ACUTE RHEUMATIC FEVER
DEFINITION
Rheumatic fever is an inflammatory process
which can involve the joints, heart, skin and
brain
It is caused by antibody cross reactivity and
occurs 2-3 weeks after a Group A
Streptococcal infection.
EPIDEMIOLOGY
Ages 5-15 yrs are most susceptible
Rare <3 yrs
Girls>boys
Common in 3rd world countries
Environmental factors-- over crowding, poor
sanitation, poverty,
Incidence more during fall ,winter & early spring
Generally occurs 2-3 weeks after Group A Streptococcal
infection (strep throat or scarlet fever)
Globally, 3% of those with an untreated streptococcal
infection develop rheumatic fever
40% of those with Acute Rheumatic Fever develop
mitral stenosis as adults
BACKGROUND
Cutaneous streptococcal infections have not
been shown to initiate Acute Rheumatic
Fever.
Strains of certain M serotypes/genotypes of
streptococci have higher associations than
other genotypes
.
PATHOPHYSIOLOGY
Exact mechanism of how Group A
streptococcal infection causes Acute
Rheumatic Fever is unknown however it is
believed to be caused by a cross reactivity of
antibodies
Suggested Theories
Toxic effects of streptococcal products (streptolysin S
or O) which then cause direct tissue injury
Serum Sickness-like reaction mediated by antigen-
antibody complexes
Autoimmune phenomenon
PATHOPHYSIOLOGY
More support for an autoimmune phenomenon
(Type II hypersensitivity reaction)
During strep infection, antigen presenting
cells present bacterial antigen to helper T
cells. These helper T cells then activate B cells
to induce production of antibodies against the
Streptococcal cell wall.
After a latent period of 1-3 weeks, antibody
induced immunological damage occur to
heart valves,joints, subcutaneous tissue
& basal ganglia of brain
PATHOGENESIS
Most patient have elevated antibody titers to
at least one streptococcal antibody
Streptolysin O
Hyaluronidase
Streptokinase
PATHOPHYSIOLOGY (CARDIAC)
Aschoff nodule with owl-eyed shape in the cross section and catapillar-
shaped
in the longitudinal section
PATHOPHYSIOLOGY (CARDIAC)
Thickened fused chordae of the mitral valve
CLINICAL MANIFESTATIONS
Latent period: time between preceding
streptococcal pharyngitis and Acute
Rheumatic fever is about 19 days (range 1-5
weeks)
If initial complaint is polyarthritis, disease
generally has more abrupt onset compared to
if initial presentation is with myocarditis.
Arthritis occurs in 75% of initial attacks,
carditis in 40-50% and chorea in 15% with
subcutaneous nodules and erythema
marginatum in <10%
CLINICAL MANIFESTATIONS
(CARDITIS)
Usually manifests within the first 3 weeks of
Acute Rheumatic Fever
Signs: new heart murmur, cardiomegaly, CHF,
perciardial friction rub, effusions
Chronic inflammatory changes may lead to
development of rheumatic heart disease.
Characteristic murmur or Rheumatic heart
disease:
mitral regurgitation
Low-pitched mid diastolic flow murmur at the apex (Carey
Coombs murmur
Aortic regurgitation
Can also get AV conduction delays
CARDITIS
Cardiomegaly
primarily prolonged PR
interval
AV conduction delays
CLINICAL MANIFESTATIONS
(JOINTS)
Arthralgias and arthritis (may be
migratory)
Warm, swollen, tender joints
Usually involves the knees, ankles,
elbows and wrists
Lasts 2-3 weeks
Arthralgias and arthritis
CLINICAL MANIFESTATIONS
Subcutaneous Nodules: usually associated
with severe carditis and occur several weeks
after onset.
