2. WHAT IS
RHEUMATIC FEVER ?

3. • Rheumatic fever is an immunologically
mediated inflammatory disorder, which
occurs as a sequel to group A streptococcal
pharyngeal infection.
• Multisystem disease affecting connective
tissue particularly of the heart, joints, brain,
cutaneous and subcutaneous tissues
• RF – not a communicable disease
but results from a communicable disease
(streptococcal pharyngitis).
• The illness is so named because of its
similarity in presentation to rheumatism.

4. • RF  RHD (rheumatic heart
disease); a crippling disease.
• Epidemiological point of view these
cannot be separated.
[WHO CHRONICLE 1969]
• RF and RHD  diseases of the poor
most prevalent in underdeveloped and
developing countries.
• Preventable disease.

5. PROBLEM STATEMENT
• RF and RHD is the most common cause
of heart disease in 5-30 age groups
throughout the world.
• It accounts for 12-65% of hospital
admissions related to CVD in developing
countries.
• There has been marked decrese in cases
of RF and RHD in places that have
implemented preventive programs.

6. IN INDIA
• RHD is prevalent in range of 5-7/1000 in 5-15
age groups.
• About 1 million cases of RHD
• RHD constitutes 20-30% hospital admissions
due to CVD.
• Streptococcal infections commonin children
living in under –privileged conditions and RF
accounts for 1-3% of the cases.

9. • Important cause of chronic disease and death in
developing world
• Underdiagnosed and undertreated
• Ages 5-15 yrs are most susceptible
• Rare <3 yrs
• Common in 3rd world countries
• Environmental factors-- over crowding, poor
sanitation, poverty,poor housing
• Incidence more during fall ,winter & early spring

10. AGENT FAC.
ENVIRONMENTAL
FAC. HOST FAC.

11. AGENT FACTORS
• Streptococcal sore throat
• Not all strains of Group A Streptococci
(GAS) lead to rf
• Rheumatogenic potential
• Recently virus (coxsackie B-4) has been
suggested as causative agent with
streptococcus acting as conditioning
agent.

12. HOST AND ENVIRONMENTAL
FACTORS
• AGEadolescents 5-15 yrs
initial attack at younger age  valvular
lesion faster
Juenile mitral stenosis
• SEX equal
• IMMUNITY Toxic –immunological
hypothesis
• SOCIO-ECONOMIC STATUSSocial
disease
• HIGH RISK GROUP 5-15 yrs school-age
children living in closed community

14. PATHOPHYSIOLOGY

16. • Based on currently based evidence, RF is
caused by group A streptococcal (GAS)
pharyngeal infection.
• Postulated that series of preceding
streptococcal infection is needed to prime
the immune system prior to final infection
that directly causes the disease.

17. • Group A strep pharyngeal infection precedes
clinical manifestations of ARF by 2 - 6 weeks.
• Body produce antibodies against streptococci .
• These antibodies cross react with human
tissues because of the antigenic similarity
between streptococcal components and human
connective tissues (molecular mimicry)
[there is certain amino acid sequence that is similar btw GAS
and human tissue]
• Immunologically mediated inflammation &
damage (autoimmune) to human tissues which
have antigenic similarity with streptococcal
components- like heart, joint, brain connective

18. • Epitopes present in cell wall ,CM, str. M protein
are immunologically similar to molecules in
human myosin, tropomyosin,keratin,actin,etc.
MOLECULAR MIMICRY

19. • Because of the similarity btw hyaluronic
acid in GAS capsule and in the connective
tissue of the joints, Ab produced against
GAS capsule will start to attack the joints
and causes arthritis.
• M-protein in GAS cell wall and the
myocardium are similar, thus Ab produced
against GAS cell wall will attack heart and
will cause carditis and so forth.
• Similarly Ab against NAG in GAS will
affect cardiac valve tissue causing valvular
damage.

20. Characteristic Aschoff bodies , composed of
swollen eosinophilic collagen surrounded by
lymphocytes and macrophages can be seen on
light microscopy. The larger macrophages may
become Aschoff giant cells
In order for R.F. to occur:
 There must be throat infection by GAS.(only
when there is GAS throat infection there is
R.F.)
 Antibodies must be produced by the body
rapidly & in high magnitude.
 These Abs will cross react with tissue of the
heart, joint, brain (especially basal ganglia),

21. CLINICAL FEATURES

22. STREPTOCOCCUS SORE THROAT
• Tender lymph nodes
• Close contact with infected
person
• Scarlet fever rash
• Excoriated nares( crusted
lesions) in infants
• Tonsillar exudates in older children
• Abdominal pain
• GOLD STANDARD POSITIVE THROAT CULTURE

24. FEATURES
Following upper airway infection with GAS
Silent period of 2 - 6 weeks
Sudden onset of fever, pallor, malaise,
fatigue.
Commonly GAS streptococcal infection is
subclinical; such cases confirmed using
streptococcal antibody testing .

