1. Rheumatic fever is a post-streptococcal disease that follows infection by group A beta hemolytic streptococci, most commonly affecting children ages 5-15. 2. It is characterized by inflammation of the heart (carditis), joints (arthritis), brain (chorea), and skin (erythema marginatum). 3. Diagnosis is based on the modified Jones criteria and treatment involves antibiotics to treat the initial strep infection, anti-inflammatory drugs like aspirin for symptoms, and long-term antibiotic prophylaxis to prevent recurrence, especially important for those who develop carditis and residual heart damage.

2. Rheumatic fever
(French physician Ernst-Charles Lasègue 1884)
“It licks the joints, but bites the heart”.
Dr Zain Ul Abidin
Bahawal Victoria Hospital
Bahawalpur
xe.zain@gmail.com

3. Scheme of Presentation.
•Aetiology
•Epidemiology
•Pathogenesis
•Pathologic lesions
•Clinical manifestations & Laboratory
findings
•Diagnosis & Differential diagnosis
•Treatment & Prevention
•Prognosis

4. Aetiology.
Acute rheumatic fever is a systemic disease of
childhood, often recurrent, that follows infection by group
A beta hemolytic streptococci(GABH).

5. It is a diffuse inflammatory disease of connective tissue,
primarily involving heart, blood vessels, joints,
subcutaneous tissue and CNS.
Epidemiology.
Ages 5-15 yrs are most susceptible. Rare under 3 years.
Girls>boys.
Common in 3rd world countries
Environmental factors: over crowding, poor sanitation,
poverty.
Incidence more during fall, winter & early spring.

7. Pathogenesis
Delayed immune response to infection with group A
beta hemolytic streptococci.
After a latent period of 1-3 weeks, antibody induced
immunologically-mediated inflammatory response occurs.

8. Pharyngitis produced by GABHS can lead to acute
rheumatic fever, rheumatic heart disease & post-
streptococcal Glomerulonephritis.

9. Pathologic Lesions
Fibrinoid degeneration of connective tissue, inflammatory
edema, inflammatory cell infiltration & proliferation of
specific cells resulting in:
Pancarditis in the heart with formation of Ashcoff
nodules which are granulomatous structures
consisting of fibrinoid change, lymphocytic
infiltration, plasma cells, and characteristically
abnormal macrophages surrounding necrotic
centres.
Arthritis in the joints
Nodules in the subcutaneous tissue
Basal ganglia lesions resulting in chorea

10. Clinical Features
Jones Criteria (Revised) for Guidance in the Diagnosis of Rheumatic Fever*
Major
Manifestation
Minor
Manifestations
Supporting Evidence
of Streptococcal Infection
Carditis
Polyarthritis
Chorea
Erythema
Marginatum
Subcutaneous
Nodules
Clinical Laboratory
Increased Titer of Anti-
Streptococcal Antibodies
ASO (anti-streptolysin O),
Others
Positive Throat Culture
for Group A Streptococcus
Recent Scarlet Fever
Previous
rheumatic
fever or
rheumatic
heart
disease
Arthralgia
Fever
Acute phase
reactants:
Erythrocyte
sedimentation
rate,
C-reactive
protein,
leukocytosis
Prolonged P-R
interval
*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 American Heart Association

11. 1. Arthritis
Flitting & fleeting migratory polyarthritis, involving
major joints
Commonly involved joints: knee, ankle, elbow &
wrist.
Occur in 75%,involved joints are exquisitely tender.
In children below 5 yrs arthritis is usually mild but
carditis is more prominent.
Arthritis does not progress to chronic disease.
2. Carditis
Manifests as pancarditis (endocarditis, myocarditis
and pericarditis),occurs in 40-50% of cases (90% in
age 3 yrs & 30% in adolescents)
Carditis is the only manifestation of rheumatic fever
that leaves a sequelae & permanent damage to the
organ.
Valvulitis occurs in acute phase
Chronic phase: fibrosis, calcification & stenosis of
heart valves (fishmouth valves)

12. CARDITIS (continued)
SIGN & SYMPTOMS:
Breathlessness, palpitation and tachycardia, chest pain,
cardiac enlargement, new and changed cardiac murmurs.
Soft systolic murmur due to MITRAL regurgitation is
very common.
Soft mid-diastolic murmur (called the CAREY COOMB’S
MURMUR, due to valvulitis with nodule formation on the
valve leaflets, is characteristic).
Aortic valve incompetence is present in 50% of cases.
Pulmonary and tricuspid valves are rarely involved.

