected
 Mitral valve disease and chorea more common in
girls whereas aortic valve involvement often seen
in boys.
 PREDISPOSING FACTORS: poor
socioeconomic conditions, unhygenic living
conditions and overcrowding

5. ETIOLOGY
 Evidence supports link between antecedent beta
hemolytic group A streptococci and acute rheumatic
fever and rheumatic heart disease. It is indicated by
no. Of observations:
1. History of preceding sore throat is available in 50%
cases; >85% shows elevated ASO titre.
2. Epidemics of streptococcal infection are followed
by higher incidence of rheumatic fever.
3. Seasonal variation of rheumatic fever and
streptococcal infection is identical.
4. In patients with established RHD, streptococcal
infection is followed by recurrence of acute
rheumatic fever.
5. Penicillin prophylaxis for streptococcal infection

6.  Certain GAS serotypes – M TYPE 1,3,5,6,18,29
are more frequently isolated from patients with
acute RF.

7. PATHOGENESIS
CYTOTOXICITY THEORY:
GAS toxin is involved in the pathogenesis of ARF
and RHD. Number of enzymes that are cytotoxic
for mammalian cardiac cells, such as
STREPTOLYSIN O, has a direct cytotoxic effect
on mammalian cells in tissue culture. Drawback-
inability to explain latent period between GAS
pharyngitis and onset of acute RF.( usually 10-21
days).
IMMUNE MEDIATED PATHOGENESIS:
the antigenicity of several GAS cellular and
extracellular epitopes and their imunologic cross
reactivity with cardiac antigenic epitopes
supports to the hypothesis of molecular mimicry.

8. Common epitopes are shared between certain GAS
components- M protein, cell membrane , group A cell wall
carbohydrate, capsular hyaluronate and specific
mammalian tissues – heartvalve, sarcolemma, brain,
joint.
certain rheumatogenic M proteins ( M1, M5,M6 and M 19)
share epitopes with human myocardial proteins such as
tropomyosin and myosin.
Another proposed pathogenetic hypothesis is binding of
M protein N terminal domain to a region of collagen type
4 leads to an antibody response to the collagen, resulting
in ground substance inflammation especially cardiac
valves and myocardium.

10. Clinical features:
 Streptococcal pharyngitis with fever followed 10
days to a few weeks later by recurrence of fever
and the manifestations of acute rheumatic fever.
 Guidelines for the clinical diagnosis of acute
rheumatic fever , suggested by T. Duckett jones
revised by american heart assosciation.
 Guidelines consists of major, minor, and
essential criteria
 Two major or one major and two minor criteria
required to diagnose the first episode of ARF.
 For recurrences, three minor criteria required.

11.  In the 2015, jones criteria revision, a major
change from previous version expands the
definition of the major criterion carditis to include
subclinical evidence (eg, in the absence of
murmur , echocardiographic evidence of mitral
regurgitation meeting specific criteria to
distinguish physiologic from pathologic MR)

14. Major criteria
1. Carditis:
-it is an early manifestation, echocardiography
indicates carditis in 90% patients
-Rheumatic carditis is designated as a
pancarditis involving the pericardium,
myocardium and endocardium.
-almost 80% of patients develop carditis within
the first two weeks of onset.
-pericarditis results in precordial pain. On
auscultation ,friction rub is present. Only small
effusions and doesnot result in tamonade or
constrictive pericarditis.

15. - Rheumatic carditis always has additional mitral
or mitral and aortic regurgitation murmurs.
- other features of carditis are cardiac
enlargement, soft first heart sound,
protodiastolic gallop, congestive heart failure
and carey coombs murmur.
- Endocarditis is represented by a pansystolic
murmur of Mitral regurgitation with or without an
associated aortic regurgitation murmur.
- 95% have mitral regurgitation murmur, a quarter
of them have also have aortic regurgitation
murmur and 5% pure AR.
- The acute hemodynamic overload resulting from
acute mitral regurgitation and or AR leads to left
ventricular failure and is the main reason for
the morbidity and mortality of rheumatic

16. SUBCLINICAL CARDITIS: carditis may
occasionally be clinically silent and only
identified by echocardiography that shows
mitral regurgitation.
2. ARTHRITIS:
- it is an early manifestation, involves 30-50%
patients.
- rheumatic arthritis is a polyarthritis involving
large joints knees, ankles, elbows.
Uncommonly smaller joints may also be
involved.
- it is a migratory arthritis with the affected joints
showing redness, warmth, swelling ,pain and
limitation of movement. There is no residual

17. 3. Subcutaneous nodules:
- Occurs in about 3-20% of cases It is a late
manifestation and and appears around 6 weeks after
the onset of rheumatic fever. They last from few days
to weeks but can last upto 1 year.
- they are non tender and appear on bony
prominences like elbows, shins, occiput and spine.
- they vary in size from pinhead to an almond.
4. CHOREA:
- Syndenham’s chorea is also a late manifestation
occurring about three months after the onset of ARF.
- it consists of semipurposeful, jerky movements
resulting in deranged speech, muscular coordination,
awkward gait and speech. Child is emotionally
disturbed.
- self limiting course of two to six weeks. 3-4 times
common in females.

