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Acute rheumatic fever
1. ACUTE RHEUMATIC FEVER
Dr M.Sanjeevappa
M.D.(paeds)
Asst.Professor
Dept. of Paediatrics
GMC ,Ananthapuramu
2. DEFINITION
Rheumatic fever is an immunologically
mediated inflammatory disorder, which
occurs as a sequel to group A streptococcal
pharyngeal infection.
3. EPIDEMIOLOGY
Incidence of ARF in India is varies from
0.42–10.9 per 1,000 population.
It is most common in children from 5 to 15
years of age.
It is rampant in the Middle East, in sub-
Saharan Africa, in the Indian subcontinent.
ARF does not have sexual predilection
4. ETIOLOGY
Risk of developing ARF after an episode of
streptococcal pharyngitis is 0.3-3%.
GAS strains M type 4 , 1, 3, 5, 6, 18, 29.
Two-thirds of patients with ARF have history
of an upper respiratory tract infection.
5. PATHOGENESIS
Cytotoxicity theory :
GAS toxin is involved in the pathogenesis of acute
rheumatic fever and rheumatic heart disease.
Streptolysin O has a direct cytotoxic effect
on mammalian cells in tissue culture.
Immune-mediated theory (molecular mimicry) :
The antigenicity of GAS cellular and extracellular
epitopes and their immunologic cross reactivity with
cardiac antigenic epitopes.
6.
7. CLINICAL MANIFESTATIONS
Multisystem disorder that usually presents with
–Fever
–Anorexia
–Lethargy
–Joint pain
Latent period: 2–3 weeks after an episode of
streptococcal pharyngitis.
Revised Duckett Jones criteria.
8.
9. JONES CRITERIA, UPDATED 2015
MAJOR MANIFESTATIONS :
Carditis
Polyarthritis
Erythema marginatum
Subcutaneous nodules
Chorea
10. JONES CRITERIA, UPDATED 2015
MINOR MANIFESTATIONS :
Clinical features:
Arthralgia
Fever
Laboratory features:
Elevated acute phase reactants:
- ESR
- C-reactive protein
Prolonged P-R interval
11. JONES CRITERIA, UPDATED 2015
SUPPORTING EVIDENCE OF ANTECEDENT
GAS INFECTION :
Positive throat culture or
Rapid streptococcal antigen test or
Elevated or increasing streptococcal antibody titer.
12. JONES CRITERIA, UPDATED 2015
Low-Risk population : ARF incidence <2 per
100,000 school-age children per year, or all-age
RHD prevalence of <1 per 1000 population.
Moderate / High-Risk population : ARF incidence
>2 per 100,000 school-age children per year, or
all-age RHD prevalence of >1 per 1000 population.
13. JONES CRITERIA, UPDATED 2015
Initial attack of ARF :
2 major manifestations or
1 major and 2 minor manifestations
plus evidence of recent GAS infection.
Recurrent attack of ARF :
2 major manifestations or
1 major and 2 minor or
3 minor manifestations (in the Moderate/High-Risk
population)
plus evidence of recent GAS infection.
14. JONES CRITERIA, UPDATED 2015
Arthritis :
Only polyarthritis in Low-Risk populations.
monoarthritis or polyarthralgia in
Moderate/High-Risk populations.
16. JONES CRITERIA, UPDATED 2015
Minor criteria :
Moderate/High-Risk group Low-Risk group
monoarthralgia polyarthralgia
Fever >38° C >38.5° C
ESR >30 mm/hr >60 mm/hr
17. EXCEPTION FOR JONES CRITERIA
(1) when chorea occurs as the only major
manifestation of acute rheumatic fever.
(2) when indolent carditis is the only manifestation
in patients who first come to medical attention only
months after the apparent onset of acute
rheumatic fever.
(3) In recurrences of acute rheumatic fever in high-
risk populations.
18.
19. ARTHRITIS
Most common and early
manifestation.
Occurs in 75% of patients
Acute painful asymmetric and
migratory inflammation of the
large joints
Typically affects the knees,
ankles, elbows and wrists.