13. Rheumatic heart disease. Abnormal
mitral valve. Thick, fused chordae
Diseased, incompetent mitral valve with
vegetations on the cusps
Aschoff bodies are microscopic
structures seen in patients
with rheumatic fever

14. 3.Sydenham’s Chorea
Occurs in 5-10% of cases
Mainly in girls of 1-15 yrs age
May appear even 6 months after the attack of ARF.
Clinically manifests as: clumsiness, deterioration of
handwriting, emotional lability or grimacing of face
Clinical signs: pronator sign, milking sign of hands.

15. 4. Erythema Marginatum
Occurs in <5%.
Unique,transient,serpiginous-looking lesions of 1-2
inches in size
Pale center with red irregular margin
More on trunks & limbs & non-itchy
Worsens with application of heat
Often associated with chronic carditis

16. 5. Subcutaneous nodules
Occur in 10%
Painless, pea-sized, palpable nodules
Mainly over extensor surfaces of joints, spine,
scapulae & scalp
Associated with strong seropositivity
Always associated with severe carditis
6. Other features (Minor features)
Fever: (upto 101 degree F)
Arthralgia
Pallor
Anorexia
Loss of weight

17. Laboratory Findings
High ESR
Anemia, leucocytosis
Elevated C-reactive protien
ASO titre >200 Todd units (peak value attained at 3
weeks then comes down to normal by 6 weeks)
Anti-DNAse B test
Throat culture: GABHStreptococci
 X-RAY CHEST: cardiomegaly and chest congestion
ECG: prolonged PR interval, 2nd or 3rd degree
blocks, ST depression,T inversion
Echo-cardiography: valve edema, mitral
regurgitation, LA & LV dilatation, pericardial effusion,
decreased contractility

18. The blood-agar culture plates show a positive streptococcus infection
with the bacteria arranged in chains and the halo effect caused by
haemolysis shows specifically a beta-hemolytic group A bacteria.
Laboratory diagnosis of rheumatic fever (contd/-)

19. Diagnosis
Rheumatic fever is mainly a clinical diagnosis
No single diagnostic sign or specific laboratory test
available for diagnosis
Diagnosis based on MODIFIED JONES CRITERIA.

20. Differential Diagnosis
Juvenile rheumatiod arthritis
Septic arthritis
Sickle-cell arthropathy
Kawasaki disease
Myocarditis
Scarlet fever
Leukemia

21. 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)

22. STEP I: Primary Prevention of Rheumatic Fever.
(Treatment of Streptococcal Tonsillo-pharyngitis)
Agent Dose Mode Duration
Benzathine penicillin G 600 000 U for patients Intramuscular 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 estolate 20-40 mg/kg/d 2-4 times daily Oral 10
(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

23. Step II: Anti inflammatory treatment
Clinical condition Drugs
Arthritis only Aspirin 60-100
mg/kg/day,give as 4
divided doses for 6
weeks
(Attain a blood level 20-
30 mg/dl)
Carditis Prednisolone 1.0-2.0
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

24. 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

25. 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

26. 26
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 valvular 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 valvular disease*)
Rheumatic fever without carditis 5 y or until age 21 y,
whichever is longer
*Clinical or echocardiographic evidence.
Recommendations of American Heart Association

27. Prognosis
Rheumatic fever can recur whenever the individual
experiences 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 with
carditis with valvular lesions

28. To summarise: salient features of ARF: