1. Acute Rheumatic Fever
Dr. Basem Enany, MD
Lecturer of Cardiology

2.  The initial illness is usually characterized by a sore
throat (pharyngitis) that may be followed, within
approximately 1 to 5 weeks, by the sudden (acute)
onset of rheumatic fever.
 "latent period."
 ARF is an inflammatory disease following group A
streptococcal infection (i.e., sequelae) multiple
tissues and organs (joints, skin, subcutaneous
tissues, heart, and brain).

4. Diagrammatic structure of the group A
beta hemolytic streptococcus
Capsule
Cell wall
Protein antigens
Group carbohydrate
Peptidoglycan
Cyto.membrane
Cytoplasm
……………………………………………
……...
Antigen of outer
protein cell wall
of GABHS
induces antibody
response in
victim which
result in
autoimmune
damage to heart
valves,
sub cutaneous
tissue,tendons,
joints & basal
ganglia of brain

5. Evidence of AUTOIMMUNITY
INDUCED BY STREPT. ANTIGENS
 Gamma-globulins in sarcolemma
of myofibrils
 Circulating ab. to heart tissue.
 No strept. can be found in
lesions.

6.  Not all of the serotypes of group A streptococci
can cause rheumatic fever. The rheumatogenic
serotypes are thought to include 1, 3, 5, 6, 14,
18, 19, and 24.
 Pharyngitis- produced by GABHS can lead to-
acute rheumatic fever , rheumatic heart disease
& post strept. Glomerulonepritis
 Skin infection- produced by GABHS leads to
post streptococcal glomerulo nephritis only.
Group A Beta Hemolytic Streptococcus

7. INCIDENCE
 20 to 50 per 100,000 /year during the period of 1940 to
1960 and declined to 1/100,000/year in 1970s.
 100/100,000/year of ARF/RHD among the younger age
group of the socially disadvantaged population.
THE ATTACK RATE
(INCIDENCE OF ARF IN PTS WITH STREPT. PHARYNGITIS)
 3% OF UNTREATED PATIENTS
 5-50% IN PTS WITH PREVIOUS ATTACKS
EPIDEMIOLOGY
 SOCIO-ECONOMIC STATUS
 OUT BREAKS OF STREPT PHARYNGITIS

8. CARDIOVASCULAR LESIONS
 MYOCARDIUM (ASCHOFF BODY)
 ENDOCARDIUM
 PERICARDIUM
EXTRACARDIAC LESIONS
 JOINTS
 SKIN
 LUNGS AND PLEURA
 CNS

9. •On pathological examination, the valves are thickened
and display rows of small vegetations along their apposing
surfaces
•Inflammation of the valves consists of oedema and
mononuclear cell infiltration of the valvular tissue and the
chordae tendineae in the acute phase; fibrosis and
calcification occur with maintenance of the inflammatory
process.
•Myocarditis is characterised by infiltration of
mononuclear cells, vasculitis and degenerative changes of
the interstitial connective tissue.
•The pathognomonic lesion is the Aschoff body in the
proliferative stage, present in 30 to 40 per cent of biopsies
of patients with acute RF

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

11. Aortic valve showing active valvulitis. The valve is slightly thickened
and displays small vegetations – "verrucae"

12. •Stenotic mitral
valve seen from
left atrium.
•Both
commissures
are fused; the
cusps are
severely
thickened.
•The left atrium
is huge.

13. Myocardial Aschoff body – the cells are large, elongated, with large
nuclei; some are multinucleate

14. Acute Rheumatic vegetations:

15. Chronic RHD:
 Valve leaflet
thickening.
 Shortening,
thickening and
fusion of
tendinous cords.

16. Fibrinous Pericarditis:

18. Jones Criteria (Revised) for Guidance in the
Diagnosis of Rheumatic Fever*
Major Manifestation Minor
Manifestations
Supporting Evidence
of Streptococal Infection
Clinical LaboratoryCarditis
Polyarthritis
Chorea
Erythema Marginatum
Subcutaneous Nodules
Previous
rheumatic
fever or
rheumatic
heart disease
Arthralgia
Fever
Acute phase
reactants:
Erythrocyte
sedimentation
rate,
C-reactive
protein,
leukocytosis
Prolonged P-
R interval
Increased Titer of Anti-
Streptococcal Antibodies ASO
(anti-streptolysin O),
others
Positive Throat Culture
for Group A Streptococcus
Recent Scarlet Fever
*The presence of two major criteria, or of one major and two minor criteria,
indicates a high probability of acute rheumatic fever, if supported by evidence of
Group A streptococcal nfection.
Recommendations of the American Heart Association

