Rheumatic fever is an autoimmune disease that can occur as a result of a streptococcal throat infection. It involves the cross-reaction of antibodies produced against the streptococcal infection with human tissues, causing inflammation of the heart, joints, brain and skin. While the throat infection precedes the onset of rheumatic fever in most cases, not all individuals who experience a streptococcal throat infection will develop rheumatic fever, due to differences in the virulence of the streptococcal strain as well as an individual's immune response. Long-term antibiotic prophylaxis is important for preventing recurrent bouts of rheumatic fever and further heart damage.

1. D R K A M R A N A F Z A L , F C P S , P H D
RHEUMATIC FEVER
RHEUMATIC HEART DISEASE

2. RHEUMATIC FEVER

3. INTRODUCTION
• RF is an immunologically mediated inflammatory
disorder, which occurs as a sequel to group A
streptococcal (GAS) pharyngeal Infection
• 2/3rd of the patients with an acute episode of RF have a history of
an URTI a few weeks before
• Not all of the serotypes of GAS can cause RF
•There is no direct invasion to the tissue by the
microorganism but its an auotoimmune disease that
involves Ag-Ab interaction
•It involves GAS throat infection
•It must be throat infection and not skin infection

4. • Multisystem disease affecting the heart, joints, brain,
cutaneous and subcutaneous tissues
•RF can cause permanent damage to the heart but not to
the joints
• Major public health problem in heavily populated
underdeveloped and developing countries
• Preventable disease

5. INCIDENCE
• 3% in epidemics of exudative streptococcal pharyngitis in
closed community(school, army)
• 0.3% in civilian population with sporadic streptococcal
throat infection
• 50% if there is a past history of RF (secondary
prophylaxis is important)
• First attack between 5-15 years(a childhood disease)
• Poor socioeconomic conditions and overcrowding

6. PATHOGENESIS
• Antibodies must be produced by the body rapidly and in
high magnitude
• These Abs will cross react with tissue of the heart, joint,
brain (especially basal ganglia), skin

7. • Because of the similarity between 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 cause 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

8. • The cytotoxicity theory suggests that a GAS toxin may be
involved in the pathogenesis of acute RF and RHD
• GAS produces several enzymes that are cytotoxic for
mammalian cardiac cells, for example, Streptolysin O has
a direct cytotoxic effect on mammalian cells in tissue
culture

9. CLINICAL MANIFESTATIONS
AND DIAGNOSIS
• Because no clinical or laboratory finding is
pathognomonic for acute RF, Duckett Jones in 1944
proposed guidelines to aid in diagnosis
• The Jones criteria, as revised in 1992 by the AHA, are
intended only for the diagnosis of the initial attack of acute
RF and not for recurrences

13. CLINICAL MANIFESTATIONS

14. LAB DIAGNOSIS
Supporting evidence for antecedent GAS infection
•Positive throat culture
(In 25% of patients and 75% may be negative)
•Rapid streptococcal antigen test
•Elevated or rising streptococcal antibody titer –
ASO[anti-streptolysin]
(others: Anti-DNAse B, AH[anti-hyoluronic acid])
• 95-100% have an elevation in these three different Abs

15. PRIMARY PREVENTION OF RF
Treatment of Streptococcal Tonsillopharyngitis

16. TREATMENT
• All patients with acute RF should be placed on bed rest
and monitored closely for evidence of carditis
• They can be allowed to ambulate as soon as the signs of
acute inflammation have subsided, however, patients with
carditis require longer periods of bed rest

17. ANTIBIOTICS
• Patient should receive 10 days of oral penicillin or
erythromycin, or a single IM injection of benzathine
penicillin to eradicate the GAS
• Anti-inflammatory therapy with salicylates, and bed rest
• Additional supportive therapy for heart failure or chorea
may be necessary
• Long term penicillin prophylaxis, preferably with IM
benzathine penicillin G, 1.2 million IU every 28 days, is
required
• Oral regimens for prophylaxis generally are not as
effective

18. DIFFERENTIAL DIAGNOSIS OF ARF

19. RHEUMATIC HEART DISEASE

20. • Rheumatic involvement of the valves and endocardium is
the most important manifestation of RF
• The valvular lesions begin as small verrucae composed
of fibrin and blood cells along the borders of one or more of
the heart valves
• The mitral valve is affected most often, followed in
frequency by the aortic valve; right-sided heart
manifestations are rare

21. • As the inflammation subsides, the verrucae tend to
disappear and leave scar tissue, with repeated attacks of
RF, new verrucae form near the previous ones, and the
mural endocardium and chordae tendinae become
involved

22. PATTERNS OF VALVULAR DISEASE
1. Mitral Insufficiency
• There is loss of valvular substance and shortening and
thickening of the chordae tendineae
• During ARF with severe cardiac involvement, heart
failure is caused by a combination of mitral insufficiency
coupled with inflammatory disease of the pericardium,
myocardium and endocardium
• Because of the high volume load and inflammatory
process, the left ventricle becomes enlarged, the left
atrium dilates as blood regurgitates into this chamber,
increased left atrial pressure results in pulmonary
congestion and symptoms of left-sided heart failure

23. 2. Mitral Stenosis
• Mitral stenosis of rheumatic origin results from fibrosis of
the mitral ring, commissural adhesions, and contracture of
the valve leaflets, chordae, and papillary muscles over
time
• It takes 10 yr or more for the lesion to become fully
established, although the process may occasionally be
accelerated

24. • Significant mitral stenosis results in increased pressure
and enlargement and hypertrophy of the left atrium,
pulmonary venous hypertension, increased pulmonary
vascular resistance, and pulmonary hypertension
• Right ventricular and atrial dilatation and hypertrophy
ensue and are followed by right-sided heart failure

26. 3. Aortic Insufficiency
• In chronic rheumatic aortic insufficiency, sclerosis of the
aortic valve results in distortion and retraction of the cusps
• Regurgitation of blood leads to volume overload with
dilatation and hypertrophy of the left ventricle
• Combined mitral and aortic insufficiency is more common
than aortic involvement alone

27. 4. Tricuspid Valve Disease
• Primary tricuspid involvement is rare after RF
• Tricuspid insufficiency is more common secondary to
right ventricular dilatation resulting from unrepaired left-
sided lesions
• The signs produced by tricuspid insufficiency include
prominent pulsations of the jugular veins, systolic
pulsations of the liver, and a blowing holosystolic murmur
at the lower left sternal border that increases in intensity
during inspiration

28. 5. Pulmonary Valve Disease
• Pulmonary insufficiency usually occurs on a functional
basis secondary to pulmonary hypertension and is a late
finding with severe mitral stenosis
• The murmur (Graham Steell murmur) is similar to that of
aortic insufficiency, but peripheral arterial signs (bounding
pulses) are absent
• The correct diagnosis is confirmed by 2-D echo and
Doppler studies

29. Q. Why not all patients that have GAS throat infection will
have RF?
A. Because there are microorganism as well as host
variables:
•Microorganism variables: Only certain strains that can
produce the immunologically active Ag
•Host variables: Some of us will produce large amount of
Abs after each infection but others don’t