Rheumatic fever (acute rheumatic fever) is a disease that can affect the heart, joints, brain, and skin. Rheumatic fever can develop if strep throat and scarlet fever infections are not treated properly. Early diagnosis of these infections and treatment with antibiotics is key to preventing rheumatic fever.

1. ACUTE RHEUMETIC FEVER
Memoona Arshad
Group 11 – 6th Sem
ISM – IUK
Presented to: Kushubakova Nadira

2. INTRODUCTION
 Rheumatic fever (acute rheumatic fever) is a
disease that can affect the heart, joints, brain, and
skin.
 Rheumatic fever can develop if strep throat and
scarlet fever infections are not treated properly.
 Early diagnosis of these infections and treatment
with antibiotics are key to preventing rheumatic
fever.

4. EPIDEMIOLOGY
 Frequency:
 USA
 Mortality/Morbidity
 Race
 Sex
 Age

6. PATH PHYSIOLOGY
 Acute Rheumatic Fever is characterized by non-
suppurative inflammatory lesions of the joints, heart,
subcutaneous tissue, and central nervous system.
 An extensive literature search has shown that, at least in
developed countries, rheumatic fever follows pharyngeal
infection with rheumatogenic group A streptococci.
 The risk of developing rheumatic fever after an episode
of streptococcal pharyngitis has been estimated at 0.3-
3%.
 Investigations of rheumatic fever occurring in the
aboriginal populations of Australia suggest that
streptococcal skin infections might also be associated
with the development of rheumatic fever and that group
C and G streptococci may also serve as initiating
pathogens.

7. PATH PHYSIOLOGY (CONTD..)
 Although several classic group A streptococcal emm
types are considered to be rheumatogenic and most
likely to be associated with acute rheumatic fever,
in Oceania and Hawaii, group A streptococcal strains
not traditionally associated with rheumatic fever have
been found to cause the disease.
 This diversity of potential inciting group A streptococcal
strains also appears to be a common phenomenon in
lower and middle income countries.
 Molecular mimicry accounts for the tissue injury that
occurs in rheumatic fever.
 Both the humoral and cellular host defenses of a
genetically vulnerable host are involved. In this process,
the patient's immune responses (both B- and T-cell
mediated) are unable to distinguish between the
invading microbe and certain host tissues.

8. PATH PHYSIOLOGY (CONTD..)
 T helper 1 and cytokine Th17 appear to be key
mediators of rheumatic heart disease.
 The resultant inflammation may persist well beyond
the acute infection and produces the protean
manifestations of rheumatic fever.

9. PATHOGENESIS

10. CLINICAL FEATURES
 Fever
 Fatigue
 Carditis
 inflammation of the heart muscle and heart tissue.
 Carditis can cause a rapid heart rate, fatigue, shortness
of breath and exercise intolerance.
 This is the most serious of the symptoms and may have
long-term effects on health.
 Carditis occurs in approximately 50 percent of those
who have rheumatic fever
 Arthritis
 swelling, redness and pain in the joints, especially
knees, ankles, elbows and wrists.
 This is a common symptom and occurs in approximately
70 percent of people who have rheumatic fever

11. CLINICAL FEATURES (CONTD..)
 Chorea
 involuntary movement of the extremities.
 This is more common in females
(previously called “St. Vita’s dance”)
 Splotchy rash that doesn’t itch
 Subcutaneous nodules
 tiny, hard lumps under the skin
 Rash that appears as pink rings with a clear center
(this is a rare symptom)

14. CLASSIFICATIONS
 Based on the duration of symptoms and the
outcome of the disease, rheumatic fever may be
classified into either acute or chronic.
Types Characteristics
Acute rheumatic
fever
•Group A beta-hemolytic streptococcal pharyngitis
•Lesions in the endocardium, myocardium, or pericardium
•Aschoff bodies (histology)
Chronic rheumatic
fever
•Pancarditis
•Cardiac abnormality
•Heart failure and/or stenosis
•MacCallum plaques (Mural endocardial lesions in the left atrium
due to regurgitant blood flow jets from incompetent mitral valve.
•Cardiac valvular lesions
•Postinflammatory marginal fibrosis of valvular leaflets/mitral
stenosis