19. Pitfalls in diagnosis
 John’s criteria is only a guideline
Problems with over diagnosis
 A minor illness is misdiagnosed as ARF  unnecessarily
therapy
 cardiac neurotic
Problems with under diagnosis
 another disease treatment for a non existent disease
 No long term prophylaxis

20. ARTHRITIS
most common
 IN 70% OF CASES
 ACUTE MIGRATORY ASYMMETRIC
POLYARTHRITIS
 USUALLY LARGE JOINTS
 Involved joint is swollen and exquisitely
painful and tender.
 RESOLVES WITHIN 1-3 WEEKS
 RESPONDS QUICKLY TO SALICYLATES,
this may be taken as a therapeutic test
 LEAVES NO PERMENANT DAMAGE

21. PANCARDITIS
 IN 50% OF CASES
 MOST SERIOUS CAUSE OF MORBIDITY AND MORTALITY
 MAY BE THE ONLY MANIFESTATION OF ARF
 LEAVES PERMENANT DAMAGE
Rheumatic carditis is pancarditis and endocardium is almost always
involved. Hence without murmur carditis cannot be diagnosed.
MYOCARDITIS:
TACHYCARDIA,ARRHYTHMIAS,A-V BLOCKS, CARDIOMEGALY, CHF
ENDOCARDITIS:
MR,AR,TR,PR (STENOTIC LESIONS ONLY AFTER MONTHS OR YEARS)
With severe cardiac failure and pericardial effusion murmur may not
be audible but in such cases the patient is usually very ill.
PERICARDITIS:DRY OR WITH EFFUSION.NEVER ALONE.

22. Chest radiograph of an 8 year old patient with acute carditis
before treatment

23. Same patient after 4 weeks

24. Two-dimensional
color flow
Doppler image of
the left
ventricular inflow
of a patient with
mitral
regurgitation in
the four-chamber
view (top panel)
and two-
dimensional
parasternal long-
axis view (lower
panel), showing
lack of apposition
of the leaflets of
the mitral valve
during systole
(arrow)

25. Two-dimensional parasternal long-axis view of a patient with mitral
stenosis, showing thickened valve cusps (arrow), with poor leaflet
separation in diastole. Left atrium is enlarged, with a thrombus in the
posterior aspect of it. Aortic valve is also stenotic

26.  UNCOMMON (<10%), but most specific
 SMALL (0.5-2 cm.)
 PAINLESS FIRM DISCRETE AND FREELY
MOBILE
 ON EXTENSOR TENDONS OF JOINTS
 OCCASIONALLY ON SCALP AND SPINE
 The subcutaneous nodules tend to appear after
the first weeks of the disease course and
usually disappear within a week or two.
Subcutaneous
nodules

27. Subcutaneous nodule on the extensor surface of elbow of
a patient with acute RF

28.  Sydenham's chorea most frequently occurs
in children or adolescents between the ages
of 5 to 15.
 Affects females approximately twice as
frequently as males, particularly in the years
around puberty. As a result, some
researchers suggest that sex hormones
(e.g., the female hormone estrogen) may
play some role in the development of the
syndrome.
CHOREA

29.  LONG LATENT PERIOD: 1 to 6 months
 In most patientsacutely
 sudden, aimless, irregular, involuntary, jerky
movements 
 A significant deterioration in handwriting (in school-aged
children)
 Slight or significant difficulties dressing, feeding, and walking
 Slurred, slowed speech (dysarthria)
 disappear with sleep and may increase with stress,
fatigue, excitement, or other factors.
 Bilateral (20% hemichorea)
 emotional or behavioral abnormalities
 spontaneously resolve within approximately 3 to 6 months
 However, in some instances, there may be residual signs of
chorea and behavioral abnormalities, which may wax and
wane over a year or more

