A lecture on rheumatic fever prevention. It goes over the important points

1. Preventing Acute Rheumatic Fever and Rheumatic Heart
Disease
Dr Mutsa Bwakura
UZ
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2. Learning Objectives
• Appreciate the burden of disease
• Revise the pathogenesis of acute rheumatic
fever and rheumatic heart disease
• Recognize features of a streptococcal sore
throat
• Know the treatment regimens for a
streptococcal sore throat
• Know the prevention of acute rheumatic fever
and rheumatic heart disease
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4. • RHD affects 35 million
people worldwide and
causes about 350,000
deaths each year.*
• Africa has very high rates
of RHD.
• High risk factors for RHD
include: overcrowded
living conditions, lack of
clean water and toilets,
and poor access to
healthcare
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RHD is a global problem
*Reference: c
On the map, countries in darker color have
more patients with RHD

5. • In Africa, up to 1-3% of
young people have signs
of early RHD.
• RHD mostly affects
children and young adults.
• Once it starts, RHD is hard
to treat. This is why it is
important to prevent RHD.
RHD is common in Sub-Saharan Africa
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6. 6
Untreated bacterial sore throat can lead to
acute rheumatic fever and then to RHD.
RHD
Bacterial
sore
throat
Acute
rheumatic
fever
RHD is caused by bacterial sore throat
ARF is the systemic non-infectious sequel to the often self-limiting
pharyngitis caused by rheumatogenic strains of
GAS

7. Signs of viral
sore throat:
Runny nose
Cough
Itchy, watery eyes
Fever (nonspecific)
Sick family members
Signs of bacterial
(strep) sore throat:
Red throat
White patches on tonsils
Tender, swollen glands
Scarlet fever rash
Abdominal pain
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Comparing bacterial and viral sore throat
Give penicillin only for bacterial sore throat
Treatment with
penicillin is needed
No antibiotic
treatment needed

8. Figure 2 Generation of a cross-reactive immune response in ARF
Carapetis, J. R. et al. (2015) Acute rheumatic fever and rheumatic heart disease
Nat. Rev. Dis. Primers doi:10.1038/nrdp.2015.84

9. Diagnosis of ARF
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10. 10

11. Rheumatic Heart Disease
• 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 develop RHD.
• Commonest valves affected are the mitral and
aortic, in that order.
– However all four valves can be affected.
• RHD can go undetected with the result that
patients present with debilitating heart failure.
– At this stage surgery is the only possible treatment
option.
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12. How to prevent further infections and heart damage
Give a penicillin injection or tablets
IM Injection – One dose
Benzathine Penicillin *1
<30 kg: 600,000 units
>30 kg: 1.2 million units
* No test dose is needed.
*The patient does not need to take extra food
before the injection.
*Patients do not develop resistance to
Benzathine Penicillin
Oral dose Penicillin V –
Duration: 10 days 1
<27kg: 250mg 2-3 times per day
>27kg: 500mg 2-3 times day
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1Reference: Prevention of Rheumatic Fever and Diagnosis and Treatment of Acute Streptococcal
Pharyngitis
Michael A. Gerber, Robert S. Baltimore, Charles B. Eaton, Michael Gewitz, Anne H. Rowley, Stanford T.
Shulman and Kathryn A. Taubert
Circulation. 2009;119:1541-1551, originally published March 23, 2009
https://doi.org/10.1161/CIRCULATIONAHA.109.191959

13. In the event of penicillin allergy
Give alternate medications
1Reference: Mayosi, “Protocols for antibiotic
use in primary and secondary prevention for
rheumatic fever”, SAMJ 2006
Agent Dose Mode
Erythromycin1 >30 kg: 500mg b.d. or 250 mg
q.i.d.
<30 kg: 125mg q.i.d.
Oral
Clindamycin2 20 mg/kg per day divided in 3
doses (1.8 g/d)
Oral for a duration of 10
days
Azithromycin2 12 mg/kg once daily (maximum
500 mg)
Oral for a duration of 5 days
Clarithromycin2 15 mg/kg per day divided BID
(maximum 250 mg BID)
Oral for a duration of 10
days
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2Reference: Prevention of Rheumatic Fever and Diagnosis and Treatment of Acute Streptococcal Pharyngitis
Michael A. Gerber, Robert S. Baltimore, Charles B. Eaton, Michael Gewitz, Anne H. Rowley, Stanford T. Shulman and Kathryn
A. Taubert
Circulation. 2009;119:1541-1551, originally published March 23, 2009
https://doi.org/10.1161/CIRCULATIONAHA.109.191959