25. MAJOR MANIFESTATIONS
Polyarthritis
Carditis
Sydenham’s chorea
Erythema marginatum
Subcutaneous nodules

26. MINOR
MANIFESTATIONS
Involvement
of lung,
Fever Epistaxis
kidneys and
CNS
Arthralgia Serositis

28. 1.POLYARTHRITIS
Most common feature: present in 90% of
patients
Joint is arthritic  ie inflammed.
 Painful, migratory, short duration.
 Usually >5 joints affected and mainly large
joints
Knees, ankles, wrists, elbows, shoulders
 Small joints and cervical spine less commonly
involved

29.  Excellent response of salicylates and
NSAIDS
Pain and swelling come on quickly and
subsides within 5-7 days
In children below 5 yrs arthritis usually
mild but carditis more prominent
Arthritis do not progress to chronic disease

30. 2.CARDITIS
• Early and most serious manifestation
• Manifest as pancarditis
• Occur in 60-70% of cases
• Carditis is the only manifestation of
rheumatic fever that leaves a sequelae &
permanent damage to the organ
• Valvular damage is the hallmark of RF
• Chronic phase- fibrosis,calcification &
stenosis of heart valves(fishmouth valves)

31. Any cardiac tissue may be affected
 Valvular lesion most common: mitral and
aortic
 Seldom see isolated pericarditis or
myocarditis
RHEUMATIC HEART DISEASE
• Rheumatic Heart Disease is the permanent
heart valve damage resulting from one or more
attacks of ARF.
• It is thought that 40-60% of patients with ARF
will go on to developing RHD.
• Sadly, RHD can go undetected with the result
that patients present with debilitating heart

32. High pulse
rate
mitral or aortic regurgitation-endocardium
Murmur
involved
Cardiomegaly myocardium involvement
Pericardial
friction rub Pericarditis
Prolonged PR Myocardial inflammation affecting
interval electrical conduction
Cardiac
failure

33. Investigations for evidence of carditis
• Chest x-ray – cardiomegaly, pulmonary
venous congestion
• ECG- First degree A-V block, T wave
changes, low voltage QRS
• Echocardiogram – cardiac dilatation, valve
involvement, pericardial effusion

35. Rheumatic heart
disease.
Abnormal mitral
valve. Thick, fused
chordae

36. Another view of
thick and fused
mitral valves in
Rheumatic heart
disease

37. 3.SYNDENHAM’ S CHOREA
• Occur in 5-10% of cases
• Mainly in girls of 1-15 yrs age
• Late manifestation of RF -months after
infection
• Spasmodic, unintentional, jerky choreiform
movements,
• Speech affected, fidgety
• Choreiform movements particularly affect the
head(darting movement of tongue)and upper

38. • First sign: difficulty walking, talking, writing
• Occurs in 30% of patients with ARF
• Usually benign and resolves in 2 - 3
months
• Disappears leaving no residual damage.

39. 4.ERYTHEMA MARGINATUM
• Occur in <7%.
• Unique,transient,serpiginous-looking
lesions of 1-2 inches in size
• Pink macules - Clear centrally ,serpiginous
spreading edge .
• More on trunks & limbs & non-itchy
• Almost never on face
• Worsens with application of heat
• Often associated with chronic carditis

41. 5.SUBCUTANEOUS NODULES
• Small,painless, mobile hard lumps beneath skin.
• Most common along -
extensor surfaces of joint-Knees, elbows,
wrists
• Also: on bony prominences, tendons, dorsi of
feet,occiput or cervical spine
• Appears 4 weeks after onset of RF
• Delayed manifestation, disappears –leaves no
residual damage.
• Occur in 9 - 20% of cases
• Often associated with carditis

42. Nodules -Firm, non-tender, isolated or in
clusters

43. 6.FEVER
• Present at onset of
acute illness
• High grade fever
>39ºC
• Lasts for about 12
weeks ,tends to recur

44. 7.EPISTAXIS

45. LAB DIAGNOSIS
• High ESR
• Anemia, leucocytosis
• Elevated C-reactive protien
• Elevated ASO or other streptococcal
antibody titer
• Anti-DNAse B test
• Throat culture-GABHstreptococci

47. • There is no definitive test.
• Diagnosis of ARF relies on presence of
combination of typical clinical features
together with evidence of the precipitating
GAS infection .
• This uncertainty led Dr.T.Duckett Jones in
1944to develop a set of criteria Jones
Criteria to aid diagnosis.
• Now Diagnosis based on MODIFIED JONES
CRITERIA .

48. 2002–2003 World Health Organization Criteria for the
Diagnosis of Rheumatic Fever and Rheumatic Heart Disease
(Based on the 1992 Revised Jones Criteria)
Diagnostic Categories Criteria
Primary episode of rheumatic fever Two major or one major and two minor
manifestations plus evidence of preceding
group A streptococcal infection
Recurrent attack of rheumatic fever in a Two major or one major and two minor
patient without established rheumatic heart manifestations plus evidence of preceding
disease group A streptococcal infection
Recurrent attack of rheumatic fever in a Two minor manifestations plus evidence of
patient with established rheumatic heart preceding group A streptococcal infectionc
disease
Rheumatic chorea Other major manifestations or evidence of
Insidious onset rheumatic carditis group A streptococcal infection not
required
Chronic valve lesions of rheumatic heart Do not require any other criteria to be
disease (patients presenting for the first diagnosed as having rheumatic heart
time with pure mitral stenosis or mixed disease