30.  RARE (5-10%)
 MACULAR NONPRURITIC RASH WITH A
SERPIGINOUS ERYTHEMATOUS BORDER
SURROUNDING NORMAL LOOKING SKIN
 BEGINS AS RED OR PINK MACULES THAT FADE
CENTRALLY
 ON TRUNK & PROXIMAL EXTREMITIES
 NEVER FACE AND HANDS
 ABOUT 1INCH IN DIAMETER
 This skin rash tends to appear early in the disease
course, may persist or recur when other
symptoms have subsided, and usually only affects
patients with carditis.

31. Erythema marginatum on the trunk, showing erythematous lesions
with pale centers and rounded or serpiginous margins

33. LABORATORY STUDIES
ISOLATION OF STREPT.
(THROAT CULTURES)
 Throat culture render positive
results in approximately 25 % of
children of ARF probably related
to early antibiotic administration.
 -VE(75% OF PTS.)
 FALSE +VE: Positive throat culture
need not indicate infection because
positive throat culture may occur in
carrier state as in many school going
children.

34. STREPTOCOCCAL AB. TESTS
ANTIGEN
EXTRACELLULAR PRODUCT
• SREPTOLYSIN-O
• SREPTOKINASE
• HYALURONIDASE
• DEOXYRIBONUCLEASE -N
• NICOTINAMIDE ADENINE
DINUCLEOTIDASE
• ALL OF THE ABOVE
CELLULAR COMPONENT
• TYPE-SPECIFIC M PROTEIN
• GROUP-SPECIFIC POLYSACCHARIDE
TEST
 ANTI-STREPTOLYSIN-0
 ANTI-STREPTOKINASE
 ANTI-HYALURONIDASE
 ANTI-DNAse B
 ANTI-NADase
 STREPTOZYME
 TYPE-SPECIFIC AB.
 ANTI-A CARBOHYDRATE

35.  positive ASOT occur only in 80 % of
streptococcal throat infection. However
sensitivity may be increased to 95 % if
AHT and anti DN ase B are also tested.

36. OTHER INVESTIGATIONS
CXR
 CARDIC SIZE(CHF,EFFUSION)
 RHC. PNUEMONITIS
ECG
 SINUS TACHCARDIA
 PROLONGED P-R
 ARRHYTHMIAS
 ST-T CHANGES
ECHOCARDIOGRAPHY
 MYOCARDIAL(DILATATION,FAILURE)
 PERICARDIAL(EFFUSION)
 ENDOCARDIAL(VALVULAR AFFECTION)

37. DIFFERENTIAL DIAGNOSIS
POLYARTHRITIS
 JUVENILE RHEUMATOID: usually involves small joints of the
fingers and here the swelling is disproportionate to the symptom
and usually the manifestation takes a longer time to subsides
and residual deformity is common.
 ‘Growing pains’ of children is mistaken for arthritis. But the
symptom is not over the joints, pain is severe at night and the
child is well during the day time.
 SLE
 MIXED COLLAGEN DSE.
 POST-INFECTIOUS REACTIVE
 INFECTIVE
 SERUM SICKNESS

38. D.D. of CARDITIS
 Innocent murmurs: The common mistake is
misinterpreting the innocent basal ejection systolic murmur
or left parasternal systolic murmur (Still’s) as evidence of
carditis since they are misinterpreted for mitral
regurgitation. Still’s murmur is vibratory in quality, usually
late systolic unlike the systolic murmur of carditis which is
usually pansystolic or occupies most of systole. The quality
is also different from Still’s murmur. Isolated ejection
systolic murmurs shall never be taken as evidence of
carditis.
 Tachycardia associated with fever and anxiety may be
misinterpreted as evidence of myocarditis. This can be
avoided if one pays attention to sleeping pulse rate.
 INFECTIVE ENDOCARDITIS
 COLLAGEN DSE.(SLE,KAWASAKI)
 VIRAL MYOCARDITIS/pericarditis

