Rheumatic Fever (Basics & Updates) BY Dr. Al Hussein Ragab Zaky Luxor International Hospital,EGYPT Tel: 00201113033672-00201012727282 Facebook : Al Hussein Ragab

1. Rheumatic Fever
(Basics & Updates)
BY
DR. AL HUSSEIN RAGAB ZAKY
LUXOR INTERNATIONAL HOSPITAL
TEL: 01113033672-01012727282
FACEBOOK : AL HUSSEIN RAGAB

3. Definition
Rheumatic fever is an auto immune inflammatory
disease following URTs with group A Beta hemolytic
streptococci, involving mainly the heart, the joints and
less frequently the CNS, the skin and subcutaneous
tissue.
It is always potentially serious as it may lead to permanent
cardiac damage (chronic valvular disease).

4. Epidemiology
 Rheumatic fever occurs at all ages except infancy but
incidence peaks between 5 - 15 years (when GAS
infections are most frequent).
 It is still the leading cause of acquired heart disease in
children in developing countries.
 Chorea is more common in female.

5. Risk populations
Low-Risk populations:
 Those with incidence ≤2 per 100,000 school-age children per year or all age
rheumatic heart disease prevalence of ≤1 per thousand population.
 Include virtually all of the United States, Canada, and Western Europe.
High-Risk populations:
 Those with incidence >2 per 100,000 school-age children per year or allage
rheumatic heart disease prevalence of >1 per thousand population.
 Include Maoris in New Zealand, aborigines in Australia, Pacific Islanders,and most
developing countries.

6. Evidence of a preceding GAS
infection
 1- Positive throat culture for streptococci.
Or rapid antigen test.
 2 - Elevated and/or rising ASO titer.
 3- Raised other streptococcal antibodies:
a) Antistreptokinase
b) Antihyalurindase
c) Antideoxyribonculease B.

7. Clinical manifestations and diagnosis
2015 Revised Jones Criteria for diagnosis of Rheumatic Fever

9. Rheumatic Arthritis
 Occurs in 75% of patients with acute rheumatic fever.
 There is often an inverse relationship between the severity of arthritis
and the severity of cardiac involvement.
 Arthritis occur in large joints and does not result in chronic joint disease.
 Following the initiation of anti-inflammatory therapy (salicylates),
arthritis may disappear in 12-24 hours (dramatic response), If untreated,
it may persist for a 1-2 weeks.
 Arthralgia (joint pain) may occur in some joints and frank arthritis in
others.
 Arthritis means (pain ,hotness, redness, swelling and limitation of
movements)

10. Differential diagnosis
Causes of arthritis such as:
 Juvenile rheumatoid arthritis & other collagen diseases.
 Infective endocarditis.
 Septic arthritis.
 TB arthritis.
 Malignancy as Leukemia.
 Sickle cell disease.
 Gonococcal infection.
 Reactive arthritis (Salmonella & Shigella).

11. Treatment of Arthritis
(Anti inflammatory)
• Salicylates provide dramatic relief.
 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 wk
 and half that dose (25 mg/kg/day) for another 2-4 wk
• Early administration of salicylates to a patient before diagnosis is
established may obscure the diagnosis.

12. Rheumatic Carditis
Rheumatic Subclinical Carditis
 Defined as carditis without a murmur of valvulitis but with
echocardiographic evidence of valvulitis.
 The echocardiographic features of subclinical carditis must
meet those from physiologic degrees of valve regurgitation.
included in the following tables in order to distinguish
pathologic

13. PATHOLOGIC MITRAL REGURGITATION (ALL 4 MET)
 1. Seen in at least 2 views
 2. Jet length ≥2 cm in at least 1 view
 3. Peak velocity >3 meters/second
 4. Pan-systolic jet in at least 1 envelope
PATHOLOGIC AORTIC REGURGITATION (ALL 4 MET)
 1. Seen in at least 2 views
 2. Jet length ≥1 cm in at least 1 view
 3. Peak velocity >3 meters/second
 4. Pan-diastolic jet in at least 1 envelope

14. Clinical Carditis
 Occurs in about 50-60% of all cases of acute
rheumatic fever (most serious).
 Pancarditis that involves the endocardium,
myocardium and pericardium.
 Involvement of the endocardium is a must for
rheumatic carditis.

15. A- Endocarditis
 1- Pansystolic murmur at the apex:
Caused by mitral valvitis. Blowing in character high pitched, soft and may be
musical, localized over the apex, not associated with thrill, disappears within 6 months
if not associated with chronic organic mitral regurge.
 2- A low-pitched middiastolic murmur at the apex (Carey Coombs
murmur).
 3- Early diastolic murmur over the aortic area.
 4- Appearance of new murmurs.
 5- Change in character of existing murmurs.

16. B- Myocarditis:
 • Tachycardia
 • Arrhythmias
 • The first heart sound may be muffled.
 • Cardiac dilatation and heart failure.
 ECG: may reveal low voltage, prolonged P-R and Q-
T intervals, ST-T changes and arrhythmia.