49. Major manifestations Carditis
Polyarthritis
Chorea
Erythema marginatum
Subcutaneous nodules
Minor manifestations Clinical: fever, polyarthralgia
Laboratory: elevated erythrocyte
sedimentation rate or leukocyte counte
Electrocardiogram: prolonged P-R interval
Supporting evidence of a preceding Elevated or rising anti-streptolysin O or
streptococcal infection within the last 45 other streptococcal antibody, or
days
A positive throat culture, or
Rapid antigen test for group A
streptococcus, or
Recent scarlet fevere

50. Source: Reprinted with permission from WHO Expert
Consultation on Rheumatic Fever and Rheumatic Heart Disease
(2001: Geneva, Switzerland): Rheumatic Fever and Rheumatic
Heart Disease: Report of a WHO Expert Consultation (WHO Tech
Rep Ser, 923). Geneva, World Health Organization, 2004.

51. Exceptions to Jones Criteria
 Chorea alone, if other causes have been
excluded
 Insidious or late-onset carditis with no
other explanation
 Patients with documented RHD or prior
rheumatic fever,one major criterion,or of
fever,arthralgia or high CRP suggests
recurrence

53. • 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)

54. PRIMARY PREVENTION
• AIM ; Prevent the first attack of RF, by identifying all
patients with streptococcal throat infection and
treating them with pencillin
• Theoretically simple , in practise its difficult,
not feasible.
• Many infections are inapparent or if apparent are not
brought to attention of health services
• VIABLE APPROACH; concentrate on high risk
groups ie school age children.
• Surveillance for streptococcal pharyngitis

55. STEP I:Primary Prevention of Rheumatic
Fever (Treatment of Streptococcal Tonsillopharyngitis)
• Agent Dose Mode Duration
• Benzathine penicillin G 600 000 U for patients Intramuscular Once
27 kg (60 lb) Least
1 200 000 U for patients >27 kg
expensive
or method
• Penicillin V Children: 250 mg 2-3 times daily Oral 10 d
(phenoxymethyl penicillin) Adolescents and adults:
500 mg 2-3 times daily
• For individuals allergic to penicillin
• Erythromycin: 20-40 mg/kg/d 2-4 times daily Oral 10 d
Estolate (maximum 1 g/d)
or
• Ethylsuccinate 40 mg/kg/d 2-4 times daily Oral 10 d
(maximum 1 g/d)
Recommendations of American Heart Association

56. Step II: Anti inflammatory treatment
CLINICAL CONDITION DRUG
Arthritis only Aspirin 75-100 mg/kg/day , give
as 4 divided doses for 6 weeks
(attain a body level 20-30 mg/dl)
Carditis Corticosteroids 1-2 mg/kg per day
– for 4-6 weeks to be tapered off

57. 3.Step III: Supportive management &
management of complications
• Bed rest
• Treatment of congestive cardiac failure:
-digitalis,diuretics
• Treatment of chorea:
-diazepam or haloperidol
• Rest to joints & supportive splinting

58. STEP IV : Secondary Prevention of Rheumatic Fever
(Prevention of Recurrent Attacks)
Agent Dose Mode
Benzathine penicillin G 1 200 000 U every 3 weeks* Intramuscular
or
Penicillin V 250 mg twice daily Oral
For individuals allergic to penicillin and sulfadiazine
Erythromycin 250 mg twice daily Oral
Recommendations of American Heart Association

59. Duration of Secondary Rheumatic Fever
Prophylaxis
Category Duration
Rheumatic fever without carditis At least 5 y or until age 18 y,
whichever is longer
Rheumatic fever with carditis and At least 10 y since last residual
heart disease episode and at least until age 40 y
(persistent valvar disease*), sometimes lifelong prophylaxis
Rheumatic fever with carditis 10 y or well into adulthood,
but no residual heart disease whichever is longer
(no valvar disease*)
More severe valvular disease Lifelong
Post-valve surgery cases
*Clinical or echocardiographic evidence.
Recommendations of Am erican Heart Association

60. • Secondary prophylaxis is more
effective when done on a Register
based method
• A register of cases of RF and RHD is
kept.
• This is used to improve treatment
adherence in order to prevent
recurrent RF and the development of
RHD, necessitating surgery.

61. NON- MEDICATED MEASURES
• Improvement of living standards.
• Breaking the poverty –disease –poverty
cycle.
• Improvements in socio-economic
EVALUATION
conditions.
• The prevalence of RHD in school children from
periodic surveys of random samples.
• Samples of school in 6-14 age groups. At 5 year
interval.
• Recommended sample size 20,000 to 30,000

62. PROGNOSIS
• Rheumatic fever can recur whenever the
individual experience new GABH
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

64. Rheumatic heart disease is the only truly
preventable chronic heart condition

65. Rapid, direct test kit for diagnosis of group A
infections, throat swab introduced to latex beads
and monoclonal antibodies
Positive- Negative
the C-carbohydrate -milky smooth
on group A streptococci reaction.
causes clumping