Firm, painless nodules (up to 2cm) found over bony
surfaces and tendons
Occur near elbows, knees, wrists, achilles tendon,
vertebral joints
Usually persist for 1-2 weeks
SUBCUTANEOUS NODULES
SUBCUTANEOUS NODULES
CLINICAL MANIFESTATIONS
Erythema Marginatum:
nonpruritic, painless
erythematous rash on trunk
and/or proximal extremities
Macular lesions with raised margins and
central clearing
May last from weeks to months
ERYTHEMA MARGINATUM
CLINICAL MANIFESTATIONS
Sydenham’s Chorea: neurologic
disorder with muscular weakness,
emotional lability and involuntary,
uncoordinated, purposeless
movements
Disappear during sleep
Mainly occur in hands, feet and face
Sensation intact
Lasts 2-4 months
Sydenham’s Chorea
DIFFERENTIAL DIAGNOSIS
Poststreptococcal reactive arthritis: is non-
migratory
Rheumatoid Arthritis
SLE
Infective endocarditis
Sickle Cell disease
Drug reactions
TB
Lyme Disease
Serum Sickness
DIAGNOSIS
JONES CRITERIA
Developed by Dr. T Duckett Jones in 1944
Need 2 major criteria or 1 major and 2 minor criteria
in the presence of a prior strep infection to make the
diagnosis
Evidence of prior strep infection with positive throat
culture or antigen test, elevated streptococcal
antibody titer, or history of rheumatic fever/heart
disease
MAJOR CRITERIA
Migratory Polyarthritis: migrating arthritis with
inflammation involving the large joints (knees, ankles,
elbows, wrists) and typically affects the leg joints first
Carditis: can manifest with new murmur, pericarditis,
congestive heart failure
Subcutaneous Nodules: a form of aschoff bodies. Are
painless nodules on the back of the wrists, elbows,
knees
Erythema Marginatum: rash beginning on the arms or
trunk and spreads outward. Lesion with ring with
central clearing. Worsens with heat. Does not involve
the face
Sydenham’s Chorea (St. Vitus’ Dance): purposeless
movements of the face and arms. Late finding
MINOR CRITERIA
Fever
Arthralgia: joint pain without inflammation
Elevated CRP, ESR or leukocytosis
EKG changes: primarily prolonged PR
interval
Evidence of Group A Streptococcal infection
via elevated antistreptolysin O titer or
DNAase
Prior history of rheumatic fever or heart
disease
TREATMENT
Step I - primary prevention
(eradication of streptococci)
Step II - anti inflammatory treatment
(aspirin,steroids)
Step III- supportive management &
management of complications
Step IV- secondary prevention
(prevention of recurrent attacks)
ANTIBIOTICS
Penicillin for at least 10 days
Penicillin 500mg BID-TID
Can use erythromycin for PCN allergic
patients (given at 40mg/kg/day given in 2-4
doses/day)
ANTI-INFLAMMATORY AGENTS
Aspirin 4-8grams/day for adults
Continue anti-inflammatory therapy until ESR
or CRP are normal
May need steroids if there is cardiac
involvement to help prevent sequelae such as
mitral stenosis
Corticosteroids, if indicated, are given at
prednisone 2mg/kg/day for 2 weeks and then
tapered
Supportive management &
management of complications
Bed rest
Treatment of congestive cardiac failure:
ACEI/ARA, digitalis,diuretics
Treatment of chorea:
Diazepam or Haloperidol
Rest to joints & supportive splinting
PROPHYLAXIS
Prophylaxis needed to prevent recurrence of
Acute Rheumatic Fever
Start prophylaxis after acute episode resolves
Can use:
Penicillin V 250mg BID or,
Penicillin G 1.2 million units IM q4weeks
For PCN allergic patients: erythromycin 250mg PO
BID
Recurrence of disease generally occurs in the
first couple years
PROPHYLAXIS
WHO GUIDELINES
At least 5 years of prophylaxis or if child until age 18
if not cardiac involvement
10 years prophylaxis or if child until age 25 if has mild
mitral regurgitation
Lifelong prophylaxis if has severe valve disease
Complications- MS
Complications- AR & AS
Complications- MR
Prognosis
Rheumatic fever can recur whenever the
individual experience new streptococcal
infection,if not on prophylactic medicines
Good prognosis for older age group & if no
carditis during the initial attack
Bad prognosis for younger children & those
with carditis with valvar lesions