Rheumatic fever is an immunological response following group A streptococcal infection, primarily affecting the heart, brain, joints, and skin, with symptoms including migratory polyarthritis, carditis, and Sydenham's chorea. Peak incidence occurs in children aged 5-15, particularly in developing countries, and diagnosis is based on modified Jones criteria. Management includes antibiotics, anti-inflammatory agents, and education on prevention, with prognosis generally favorable for those without cardiac damage.

1. RHEUMATIC FEVER
Presenter:Dr Bijith
Moderator-Dr Sneha

2. INTRODUCTION
 It is an immunological response that occurs as a
delayed sequelae of GROUP A STREPTOCOCCAL
infection of phaynx.
 It is a diffuse inflammatory disease of connective
tissue primarily involving heart,brain,joints and
skin.

3. ETIOLOGY
 2/3rd
patients with an acute episode of rheumatic fever have history
of an upper respiratory tract infection weeks before.
 Peak age & seasonal incidence of acute rheumatic fever closely
parallel that of group A streptococcus pharyngitis.
 Patients with Acute rheumatic fever almost always have serological
evidence of Group A streptococcus infection
 Serotypes of Group A streptococcus (M types 1,3,5,6,18,29)are more
frequently isolated from patients with acute rheumatic fever.

4. Epidemiology
 Ages 5-15 yrs are most susceptible( peak at 8 yrs of age)
 Girls>boys
 Environmental factors-over crowding, poor sanitation,
poverty.
 Incidence more during fall ,winter & early spring
 In developing countries incidence is 50/100,000.
 Worldwide RHD remains the most common of acquired
heart disease in all age groups accounting for 50%.

5. Pathogenesis
 Delayed immune response to infection with group A
beta hemolytic streptococci.
 Most common serotypes isolated are GAS M type
1,3,5,6,18,29
 After a latent period of 1-5 weeks, antibody induced
immunological damage occur to heart valves, joints,
subcutaneous tissue & brain.

6. Cytotoxic theory-
 streptolysin O(direct cytotoxic effect).
Immune mediated-
 crossreactivity of GAS epitopes ( M Protein,cell
membrane,capsular hyaluronate, cell wall
carbohydrate)with cardiac antigenic epitopes.
 Recently proposed hypothesis is-Binding of M protein N
terminus domain to a region of type IV collagen leading to
ground substance inflammation of valves and myocardium.

7. Clinical manifestations

8. Modified JONES (2015)criteria
 Low risk populaion- ARF incidence <2 per 100000
school aged children(5-14 yrs) per year or on all age
prevalence of RHD <1 per 1000 population per year.
Rest are Moderate/High-risk populations.

9. Major criteria

10. Minor criteria

11.  Initial Attack- 2 major,
or 1 major plus 2 minor manifestations ,
plus evidence of recent GAS infection.
 Recurrent attack- 2 major,
or 1 major and 2 minor,
or 3 minor manifestations (only in high risk
population),
plus evidence of recent GAS infection.

12. CLINICAL FEATURES
MIGRATORY POLY ARTHRITIS:
 Occur in 75%
 Involved joints are swollen ,tender , hot and limitation of
motion is seen.
 Commonly involved joints are knee,ankle,elbow & wrist.
 More than one joint is involved simultaneously or in
succession and MIGRATORY.
 Aseptic monoarthritis may be seen in selected high risk
population.
 Response to salicylate therapy within 48 hrs.

13. Subsides within few days even without
treatment.
Arthritis is the earliest manifestations of
rheumatic fever & correlate temporally
with peak antistreptococcal antibody titers.
No permanent damage

14. CARDITIS:
 Occurs in 50-60% cases.
 Rhuematic carditis characterised by pancarditis.
 Endocarditis (valvulitis) is a universal finding in
rheumatic carditis.
 In rheumatic myocarditis contractility is rarely
impaired.
 Valvular lesions –mitral & less commonly aortic and
rarely triscuspid and pulmonary valves.
Subclinical carditis is defined as without murmur but
echo evidence of valvulitis.

15. Doppler criteria:
Pathological mitral regurgitation—four criteria (all must be met):
Visible at least in two projections
 Regurgitation jet length 2 cm at least in one projection
≥
 Regurgitation peak velocity > 3 m/s.
 Regurgitation pansystolic
Pathological aortic regurgitation—four criteria (all must be met):
Visible at least in two projections
 Regurgitation jet length 1 cm at least in one projection
≥
 Regurgitation peak velocity > 3 m/s.
 Regurgitation pandiastolic

16.  Carey coombs murmur-diastolic murmur due to
increased flow across thickened mitral valve.
 Can be differentiated from mitral valve stenosis
by absence of opening snap.
 As soon as the valvulitis resolves,the murmur
disappears.

