Rheumatic Fever and
Rheumatic heart disease
Dr. Muhammad Imran
6/2/2015 1
Learning Outcomes
After attending this presentation the audience
will be able to:
• Define Rheumatic fever
• Describe the Pathogenesis of Rheumatic
fever(RF)
• Illustrate the Clinical features of RF
• Write the diagnosis of Rheumatic fever
• Devise the prevention of RF
6/2/2015 2
Rheumatic Fever
• Rheumatic fever (RF) is an acute,
immunologically mediated,
multisystem inflammatory disease
that occurs a few weeks following an
episode of group A streptococcal
pharyngitis
• Major involvement of systemic
connective tissue; heart, joints, skin,
and subcutaneous and vascular
connective tissue
Oxford Handbook of Cardiology. 2nd edition. Page:146-1506/2/2015 3
Epidemiology
• <1:1000 in developed countries; 10:1000
schoolchildren in developing countries
• It is rarer, but still accounts for half of cardiac
disease in the developing world
• Typically affects children aged 5–15 years
from lower socio-economic
• No sex difference but mitral stenosis are
more common in females
Oxford Handbook of Cardiology. 2nd edition. Page:146-1506/2/2015 4
Pathology
• Typically occurs several weeks after
a streptococcal pharyngitis
• Usually group A beta haemolytic
streptococci: Streptococcus
pyogenes serotype M. Antigenic
mimicry is implicated — antibodies
to carbohydrate in cell wall (anti-M
antibodies) of group A
Streptococcus cross-react with
protein in cardiac valves
Oxford Handbook of Cardiology. 2nd edition. Page:146-1506/2/2015 5
Pathology (Continue)
• Delay from acute infection to
onset of rheumatic fever is
usually 3–4 weeks
• RF is thought to complicate up to
3 % of untreated streptococcal
sore throats
• Commonly causes a pancarditis
Oxford Handbook of Cardiology. 2nd edition. Page:146-1506/2/2015 6
Pathogenesis
6/2/2015 7
Clinical features
• Sore throat 1–5 weeks earlier is
reported in two-thirds of cases
• Fever, abdominal pain, and epistaxis
• Migratory large-joint polyarthritis
starting in the lower limbs in 75 % of
cases
• Pancarditis in 50 % of cases with
features of acute heart failure, mitral
and aortic regurgitation, an apical,
and pericarditis
• Chorea in 10–30 % , usually 1–6
months after the index pharyngitis
6/2/2015 8Oxford Handbook of Cardiology. 2nd edition. Page:146-150
Clinical features (Continue)
• Erythema marginatum is an
evanescent rash and central
clearings on the trunk and
proximal limbs
• Subcutaneous nodules in 0–8 %
of cases several weeks after the
onset of severe pancarditis
6/2/2015 9Oxford Handbook of Cardiology. 2nd edition. Page:146-150
Diagnosis Criteria
Oxford Handbook of Cardiology. 2nd edition. Page:146-1506/2/2015 10
Rheumatic heart disease
• Rheumatic heart disease is a
complication of rheumatic
fever
• Divided into rheumatic
endocarditis, rheumatic
myocarditis and rheumatic
pericarditis, often for
rheumatic pancarditis
• 60% to 80% children are
associated with pancarditis
6/2/2015 11Oxford Handbook of Cardiology. 2nd edition. Page:146-150
6/2/2015 12
Key morphologic
features of acute
rheumatic heart
disease
Prevention
• The only known way to
prevent rheumatic fever
is to treat strep. throat
infections promptly with
a full course of
appropriate antibiotics
6/2/2015 13Oxford Handbook of Cardiology. 2nd edition. Page:146-150
Prevention (Continue)
• If you have or suspect you have
rheumatic fever see your doctor or local
health clinic
• If you have had rheumatic fever make
sure you always have your
regular penicillin injections
• Get regular check-ups at your local health
clinic
• Do not ignore a sore throat. Consult your
doctor
6/2/2015 14Oxford Handbook of Cardiology. 2nd edition. Page:146-150
Prevention (Continue)
• Keep sores clean and
covered
• Wash hands regularly
• Watch out for
symptoms in children –
people aged between 5
and 14
• Eat a healthy diet
6/2/2015 15Oxford Handbook of Cardiology. 2nd edition. Page:146-150
Prevention (Continue)
Primary Prevention:
Primary prevention of
rheumatic fever is possible
with a 10-day course of
penicillin therapy for
streptococcal pharyngitis.
However, primary prevention
is not possible in all patients
Park's Pediatric Cardiology for Practitioners. 6th edition. Page: 603-610
6/2/2015 16
Secondary Prevention
Who should receive prophylaxis?
Patients with documented histories
of rheumatic fever, including those
with isolated
chorea and those without evidence
of rheumatic heart disease, must
receive prophylaxis
Park's Pediatric Cardiology for Practitioners. 6th edition. Page: 603-610
6/2/2015 17
Secondary Prevention (Continue)
For how long?
Ideally, patients should receive
prophylaxis indefinitely. For
patients who had acute
rheumatic fever without
carditis, the prophylaxis should
continue for at least 5 years or
until the person is 21 years of
age
Park's Pediatric Cardiology for Practitioners. 6th edition. Page: 603-610
6/2/2015 18
Key message
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Rheumatic Fever

  • 1.