17. C- Pericarditis
 Dry pericarditis: precordial pain and friction rub.
 Pericarditis with mild to moderate effusion.
 Pericarditis with massive pericardial effusion:
• Weak pulse.
• Pulsus paradoxus: Decreased pulse volume during inspiration caused by
exaggeration of the normal phenomenon of slight decrease in systolic
blood pressure during inspiration.
• Congested non pulsating neck veins.
• Apex beat is weak or impalpable.
• Dullness outside the apex by percussion.
• Heart sounds are distant and muffled.
• Ewart’s sign: compression of the left lung produces dullness and
bronchial breathing at the base posteriorly.

18. Differential diagnosis:
 Causes of carditis such as viral myocarditis.
 Causes of pericarditis.
 Infective endocarditis.
 Mitral valve prolapse.
 Congenital heart disease.
 Kawasaki disease.

19. Treatment of carditis:
Mild cases without evidence of congestive heart
failure:
• Salicylates (antinflammatory) alone are indicated in
 -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 wk
 -and half that dose (25-35 mg/kg/day) for another 2-4 wk

20. Moderate and severe cases associated with
cardiomegaly &/or heart failure:
Prednisone (ant inflammatory)
 2 mg/kg/day in 4 divided doses for 2-3 wk
 Followed by half the dose (1 mg/kg/day) for 2-3 wk
 Then [When the patient responds clinically & on lab tests (ESR, CRP)]
tapering of the dose by 5 mg/24 hr every 2-3 days.
 At the beginning of tapering steroid dose, Salicylates (50 mg/kg/day) in 4
divided doses for 6 wk to prevent rebound of inflammation.
 Supportive therapies include digoxin, fluid & salt restriction, diuretics, and O2.
NB: The cardiac toxicity of digoxin is enhanced with myocarditis.

21. Rheumatic Chorea (Sydenham, St.
Vitus Dance Chorea)
 Occurs in about 10-15% of patients with acute rheumatic fever.
 Involuntary, static ,semipurpouseful, jerky, sudden,irregular.
 Involved mainly face, trunk,limbs.aggravated by emotional stress.
 Usually presents as an isolated, frequently subtle, neurologic
behaviordisorder (pure chorea).
 Has long latent period (2-6 months) so it may be considered the
only manifestation of rhuematic fever.

22. Emotional disorders:
 There is alteration of temperament with characteristic lability of emotions.
 The child laughs and cries without apparent reason.
Muscular hypotonia:
 Darting tongue
 Mik-maid grip
 Pendular knee reflex
 Boat-shaped hands
 Pronator sign
 Piano-player sign

23. Differential diagnosis:
 Degenerative (Huntington’s chorea). It has a progressive course and the age
incidence ranged from 30-50 years.
 Postencephalitic chorea: there is a past history of encephalitis and is regressive in
course.
 Collagen: SLE.
 Wilson disease.
 In addition ; drugs, cerebral palsy, tics and hyperactivity should be excluded.
Prognosis:
 Chorea is a self limited condition.
 Mild cases subside within few weeks but a course of 3 months is average.
 The disease may progress to become so severe as to require a padded cot.

24. Treatment of Rheumatic Chorea:
 Phenobarbital (16-32 mg every 6-8 hr PO) is the drug of
choice. If ineffective,Haloperidol (0.01-0.03 mg/kg/24 hr
divided bid PO)
 Or Chlorpromazine (0.5 mg/kg every 4-6 hr PO) should be
initiated.
 Some patients may benefit from a few week course of
corticosteroids

25. Erythema marginatum:
 • It occurs occasionally in rheumatic fever patients (<3%).
 • The lesion begins as red, raised non pruritic macules then
extend outward to form wavy lines or rings with pale centers.
 • Coalesce forming irregular circinate patterns, which vary in
shape and site from hour to hour.
 • Changing in pattern and evanescent .
 • Usually seen over the trunk and spares the face.

26. Subcutaneous nodules:
 • Bilaterally symmetrical lesions comprising firm nodules
varying in diameter from a few millimeters to a centimeter.
 • Movable painless and not tender.
 • Occur mainly over the bony prominences.
 • Best demonstrated by fully flexing the joint and stretching the
skin over the extensor surface.
 • When occurs (≤1% of patients with acute rheumatic fever),
usually severe carditis is present.

27. Associated findings may be noted in
patients with ARF.
 • Abdominal pain
 • Rapid sleeping pulse rate
 • Tachycardia out of proportion to fever
 • Malaise
 • Anemia
 • Leukocytosis
 • Epistaxis
 • Precordial pain

28. Complications:
 Chronic valvular heart disease after an attack of rheumatic
carditis.
 Severe acute carditis is the commonest cause of death of
rheumatic fever.

29. Treatment of acute rheumatic fever:
can be divided into three approaches
 1. Treatment of GAS infection.
 2. Bed Rest
 3. Use of anti-inflammatory agents.
 4. Therapy for complications, such as heart
failure.

31. Prevention
Primary prophylaxis :
 Treatment of streptococcal upper respiratory tract infection (within 9
days) to prevent an initial attack of rheumatic fever.