17. Signs include some or all of the following
Tachycardia- out of proportion to fever.
Murmurs
Pericarditis with friction rub,effusion,chest
pain
Cardiomegaly on CXR – indicates valvulitis
Features of congestive heart failure
Causes permanent cardiac damage.
Mild carditis disappear in wks & severe carditis
lasts for 2-6 months.

18. SYDENHAMS CHOREA:
 Also called st.vitus dance
 Occur in 10-15% of cases
 Mainly in girls of 8-12 yrs age
 Neuropsychiatric disorder with
neurological signs like choreic movements
hypotonia

19.  Psychiatric signs like emotional lability
hyperactivity
separation anxiety
obsessions and compulsions.
 Psychiatric symptoms appear initially and are
replaced by spontaneous, purposeless movements of
CHOREA followed by motor weakness.

21.  Exacerbated by stress and disappear with sleep.
 Clinical maneuvers to elicit chorea include
 milkmaids grip
 spooning and pronation of hands when arms are
extended.
 wormian darting movements of tongue on
protrusion
 hand writing(MICROGRAPHIA)
 Elevated ANTI NEURONAL antibodies.
 Gradually subsides in 6-7 months.
 No permanent neurologic sequalae.

23. ERYTHEMA MARGINATUM:
 Seen in less approximately 1%
 Non pruritic serpiginous or annular
erythematous rashes with pale centre.
 Prominent on trunk and on inner side of
extremities, but not on the face.
 Dissapear on exposure to cold and reappear
after a hot shower.

24. SUBCUTANEOUS NODULES:
 Occur in <1%.
 Commonly seen in recurrences.
 Never a sole manifestation
 Hard,painless,non pruritic,freely movable
 Approximately 1cm in size.
 Along extensor aspect of joints near bony
prominences, over scalp & along the spine.
 Lasts for weeks
 Significant association with carditis.

25. Minor criteria
 Arthalgia- joint pain without objective changes.
-NOT TO BE USED AS MINOR CRITERIA WHEN
ARTHRITIS IS TAKEN AS MAJOR CRITERIA.
 FEVER
 Elevated CRP&ESR -suggests inflammation
 Prolonged PR interval –neither specific for ARF nor
indicates active carditis.

26. Evidence of recent infection
 Increased or rising ASO titre or other streptococcal
antibodies
 Positive throat culture for GAS.
 Positive rapid group A streptococcal carbohydrate
antigen test in a child.
 Onset of clinical manifestations coincides with peak
antibody response.

27. ASO TITRES:
 most widely used
 Elevated in 80 – 85% with Acute rheumatic fever
& 20% normal individuals.
 67% with isolated chorea have elevated ASO.
 Elevation- 333 Todd units in children
250 todd units in adults
 A single low titre doesn’t exclude the diagnosis.
 Antistreptolysin O ,anti Dnase B ,
antihyaluronidase are elevated in 95-100%.

28. STREPTOZYME TEST:
 Screening test for antibodies to streptococcal
antigens NADase,DNase,streptokinase,streptolysin O
and hyaluronidase.
 Can detect several antibodies in a single assay
 Technically quick & easy.
 But less standardized , less reproducible than other
tests.

29. DIAGNOSIS
 Two major are always stronger than one major plus
two minor
 Arthralgia or prolonged PR cannot be used as minor
criteria when arthritis and carditis are used as major
criteria.
 Absence of evidence of antecedent infection makes
the diagnosis of ARF unlikely.
 Sub therapeutic doses of aspirin may suppress full
manifestations.

30. Exception to JONES criteria include
 Chorea may occur as single manifestation.
 Indolent carditis only manifestation in those who
seek medical attention months after ARF.
 Reccurences may not fulfill the JONES criteria.

31. Differential diagnosis
 Juvenile rheumatoid arthritis-involves peripheral
small joints
-Symmetrical involvement of large joints without
migratory arthritis
-Pallor of joints
-less response to salicylates
-No evidence of previous streptococcal infection.
 SLE

32.  Reactive arthritis
 Gonococcal arthritis
 Virus associated arthritis
 Infective endocarditis
 Hematological disorders like leukemia , sickle cell
anemia.