39. 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)

40. STEP I: Primary Prevention of Rheumatic Fever
(Treatment of Streptococcal Tonsillopharyngitis)
Agent Dose Mode Duration
Benzathine penicillin G 600 000 U for patients< IM Once
27 kg (60 lb)
1 200 000 U for patients >27 kg
or
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
(maximum 1 g/d)
Recommendations of American Heart Association

41. Arthritis only Aspirin 75-100
mg/kg/day,give as 4
divided doses for 6
weeks
(Attain a blood level 20-
30 mg/dl)
Carditis Prednisolone 2-2.5
mg/kg/day, give as two
divided doses for 2
weeks
Taper over 2 weeks &
while tapering add
Aspirin 75 mg/kg/day
for 2 weeks.
Continue aspirin alone
100 mg/kg/day for
another 4 weeks
Step II: Anti inflammatory treatment
Clinical condition Drugs

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

43. STEP IV : Secondary Prevention of Rheumatic Fever
(Prevention of Recurrent Attacks)
Agent Dose Mode
Benzathine penicillin G 1 200 000 U every 4 weeks* Intramuscular
or
Penicillin V 250 mg twice daily Oral
or
Sulfadiazine 0.5 g once daily for patients 27 kg (60 lb Oral
1.0 g once daily for patients >27 kg (60 lb)
For individuals allergic to penicillin and sulfadiazine
Erythromycin 250 mg twice daily Oral
*In high-risk situations, administration every 3 weeks is justified and
recommended
Recommendations of American Heart Association

44. Duration of Secondary Rheumatic Fever
Prophylaxis
Category Duration
Rheumatic fever with carditis and At least 10 y since last
residual heart disease episode and at least until
(persistent valvar disease*) age 40 y, sometimes lifelong
prophylaxis
Rheumatic fever with carditis 10 y or well into adulthood,
but no residual heart disease whichever is longer
(no valvar disease*)
Rheumatic fever without carditis 5 y or until age 21 y,
whichever is longer
*Clinical or echocardiographic evidence.
Recommendations of American Heart Association

45. For those who receive salicylate
therapy, blood levels and liver
function must be regularly
monitored (i.e., with blood and
urine tests) to help reduce the
possibility of salicylate toxicity, a
condition that may be
characterized by headache, rapid
breathing
(tachypnea), vomiting, irritability,
reduced levels of sugar in the
blood (hypoglycemia), and/or
other findings.

46. SYDENHAM’S CHOREA
 PHYSICAL & MENTAL REST
 As Sydenham's chorea may spontaneously
resolve or not cause significant functional
impairment, many experts indicate that
treatment should be avoided unless
associated chorea is functionally disabling
or associated with potentially violent
flailing motions of the limbs that may
result in self-injury.

47.  First-line therapy with anticonvulsant
medication: valproate sodium
(Depakene®) may be beneficial
 Carbamazepine has also been suggested
as a first-line treatment for Sydenham’s
chorea.

48.  Dopamine antagonists are usually reserved for
those patients who fail to respond to valproate or
who present with severe forms (i.e., chorea
paralytica).
 Haloperidol (initial dose of 0.5 to 1mg/kg/day,
maximum, 5mg/day)
 If fails, the next steps may include
immunomodulatory treatment, steroids, IV IgG,
or plasmapheresis.
 Treatment is usually maintained for 8-12
weeks.

49.  ARF IS THE MOST COMMON CAUSE OF
ACQUIRED HEART DISEASE IN CHILDREN
AND YOUNG ADULTS.
 DIAGNOSIS OF ARF SHOULD DEPEND ON
CLINICAL,LABORATORY & IMAGING
INVESTIGATIONS.
 TREATMENT OF CARDITIS WITH
SALICYLATES , STEROIDS.
 LONG-TERM PROPHYLAXIS WITH LONG
ACTING PCN. IS HIGHLY RECOMMENDED.

50. 0102229098
Basem_enany@yahoo.com