14. What brought down the incidence of
ARF?
• Progressive decline in the occurrence and
severity of acute RF and RHD, with a marked
decrease in the prevalence of RHD in school
children.
• A marked and progressive decline was also
seen in the incidence and severity of ARF.
• Early detection and treatment of sore throats and
streptococcal pharyngitis.
• Primary and secondary prevention of RF/RHD,
training of personnel, health education,
dissemination of information, community
involvement and epidemiological surveillance.
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15. RHEUMATIC FEVER IS PREVENTABLE
Costa Rica
Cuba

16. For a patient who has RHD
Give secondary prevention as long as needed
according to WHO guidelines
Category of patient Duration of secondary prevention
Patient without proven
carditis
For 5 years after last attack, or until 18 years
of age (whichever is longer)
Patient with mild carditis* For 10 years after the last attack, or at least
until 25 years of age (whichever is longer)
More severe valvular
disease
Lifelong
After valve surgery Lifelong
*Carditis is inflammation of muscle tissue in the heart. Mild carditis in an RHD patient
is described as mitral valve regurgitation or healed carditis.
Reference: Mayosi, “Protocols for antibiotic use in primary and secondary prevention for rheumatic fever”, SAMJ 2006.
http://www.samj.org.za/index.php/samj/article/viewFile/1389/813
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17. 17
RHD pathway
• Origin: RHD is a complication of untreated
streptococcal (“strep”) sore throat.
• Primordial prevention: Improved living conditions
(i.e., reduced poverty, nutrition, overcrowding)
decreases risk of strep sore throat.
• Primary prevention: Prompt treatment of patients
with sore throat prevents acute rheumatic fever and
RHD. Single-dose injectable benzathine penicillin is
preferred to ensure compliance.
• Secondary prevention: Monthly penicillin injections
for a period of years or decades reduces progression
of heart disease in patients with RHD.
• Tertiary intervention: Special heart medications and
surgery are sometimes needed for patients with
advanced RHD. Left untreated, progressive RHD is
often fatal.
Environmental
conditions
Group A
Streptococcal
pharyngitis
Acute rheumatic
fever/RHD
Cardiac surgery
Primordial
prevention
Primary
prevention
Secondary
prevention
Tertiary
intervention
Pathway to the development of RHD
and intervention nodes for prevention

18. Prevention of RHD
Type of prevention Aim (what) Modality (how)
Primordial prevention
Eliminate factors
conducive for infection
through developmental
measures at the
population level
Socio-economic
development: avoidance
of overcrowding,
improved hygiene.
Primary prevention
Eliminate infection early
to prevent the first
episode of ARF
Prompt treatment of all
sore throat by penicillin in
endemic areas
Secondary prevention
Eliminate recurrence of acute
rheumatic fever to prevent
cumulative damage leading to
rheumatic heart disease
Regular antibiotic prophylaxis
(monthly injection of long-acting
penicillin), sometimes lifelong,
after a first episode of acute
rheumatic fever or diagnosed
rheumatic heart disease
Prevention of Acute Rheumatic Fever and Rheumatic Heart Disease
Mariana Mirabel, Kumar Narayanan, Xavier Jouve and Eloi Marijon
Circulation. 2014;130:e35–e37

19. Prevention of ARF and RHD

20. Early detection by
hand-held ultrasound
screening and
antibiotic prophylaxis
have the potential to
successfully eliminate
the scourge of
rheumatic heart
disease (RHD) from
the developing world.
Reproduced with
permission from
General Electric
Company.
In a low-income Caribbean Island, Santa Lucia, success was achieved by
reorienting part of the budget allocated to cardiac surgery to an acute rheumatic
fever (ARF)/rheumatic heart disease (RHD) control program including primary and
secondary prophylaxis. Adapted from Marijon et al2 with permission of the
publisher. Copyright ©2012, Elsevier

21. In Summary
• Up to 30% of sore throats in children and young people
are caused by group A streptococci (GAS)
• Without antibiotic treatment, some of these children
will develop rheumatic fever (RF) (0.3-3%)
• Repeated episodes of GAS infection and RF cause
progressive heart valve damage (RHD)
• A triad of environmental, genetic and bacterial factors
appear to be important in the development of clinically
significant disease.
• ARF can be prevented
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23. Acknowledgements
• Slides from
– Dr John Masuku (UTH- Zambia)
– Dr Liesl Zuhlke Cape Town
• WHF
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