RHEUMATIC FEVER

By:
Rey Martino

FOR EDUCATIONAL USE ONLY

Rheumatic fever
• Immunologically mediated inflammatory
disorder, which occurs as a sequel to group
A streptococcal pharyngeal infection.

• Multisystem disease affecting the heart,

joints, brain, cutaneous and subcutaneous
tissues
 Epidemiology

• Ages 5-15 years are most susceptible

• Rare < 3 years
• Girls > boys
• Common in 3rd world countries
• Environmental factors: over crowding, poor
sanitation, poverty
 Pathogenesis
Body produce
antibodies
GABS against
infection streptococci

Immunologically
Immunologically mediated
mediated
inflammation
inflammation & & damage
damage
(autoimmune)
(autoimmune) to to human
human
tissues
tissues which
which have
have cross react
cross react with
with human
human
antigenic
antigenic similarity
similarity with
with tissues because
tissues because ofof the
the
streptococcal
streptococcal components
components antigenic similarity
antigenic similarity
like between streptococcal
between streptococcal
like heart,
heart, joint,
joint, brain
brain
components and
components and human
human
connective
connective tissues
tissues connective tissues
connective tissues
(molecular mimicry)
(molecular mimicry)
Pathogenesis
Pathogenesis
Clinical features
1. Arthritis
-Polyarthritis, fleeting, migratory, large joints, no
residual deformity, rapid response to aspirin(if
aspirin given,24to48hrs joint pain will disappear;
thus used as diagnostic test)

2. Sydenham Chorea
-Spasmodic, unintentional, jerky choreiform
movements, speech affected, fidgety, late
manifestation (thus no ESR or ASO titre elevation)
Clinical signs: pronator sign, jack in the box sign ,
milking sign of hands
Clinical features
3. Carditis
• Manifest as pancarditis ( endocarditis, myocarditis
and pericarditis ), occur in 40-50% of cases
• Carditis is the only manifestation of rheumatic
fever that leaves a sequelae & permanent damage
to the organ
• Valvulitis occur in acute phase
• Chronic phase: fibrosis, calcification & stenosis of
heart valves (fishmouth valves)
• Rheumatic
heart
disease.
Abnormal
mitral valve.
Thick, fused
chordae
Clinical features
4. Erythema Marginatum
• Occur in <5%.
• Unique, transient, serpiginous-looking lesions of
1-2 inches in size
• Pale center with red irregular margin
• More on trunks & limbs & non-itchy
• Worsens with application of heat
• Often associated with chronic carditis
Clinical features
5. Subcutaneous nodules
• Occur in 10%
• Painless,pea-sized,palpable nodules
• Mainly over extensor surfaces of
joints,spine,scapulae & scalp
• Associated with strong seropositivity
• Always associated with severe carditis
Clinical features
Other features (Minor features)
• Fever
• Arthralgia
• Pallor
• Anorexia
• Loss of weight
Diagnosis
2002–2003 WHO criteria for diagnosis of RF and RHD (based on
the revised Jones criteria)

The revised WHO criteria facilitate the diagnosis of:

1) a primary episode of RF
2) recurrent attacks of RF in patients without
RHD
3) recurrent attacks of RF in patients with RHD
4) rheumatic chorea/insidious onset rheumatic
carditis
5) chronic RHD.
2002–2003 WHO criteria for diagnosis of RF and RHD (based
on the revised Jones criteria)
Diagnostic categories Criteria
Primary episode of RF Two major *or one major and two minor**
manifestations plus evidence of a
preceding group A streptococcal
infection***.

Recurrent attack of RF in a patient without Two major or one major and two minor
established RHD. a manifestations plus evidence of a
preceding group A streptococcal infection. b

Recurrent attack of RF in a patient with Two minor manifestations plus evidence of a

established RHD preceding group A streptococcal infection.

Rheumatic chorea. Other major manifestations or evidence of

Insidious onset rheumatic carditis. a group A streptococcal infection not required.

Chronic valve lesions of RHD (patients Do not require any other criteria to be
presenting for the first time with pure diagnosed as having rheumatic heart
mitral stenosis or mixed mitral valve disease.
disease and/or aortic valve disease). c
* Major manifestations — carditis
— polyarthritis
— chorea
— erythema marginatum
— subcutaneous nodules

** Minor manifestations — clinical: fever, polyarthralgia

— laboratory: elevated acute phase
reactants (erythrocyte sedimentation rate
or leukocyte count)

*** Supporting evidence of a preceding — electrocardiogroram: prolonged P-R interval

streptococcal infection within the last — elevated or rising antistreptolysin-O or other
45 days streptococcal antibody, or
— a positive throat culture, or
— rapid antigen test for group A streptococci, or
— recent scarlet fever

a
Infective endocarditis should be excluded.
b
Some patients with recurrent attacks may not fulfil these criteria.
c
Congenital heart disease should be excluded.
Treatment
• Bed rest, restrict activity until acute phase
reactants return to normal

• Anti-streptococcal therapy
– IV C. Penicillin 50 000U/Kg/dose 6H or oral Penicillin V
250mg 6H (>30kg) for 10 days
– Oral Erythromycin for 10 days if allergic to penicillin

• Anti-inflammatory therapy
– Mild/no carditis: oral aspirin 80-100mg/kg/day in 4 doses
for 2-4 weeks, taper over 4 weeks
– Pericarditis/moderate to severe carditis: oral prednisolon
2mg/kg/day in 2 divided doses for 2-4 weeks, taper with
addition of aspirin as above
Treatment
• Anti-failure medications
– Diuretics, ACEi, digoxin

• Consider early referral to paediatric cardiologist if

heart failure persists or worsens during the acute
phase despite aggressive medical therapy. Surgery
may be indicated.
Treatment
• Secondary prophylaxis of Rheumatic Fever
– IM Benzathine Penicillin 0.6 mega units (<30kg) or 1.2
mega units (>30kg) every 3-4 weeks
– Oral Penicillin V 250mg twice daily
– Oral Erythromycin 250mg twice daily if allergic to
penicillin.

• Duration of prophylaxis
– Until age 21 years or 5 years after last attack of ARF
whichever was longer
– Lifelong for patients with carditis and valvular
involvement
Prognosis
• Prognosis is good if recurrence is prevented by continuous
antibiotic prophylaxis-particularly if no carditis in the initial
attack

• If carditis, half of them can develop chronic rheumatic heart

disease. Recurrence following streptococcal sore throat is
high in patience with previous carditis

• For development of RHD, it takes 10-20 years in western

world but earlier in underdeveloped world due to malignant
nature of the disease

• Mitral valve is most commonly affected, followed by aortic

and tricuspid valves
Thank You