33. MANAGEMENT
Investigations:
 CBC
 ESR
 CRP
 Throat culture
 ASO titre
 CXR
 2D ECHO
 Doppler

34. TREATMENT
 All patients with acute rheumatic fever should be placed on
bed rest & monitor for evidence of carditis.
Antibiotic therapy:
 Patient should receive 10 days of oral penicillin or amoxicillin or a
single intramuscular Benzathine penicillinG to ensure eradication
of group A streptococcus from upper respiratory tract
 If penicillin allergic 10 days of Erythromycin ,Azithromycin
(5days) or clindamycin used.
 Anti inflammatory or suppressive therapy with steroids should
not be started until definitive diagnosis is made.

35.  Once diagnosis is confirmed, parent education to
prevent subsequent infection through continuous
antibiotic prophylaxis.
 Bed rest-1-2weeks for isolated arthritis and several
wks for severe carditis followed by indoor
ambulation.
 ESR helpful tool to determine the duration of
restriction of activities

36. Anti inflammatory agents:
 Patients with typical migratory poly arthritis and
those with carditis should be treated with oral
salicylates.
 Dose of Aspirin is50-70mg/kg/day in 4 divided doses
PO for 3-5 days,followed by 50mg/kg/day 4divided
doses for 3wks and half that dose for another 2-
4wks. Arthritis alone Mild carditis Moderate
carditis
Severe carditis
prednisolone --------------------- ------------------- ---------------------- 2-6 wks
Aspirin 1-2 wks 3-4 wks 6-8 wks 2-4 months

37.  Patients with carditis more than minimal
cardiomegaly or congestive heart failure should
receive corticosteriods.
 Prednisone – 2mg/kg /day as 4 divided doses for 2-
3weeks f/b half the dose for 2-3wks& then tapering by
5mg/24hr every 2-3days.
 When prednisone is being tapered, aspirin
50mg/kg/day in 4 divided doses for 6 weeks should
started to prevent rebound inflammation.

38. CHF:
Complete bed rest
Prednisolone for severe carditis
Digoxin-begin with half the recommended
dose because of supersensitivity to digitalis.
Diuretics

39. CHOREA:
 Reduce physical activity and stress
 Benzathine penicillin 1.2 million units,i.m initially for
eradication and every 28 days for recurrence prevention.
 Anti inflammatory agents not needed.
 phenobarbital- 15 to 30 mg every 6-8 hrs
-haloperidol 0.01-0.03mg/kg/24hrs BD PO
-chlorpramazine (0.5mg/kg every 4-6hrs).
-corticosteriods.

40. COMPLICATIONS
Arthritis and chorea resolve completely without
sequelae.
Infective endocarditis may develop.
-So penicillin prophylaxis should start in those
with prosthetic valve or prosthetic material
used in valve repair.
-Maintain oral hygeine.

41. Prognosis
 Presence or absence of cardiac damage determines the
prognosis
 50-70% of patients with carditis recover with no residual
heart disease.
 Development of Residual heart disease influenced by
-cardiac status at start of treatment
-recurrence
 20% of patients who present with pure chorea who are not
given secondary prophylaxis develop RHD with in 20yrs.

42. Prevention
Prevention of initial attacks depends on identification &
eradication of group A sreptococcus causing acute
pharynigitis.
Primary prophylaxis:
antibiotic therapy started before the 9th
day of symptoms of
acute GAS pharyngitis.
Secondary prophylaxis:
Indicated in patients with documented history of rheumatic
fever
isolated chorea
those without evidence of rheumatic heart disease

43. antibiotic dose route frequency
BENZATHINE
PENICILLING
<27kgs -6lakh units & >
27kgs-1.2 million units
IM EVERY 28 DAYS
PENICILLIN V 250mg ORAL BD
SULFADIAZINE < 27 kg -0.5 gr & >
27kgs-1 gr
ORAL OD
ERYTHROMYCIN 250mg ORAL BD

44. DURATION
category duration
Rheumatic fever without carditis Atleast for 5 yrs or until 21yrs whichever is
longer
Rheumatic fever with carditis but no valvular
disease
Atleast for 10 yrs or until 21 yr of age.
Rheumatic fever with valvular heart disease
and carditis
Atleast 10 yrs since last episode or until 40
yrs,sometimes lifelong

46. references
 Nelson textbook of pediatrics
 park textbook of cardiology 6th
edition