Rheumatic fever- a multifactorial disease that follows GAS pharyngitis in a susceptible individual who lives under deprived social conditions, characterized by acute inflammation of the heart, joints, skin, subcutaneous tissue & CNS, that gives rise to typical clinical feature including Arthritis, Carditis, Chorea, Subcutaneous nodules & Erythema marginatum. Latent period of 2-3 weeks following GAS pharangitis. Destructive effects on heart valves leads to RHD with serious hemodynamic disturbances causing HF, stroke & infective endocarditis.

1. RHEUMATIC FEVER
DR SHADAB

2. INTRODUCTION
Rheumatic fever- a multifactorial disease that follows GAS pharyngitis in a
susceptible individual who lives under deprived social conditions,
characterized by acute inflammation of the heart, joints, skin, subcutaneous
tissue & CNS, that gives rise to typical clinical feature including
• Arthritis
• Carditis
• Chorea
• Subcutaneous nodules &
• Erythema marginatum
• Latent period of 2-3 weeks following GAS pharangitis
• Destructive effects on heart valves leads to RHD with serious hemodynamic
disturbances causing HF, stroke & infective endocarditis

3. EPIDEMIOLOGY
• Ages 4-15 years are most
susceptible
• In developing countries (such as
Saudi Arabia & India), juvenile
MS may occur at age 3-5 years
• Rare in <3 years

4. PATHOGENESIS
• GAS pharyngitis triggers an
autoimmune response to epitopes
in the organism that cross react
with similar epitopes in the heart,
brain, joints and skin , and
repeated episodes of rheumatic
fever lead to RHD (theory of
molecular mimicry)

5. GAS PHARYNGITIS

6. ARTHRITIS
• Joint involvement is more common (almost 100%), more
severe in young adults than in teenagers (82%), &
children(66%)
• Migratory arthritis- sequential involvement of joints with
inflammation resolving in one joint & then begins in another
joint (sometimes may be additive)
• Usually Subside within days to 1 week, may last for another 1-
2 weeks, if persist after 4 weeks, it becomes necessary to
consider other conditions such as juvenile idiopathic arthritis
or SLE
• Large joints- knee, ankle, elbow & wrists are most frequently
involved
• Hip , shoulder, and small joints of hands & feet are less
frequently involved
• Respond promptly to NSAIDS

7. JACCOUD ARTHRITIS
• Chronic post-rheumatic fever arthropathy
• Ulnar deviation of fingers (esp. 4th & 5th
finfers), flexion of metacarpophalangeal
joints, & hyperextension of proximal
interphalangeal joints (i.e., swan neck
deformity)
• Usually painless & no signs of inflammation
• Results from recurrent inflammation of
fibrous articular capsule & there are no true
erosion on xray
• Rheumatoid factors are usually negative

8. CARDITIS
• Incidence of carditis varies with age with
first attack of rheumatic fever:
• 90-92% of children age<3 years
• 50% of children age 3-6 years
• 32% of teenagers age 14-17 years
• 15% of adults
• Symptoms & signs depend on involvement
of endocardium (heart valves), myocardium
& pericardium
• Most serious manifestation of rheumatic
fever

9. 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
• Inflammation, edema & fibrin deposits on valve leaflets along
the line of closure(verrucous vegetations)
• Map-like areas of thickened, roughened, and wrinkled part of
the endocardium in the left atrium caused by regurgitant jets
of MR (MacCallum plaque)
• The most common valvular lesion is MR causing an apical
PSM, pathogenesis include valvulitis, mitral annular
dilatation, leaflet prolapse or chordal rupture, AR is less
common.
• Stenotic lesions are uncommon in the early stages , but a
transient apical mid-diastolic murmur (Carey-Coombs) may
occur in association with MR
• With H/O previous RHD, a change in character of murmurs or
appearance of a new murmur will indicate the presence of
acure rheumatic carditis.

10. MYOCARDITIS
• Almost always associated with valvulitis
• Should be accompanied by an apical systolic or basal
diastolic murmur.
• May develop cardiomegaly & CHF
• ECG changes include varying degree of heart block, with
1st degree heart block are usually asymptomatic
• Patients with 2nd & 3rd degree heart block are
symptomatic and require a pacemaker if they develop
CHF
• Aschoff body consists of a focus of fibrinoid necrosis
(site of Ag-Ab reaction) surrounded by activated
histiocytes (Anitschkow’s cells/caterpillar cells) &
lymphocytes

11. PERICARDITIS
• Detected clinically in 10% of
patients
• Associated with anterior chest pain
& pericardial friction rub
• Pericardial effusion may be large,
but cardiac tamponade is rare
• Constrictive pericarditis does not
occur
• “Bread & butter” appearance due
to fibrinous inflammation

12. MINIMUM ECHO CRITERIA FOR DIAGNOSIS OF VALVULAR
REGURGITATION CAUSED BY RHEUMATIC CARDITIS
• All four Doppler criteria must be
met
• A regurgitant jet length should be
measured from the vena
contracta to the last pixel of
regurgitant color on
nonmagnified images.
PATHOLOGIC MR PATHOLOGIC AR
1. Seen in at least two
views
1. Seen in at least two
views
2. In at least one view, jet
length is ≥2 cm
2. In at least one view, jet
length is ≥1 cm
3. Peak velocity ≥3 m/sec 3. Peak velocity ≥3 m/sec
4. Pansystolic jet in at
least one envelope
4. Pansystolic jet in at
least one envelope.