    Rheumatic Fever and Rheumaticheart disease Dr. Muhammad Imran 6/2/2015 1
  • 2.
    Learning Outcomes After attendingthis presentation the audience will be able to: • Define Rheumatic fever • Describe the Pathogenesis of Rheumatic fever(RF) • Illustrate the Clinical features of RF • Write the diagnosis of Rheumatic fever • Devise the prevention of RF 6/2/2015 2
  • 3.
    Rheumatic Fever • Rheumaticfever (RF) is an acute, immunologically mediated, multisystem inflammatory disease that occurs a few weeks following an episode of group A streptococcal pharyngitis • Major involvement of systemic connective tissue; heart, joints, skin, and subcutaneous and vascular connective tissue Oxford Handbook of Cardiology. 2nd edition. Page:146-1506/2/2015 3
  • 4.
    Epidemiology • <1:1000 indeveloped countries; 10:1000 schoolchildren in developing countries • It is rarer, but still accounts for half of cardiac disease in the developing world • Typically affects children aged 5–15 years from lower socio-economic • No sex difference but mitral stenosis are more common in females Oxford Handbook of Cardiology. 2nd edition. Page:146-1506/2/2015 4
  • 5.
    Pathology • Typically occursseveral weeks after a streptococcal pharyngitis • Usually group A beta haemolytic streptococci: Streptococcus pyogenes serotype M. Antigenic mimicry is implicated — antibodies to carbohydrate in cell wall (anti-M antibodies) of group A Streptococcus cross-react with protein in cardiac valves Oxford Handbook of Cardiology. 2nd edition. Page:146-1506/2/2015 5
  • 6.
    Pathology (Continue) • Delayfrom acute infection to onset of rheumatic fever is usually 3–4 weeks • RF is thought to complicate up to 3 % of untreated streptococcal sore throats • Commonly causes a pancarditis Oxford Handbook of Cardiology. 2nd edition. Page:146-1506/2/2015 6
  • 7.
  • 8.
    Clinical features • Sorethroat 1–5 weeks earlier is reported in two-thirds of cases • Fever, abdominal pain, and epistaxis • Migratory large-joint polyarthritis starting in the lower limbs in 75 % of cases • Pancarditis in 50 % of cases with features of acute heart failure, mitral and aortic regurgitation, an apical, and pericarditis • Chorea in 10–30 % , usually 1–6 months after the index pharyngitis 6/2/2015 8Oxford Handbook of Cardiology. 2nd edition. Page:146-150
  • 9.
    Clinical features (Continue) •Erythema marginatum is an evanescent rash and central clearings on the trunk and proximal limbs • Subcutaneous nodules in 0–8 % of cases several weeks after the onset of severe pancarditis 6/2/2015 9Oxford Handbook of Cardiology. 2nd edition. Page:146-150
  • 10.
    Diagnosis Criteria Oxford Handbookof Cardiology. 2nd edition. Page:146-1506/2/2015 10
  • 11.
    Rheumatic heart disease •Rheumatic heart disease is a complication of rheumatic fever • Divided into rheumatic endocarditis, rheumatic myocarditis and rheumatic pericarditis, often for rheumatic pancarditis • 60% to 80% children are associated with pancarditis 6/2/2015 11Oxford Handbook of Cardiology. 2nd edition. Page:146-150
  • 12.
    6/2/2015 12 Key morphologic featuresof acute rheumatic heart disease
  • 13.
    Prevention • The onlyknown way to prevent rheumatic fever is to treat strep. throat infections promptly with a full course of appropriate antibiotics 6/2/2015 13Oxford Handbook of Cardiology. 2nd edition. Page:146-150
  • 14.
    Prevention (Continue) • Ifyou have or suspect you have rheumatic fever see your doctor or local health clinic • If you have had rheumatic fever make sure you always have your regular penicillin injections • Get regular check-ups at your local health clinic • Do not ignore a sore throat. Consult your doctor 6/2/2015 14Oxford Handbook of Cardiology. 2nd edition. Page:146-150
  • 15.
    Prevention (Continue) • Keepsores clean and covered • Wash hands regularly • Watch out for symptoms in children – people aged between 5 and 14 • Eat a healthy diet 6/2/2015 15Oxford Handbook of Cardiology. 2nd edition. Page:146-150
  • 16.
    Prevention (Continue) Primary Prevention: Primaryprevention of rheumatic fever is possible with a 10-day course of penicillin therapy for streptococcal pharyngitis. However, primary prevention is not possible in all patients Park's Pediatric Cardiology for Practitioners. 6th edition. Page: 603-610 6/2/2015 16
  • 17.
    Secondary Prevention Who shouldreceive prophylaxis? Patients with documented histories of rheumatic fever, including those with isolated chorea and those without evidence of rheumatic heart disease, must receive prophylaxis Park's Pediatric Cardiology for Practitioners. 6th edition. Page: 603-610 6/2/2015 17
  • 18.
    Secondary Prevention (Continue) Forhow long? Ideally, patients should receive prophylaxis indefinitely. For patients who had acute rheumatic fever without carditis, the prophylaxis should continue for at least 5 years or until the person is 21 years of age Park's Pediatric Cardiology for Practitioners. 6th edition. Page: 603-610 6/2/2015 18
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