19. Syndenhams chorea

20. 5. ERYTHEMA MARGINATUM:
- it is an early manifestation, predominantly seen
over the trunk.
- it starts as a red spot with pale centre,
increasing in size to coalasce with adjacent spots
to form a serpiginious outline.
- rash is nonitching.

23. MINOR CRITERIA
CLINICAL CRITERIA:
1. FEVER: rheumatic fever almost always
associated with fever. Temperature rarely goes
above 39.5 C.
2. ARTHRALGIA: arthralgia and arthritis together
occur in about 90% of the patients. It is a
subjective pain.
3. PREVIOUS RHEUMATIC FEVER OR RHD:
this criterion applies only for recurrent episodes
of rheumatic fever.
 LABORATORY MANIFESTATION:
1. ACUTE PHASE REACTANTS: the leukocyte
count usually lies between 10000 to

24. ESR is elevated during ARF and remains for 4-10
weeks in almost 80% patients.
C reactive protein is elevated in all patients and
subsides rapidly if the patient is treated with
corticosteroids. Its presence is nonspecific, while
absence is against the diagnosis of ARF.
2. PROLONGED PR INTERVAL: it is prolonged in
many infections and is not diagnostic of carditis.
Higher grades of block like second degree AV
block especially Wenckebachs type may be
seen.

25. ESSENTIAL CRITERIA:
1. ASO TITRE: these include evidence of recent
streptococcal infection. Elevated levels of ASO
indicate previous infection and not rheumatic
fever. A basal ASO titre of 50 u/dl that goes
upto 250 u/dl is indicative of recent infection.
Rising titre is a strong evidence.
2. ECHOCARDIOGRAPHY: the recent revision of
jones criteria includes echocardiographic
findings for the diagnosis of rheumatic carditis.
Features include annular dilation, elongation of
chordae to the anterior leaflet of the mitral valve
causing a prolapse and lack of coaptation of
the two leaflets resulting in mitral regurgitation.
This has lead to recognition of subclinical
carditis.

26. TREATMENT
Management is symptomatic combined with
suppressive therapy.
Bed rest: Bed rest is generally recommended for
ARF. Prolonged bed rest >2-3 weeks is seldom
necessary unless there is clinically apparent
carditis with heart failure.
Penicillin: after obtaining throat culture.
A single injection of benzathine penicillin is
given.
Penicillin V is an alternative. 250MG four
times a day for 10 days.
Erythromycin 250 MG four times a day for 10
days is given to penicillin allergy.

27. SUPPRESSIVE THERAPY:
Aspirin or corticosteroids are given as
suppressive therapy. Steroids are more potent
suppressive agent as compared to aspirin.
Following guidelines are used :
 CARDITIS WITH CONGESTIVE CARDIAC
FAILURE: corticosteroids.
 CARDITIS WITHOUT CONGESTIVE
CARDIAC FAILURE: either corticosteroids or
aspirin ; former preferred.
 NO EVIDENCE OF CARDITIS: aspirin.

28.  Total duration of therapy – 12 weeks.
 Aspirin is given at a dose of 90-120 mg/kg/day
in four divided doses for 10 weeks and tapered
in next two weeks.
 Prednisolone at a dose of 2mg/kg daily ;
maximum dose 60mg is given for three weeks
and then tapered gradually in next 9 weeks.
 Surgical replacement of the mitral and/or aortic
valve is sometimes indicated ,if the patient is
deteriorating despite aggressive decongestive
measures.

29.  Management of chorea:
the patient as well as the parents are reassured
about the self limiting course. The signs and
symptoms of chorea do not respond well to anti
inflammatory agents or steroids. Supportive
measures such as rest in quiet room,
medications such as haloperidol, diazepam, and
carbamazepine are effective.

30. Prevention of rheumatic fever:
PRIMARY PREVENTION:
It includes:
I. Prompt identification of sore throat
II. Rapid confirmation of a streptococcal
etiology
III. Treatment with penicillin.
 SECONDARY PREVENTION:
It consists in giving long acting benzathine
penicillin. The dose is 1.2 million units once
every 3 weeks or 0.6 million units every
alternate week.

31.  Patient without proven carditis should receive
prophylaxis for 5 years after the last episode or
until they are 18 year old (whichever is longer)
 Patient with carditis – mild mitral regurgitation or
healed carditis should receive prophylaxis for 10
years after the last episode or till 25 years of age
(whichever is longer)
 Patients with established RHD or following valve
surgery or balloon valvotomy should receive
lifelong prophylaxis.
 Some cardiologists recommend discontinuation
of prophylaxis after the age of 40 years, since
the recurrence beyond this age is minimal.

32. Thank you....