13. SYDENHAM CHOREA
• May be the only presenting manifestation of rheumatic fever
• Female predominance
• Latent period 6-8 week (longer than arthritis & carditis, 2-3 weeks)
• Usually last for 8-15 weeks (may last for 1 week to 2 years)
• Characterized by the presence of involuntary, purposeless, jerky movements
of hands, arms, shoulders, feet, legs, face & trunk associated with hypotonia
& weakness, sometimes may be unilateral (hemichorea)
• The purposeless movements interfere with voluntary activity & disappear
during sleep
• Jack-in-the-box tongue
• Milking sign
• Chorea does not occur simultaneously with arthritis but may coexist with
carditis

14. SYDENHAM’S CHOREA VS PANDAS
Sydenham’s chorea PANDAS
Age of onset 5-15 years 3 years – puberty
Gender Female predominance Male > Female
Pathogen Several M-strains of Streptococcal bacteria No unique strain identified
Incidence of psychiatric
disorders in 1st degree relatives
No studies 39% motor or vocal tics, 26% OCD
Neurological abnormalities Hypotonia, Darting tongue, Milkmaid sign,
Pronator sign, choreic hand, dysarthria
Motor hyperactivity, adventitious movements
such as choreiform movements
Psychiatric manifestation Emotional liability, anxiety, tics, OCD in
70% of patients
Emotional liability, separation anxiety, night
time fears, oppositional behaviors
ASO titer Elevated in 80% of cases Temporal relation with ASO
Duration of symptoms Usually resolve in 1-6 months, may persist
up to 2 years
Remitting-relapsing course
Anti-basal ganglia antibodies 40, 45, 60 kDa autoantigens molecule 60kDa ABGA very frequently present
MRI Volumetric increase in caudate, putamen &
globus pallidus
Volumetric increase in caudate, putamen, &
globus pallidus
Treatment Benzathine penicillin prophylaxis 1.2 MU
I/M 3 weekly
IVIG & plasma exchange, tonsilectomy

15. SUBCUTANEOUS NODULES
• Resemble the nodules of rheumatoid
arthritis
• May be detected over occiput,
elbows(over olecranon), knees, ankles,
and Achilles tandons.
• Usually firm, painless, & freely movable
over the subcutaneous tissue
• Size 0.5-2 cm & tend to occur in crops
• Usually smaller, more discrete, &
persistent than rheumatoid nodules
• Persist for few weeks but seldom >1
month
• Multiple crops may be related to the
severity of rheumatic carditis

16. ERYTHEMA MARGINATUM
• Occur on upper arms or trunk, but
not the face
• The rash is evanescent, pink, and
nonpruritic
• Extend centrifugally while the skin
at the center returns to normal
• Has an irregular serpiginous border
• Rash become more prominent after
a hot shower

17. DIFFERENTIAL DIAGNOSIS
ARTHRITIS CARDITIS CHOREA
Rheumatoid arthritis Viral myocarditis Huntington chorea
Reactive arthritis (eg., Shigella,
Salmonella, Yersinia)
Viral pericarditis Wilson disease
Serum sickness Infective endocarditis SLE
Sickle cell disease Kawasaki disease Cerebral palsy
Malignancy Congenital heart disease Tics
SLE Mitral valve prolapse Hyperactivity
Lyme disease Innocent murmur
Gonococcal infection

18. MINOR MANIFESTATIONS
Fever
• Usually raised during attacks
• Ranges from 38.4˚C to 40˚C
• Usually decreases within 1 week & rarely last >4 weeks
Abdominal pain
• May mimic acute appendicitis
Epistaxis
Rapid sleeping pulse rate, tachycardia out of proportion to fever
Malaise
Anemia
Rheumatic pneumonia

