Acute rheumatic fever

for pediatrics UG teaching

1. ACUTE RHEUMATIC FEVER
Dr M.Sanjeevappa
M.D.(paeds)
Asst.Professor
Dept. of Paediatrics
GMC ,Ananthapuramu

2. DEFINITION
Rheumaticfeverisanimmunologically
mediatedinflammatorydisorder,which
occursasasequeltogroupAstreptococcal
pharyngealinfection.

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
Cytotoxicitytheory :
GAS toxin is involved in the pathogenesis of acute
rheumatic fever and rheumatic heart disease.
StreptolysinO has a direct cytotoxiceffect
on mammalian cells in tissue culture.
Immune-mediated theory (molecular mimicry) :
The antigenicityof GAS cellular and extracellular
epitopesand their immunologic cross reactivity with
cardiac antigenic epitopes.

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 DuckettJones criteria.

9. JONES CRITERIA, UPDATED 2015
MAJOR MANIFESTATIONS :
Carditis
Polyarthritis
Erythemamarginatum
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
plusevidence 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)
plusevidence of recent GAS infection.

14. JONES CRITERIA, UPDATED 2015
Arthritis :
Only polyarthritisin Low-Risk populations.
monoarthritisor polyarthralgiain
Moderate/High-Risk populations.

15. JONES CRITERIA, UPDATED 2015
Carditis : clinical and/or subclinical
(echocardiographicvalvulitis).

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 carditisis 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.

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
maculesthat 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

25. INVESTIGATIONS
Throat swab culture
AntistreptolysinO,
Anti–dnaseB,
Antihyaluronidase
CXR
ECG
2D ECHO
CBP
ESR
CRP

26. DIFFERENTIAL DIAGNOSES
Infective Endocarditis
Juvenile Idiopathic Arthritis
Mixed Connective-Tissue Disease
Reactive Arthritis
Rheumatoid Arthritis
Kawasaki Disease
Septic Arthritis

27. TREATMENT
Bed rest and monitored closely for evidence of
carditis.
Antibiotic Therapy :
Single dose of benzathinepenicillin 1.2 mUI.M.
Oral phenoxymethylpenicillin250 mg 6-hourly for
10 days.
Oral amoxycillinfor 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 phenobarbitalis 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.
Benzathinepenicillin 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
carditisbut without
residual heart disease
(no valvulardisease)
Rheumatic fever with
carditisand 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

35. THANK YOU