Pain characteristically
responds to aspirin
If not, the diagnosis is in doubt
20. CARDITIS
Carditis occurs in approximately 50-60% of all
cases of ARF.
Involves the endocardium, myocardium and
pericardium.
Incidence declines with increasing age - ranging
from 90% at 3 years to around 30% in
adolescence.
21. ERYTHEMA MARGINATUM
Occurs in < 5% of patients.
Lesions start as red
macules that fade in the
centre but remain red at
the edges.
Occur mainly on the trunk
and proximal extremities
but not the face.
22. SUBCUTANEOUS NODULES
Occur in 5–7% of patients.
Small (0.5–2.0 cm), firm and
painless.
Best felt over extensor
surfaces of bone or tendons.
Appear more than 3 weeks
after group A streptococcal
pharyngeal infection.
23. SYDENHAM’S CHOREA (ST VITUS DANCE)
Late neurological manifestation.
Appears at least 3 months after the episode of ARF.
all the other signs may have disappeared.
Occurs in up to 1/3rd of cases and is more common in
females.
Emotional breakdown or changes may be the first
feature.
Typically followed by purposeless, involuntary
choreiform movements of the hands, feet or face.
Speech may be explosive and halting.
Spontaneous recovery usually occurs within a few
months
27. TREATMENT
Bed rest and monitored closely for evidence of
carditis.
Antibiotic Therapy :
Single dose of benzathine penicillin 1.2 mU I.M.
Oral phenoxymethylpenicillin 250 mg 6-hourly for
10 days.
Oral amoxycillin for 10 days.
Penicillin-allergic:
Erythromycin -10 days.
azithromycin - 5 days.
28. ANTI INFLAMMATORY THERAPY
Migratory polyarthritis :
Aspirin is drug of choice.
Dosage :
50-70 mg/kg/day in 4 divided doses PO for 3-5 days.
followed by
50 mg/kg/day in 4 divided doses PO for 3 weeks
and
Half that dose for another 2-4 weeks.
29. ANTI INFLAMMATORY THERAPY
Carditis : prednisone is drug of choice.
Dosage :
2 mg/kg/day in 4 divided doses for 2-3 weeks
Followed by
1 mg/kg/day for 2-3 weeks
tapering of the dose by
5 mg/day every 2-3 days.
When prednisone is being tapered, aspirin should
be started at 50 mg/kg/day in 4 divided doses for 6
wk to prevent rebound of inflammation.
30. SYDENHAM CHOREA
Phenobarbital is the drug of choice.
Dosage : 16-32 mg every 6-8 hr PO.
If phenobarbital is ineffective :
Haloperidol (0.01-0.03 mg/ kg/24 hr divided bid PO)
or
Chlorpromazine (0.5 mg/kg every4-6 hr PO).
31. COMPLICATIONS
The arthritis and chorea of acute rheumatic fever
resolve completely without sequelae.
The long-term sequelae of rheumatic fever is
RHD.
32. PREVENTION
Primary Prevention :
Appropriate antibiotic therapy instituted before the
9th day of symptoms of acute GAS pharyngitis is
highly effective in preventing first attacks of acute
rheumatic fever.
33. PREVENTION
Secondary prevention :
prevention of recurrent attacks of RF.
Benzathine penicillin G
1.2 million units IM every 3 weeks if wt > 27 kg
0.6 million units IM every 3 weeks if wt < 27 kg
Or
Penicillin V 250 mg twice daily orally.
If allergic to both – Erythromycin 250 mg twice
daily orally
34. DURATION OF SECONDERY PROPHYLAXIS
CATEGORY
Rheumatic fever
without carditis
Rheumatic fever with
carditis but without
residual heart disease
(no valvular disease)
Rheumatic fever with
carditis and residual
heart disease
(persistent valvular
disease)
DURATION
5 yr or until 21 yr of age,
whichever is longer.
10 yr or until 21 yr of age,
whichever is longer
10 yr or until 40 yr of age,
whichever is longer
sometimes
lifelong prophylaxis