19. EVOLUTION OF JONES CRITERIA

20. 2015 AHA-REVISED JONES CRITERIA FOR DIAGNOSIS OF RHEUMATIC FEVER
MAJOR CRITERIA
LOW-RISK
POPULATIONS
MODERATE & HIGH-
RISK POPULATIONS
1. Carditis
(clinical/subclinical)
Carditis
(clinical/subclinical)
2. Arthritis (polyarthritis
only)
Arthritis (including
polyarthritis, monoarthritis/
polyarthralgia)
3. Chorea Chorea
4. Erythema marginatum Erythema marginatum
5. Subcutaneous nodules Subcutaneous nodules
MINOR CRITERIA
LOW RISK
POPULATIONS
MODERATE & HIGH
RISK POPULATIONS
1. Polyarthralgia Monoarthralgia
2. Fever (≥38.5˚C) Fever (≥38˚C)
3. ESR ≥60 mm in the 1st
hour &/or CRP ≥3.0 mg/dl
ESR ≥30 mm in the 1st hour
&/or CRP ≥3.0 mg/dl
4. Prolonged PR interval,
after accounting for age
variability (unless carditis is
a major criterion)
Prolonged PR interval, after
accounting for age
variability (unless carditis is
a major criterion)
• Joint manifestations are considered in either the major or the minor category, but not in both categories in the same
patient
• Low-risk communities are defined as having an ARF incidence of ≤2 per 100,000 school-aged children (usually 5 to
14 years old) per year, or an all-age prevalence of RHD of ≤1 per 1000 population per year

21. DIAGNOSIS
• The initial diagnosis of ARF is made if, in the presence of preceding
GAS infection, two major criteria or one major and two minor criteria
are present.
• The diagnosis of recurrent ARF requires two major, one major and
two minor, or three minor criteria in the presence of preceding GAS
infection.
• Evidence of preceding GAS infection, (essential for diagnosis), may
be obtained from
• Throat swab culture (only positive in approx. 11% of patients), or
• By demonstrating a rising titer of antistreptococcal antibodies, either ASO or
anti-Dnase B, or
• By a positive rapid group A streptococcal carbohydrate antigen test in a child
whose clinical presentation suggests a high pretest probability of streptococcal
pharyngitis.

22. POSSIBLE RHEUMATIC FEVER
• The 2015 Jones criteria also recognize the clinical entity of “possible
rheumatic fever”
• Applied in part of the world where rheumatic fever remains common
and where it is not possible to fulfill the Jones criteria because of a
lack of lab facilities to conduct the recommended investigations of a
patient with suspected rheumatic fever.
• When a diagnosis of possible rheumatic fever is made, it is reasonable
to consider offering 12 months of secondary prophylaxis, followed by
reevaluation based on history, physical examination, and repeat
echocardiogram.

23. INVESTIGATIONS IN SUSPECTED RHEUMATIC FEVER
Recommended for All Cases Test for Alternative Diagnoses, Depending on
Clinical Features
WBC count Repeated blood culture with temperature spikes if
infective endocarditis is suspected
ESR or CRP Joint aspirate for possible septic arthritis (microscopy
& culture)
Throat swab before giving antibiotics for GAS culture Copper, ceruloplasmin, ANA, and drug screen for
choreiform movements
Blood culture, if febrile Serology and autoimmune markers for arboviral,
autoimmune, or reactive arthritis
Antistreptococcal serology: both ASO & antiDNase B
titers (repeat after 10-14 days if first test is not
confirmatory)
Peripheral blood smear for sickle cell disease
ECG
Chest X-ray
Echocardiogram

24. TREATMENT
• Bed rest- to lessen joint pain
• Ambulation can usually be started once the fever has subsided and acute phase
reactants are returning to normal
• Sternous exertion should be avoided, esp. for those with carditis
• Antibiotics
• Benzathine benzylpenicillin (Penicillin G)- 1.2 MU I/M single dose (50% if weight
<30 kg)
• Phenoxymethylpenicillin (Penicillin VK)- 250-500 mg oral three times daily for 10
days
• Erythromycin ethylsuccinate- oral for 10 days
• Anti-inflammatory agents
• Aspirin 100mg/kg/day in four to five divided doses for approx. 1 month or until
inflammation has subsided (for milder attack with little or no carditis)
• Prednisone 1-2 mg/kg/day for 2-3 months then graduallt weaned (for more severe
cases)

25. SECONDARY PREVENTION
ANTIBIOTICS DOSE
Benzathine benzylpenicillin 1.2 MU I/M every 3-4 weeks (for adults & children ≥ 30 kg
weight)
0.6 MU I/M every 3-4 weeks (for children < 30 kg weight)
Penicillin V 250 mg oral twice daily
Sulfonamides (sulfadiazine, sulfadoxine, &
sulfisoxazole)
1 g/day (for adults & children ≥ 30 kg weight)
0.5 g/day (for children < 30 kg weight)
Erythromycin 250 mg twice daily
DURATION OF SECONDARY PROPHYLAXIS
CATEGORY OF PATIENTS DURATION
Patients without proven carditis For 5 years after last attack or until 18 years of age
(whichever is longer)
Patient with carditis (mild MR or healed carditis) For 10 years after last attack or at least until 25 years of age
(whichever is longer)
More severe valvular disease Lifelong
After valve surgery Lifelong

26. THANK YOU