15. CAUSES
 Rheumatic fever may develop after strep
throat or scarlet fever infections that are not treated
properly.
 Bacteria called group A Streptococcus or group A strep
cause strep throat and scarlet fever.
 It usually takes about 1 to 5 weeks after strep throat or
scarlet fever for rheumatic fever to develop.
 Rheumatic fever is thought to be caused by a response
of the body’s defense system (the immune system).
 The immune system responds to the earlier strep throat
or scarlet fever infection and causes a generalized
inflammatory response.

16. COMPLICATIONS
 Inflammation caused by rheumatic fever can last a few
weeks to several months.
 In some cases, the inflammation causes long-term
complications.
 Rheumatic fever can cause permanent damage to the
heart (rheumatic heart disease).
 It usually occurs 10 to 20 years after the original illness,
but severe cases of rheumatic fever can cause damage
to the heart valves while your child still has symptoms.
 Problems are most common with the valve between the
two left chambers of the heart (mitral valve), but the
other valves can be affected.

17. COMPLICATIONS (CONTD..)
 The damage can result in:
 Narrowing of the valve
 This decreases blood flow.
 Leak in the valve
 A leaky valve causes blood to flow in the wrong direction.
 Damage to heart muscle
 The inflammation associated with rheumatic fever can weaken
the heart muscle, affecting its ability to pump.

18. COMPLICATIONS (CONTD..)
 Damage to the mitral valve, other heart valves or
other heart tissues can cause problems with the
heart later in life.
 Resulting conditions can include:
 An irregular and chaotic heartbeat (atrial fibrillation)
 Heart failure

19. RISK FACTORS
 Factors that can increase the risk of rheumatic
fever include:
 Family history
 Some people carry a gene or genes that might make them
more likely to develop rheumatic fever.
 Type of strep bacteria
 Certain strains of strep bacteria are more likely to contribute to
rheumatic fever than are other strains.
 Environmental factors
 A greater risk of rheumatic fever is associated with
overcrowding, poor sanitation and other conditions that can
easily result in the rapid transmission or multiple exposures to
strep bacteria.

21. EFFECTS
 The long-term effects of rheumatic fever can be
disabling if your child has a severe case.
 Some of the damage caused by the illness might
not show up until years later.
 Be aware of long-term effects as your child grows
older.
 If your child does experience long-term damage
related to rheumatic fever, there are support
services available to help them and your family.

22. DIAGNOSIS
 Your child’s doctor will first want to get a list of your child’s symptoms
and their medical history.
 They’ll also want to know if your child has had a recent bout of strep
throat.
 Next, a physical exam will be given.
 Your child’s doctor will do the following, among other things:
 Look for a rash or skin nodules.
 Listen to their heart to check for abnormalities.
 Perform movement tests to determine their nervous system dysfunction.
 Examine their joints for inflammation.
 Test their throat and sometimes blood for evidence of strep bacteria.
 Perform an electrocardiogram (ECG or EKG), which measures the
electric waves of their heart.
 Perform an echocardiogram, which uses sound waves to produce images
of their heart.

24. DIAGNOSIS (CONTD..)
Major Criteria Arthritis (most common symptom) –
inflamed joints
Carditis (heart inflammation) >> valves
Chorea (jerky movements)
Subcutaneous nodules (rare)
Erythema marginatum (skin rash)
Minor Criteria Arthralgia (joint aches), fever, elevated
acute phase reactants, prolonged PR
interval (change on ECG)

25. JONES CRITERIA
 In the revised 2015 Jones criteria, a low, medium
and high-risk population was identified. A low risk
population is one in which cases of acute RF occur
in ≤ 2/100 000 school-age children or rheumatic
heart disease is diagnosed in ≤ 1/1000 patients at
any age during one year.

26. MODIFIED BY JONES
Major Criteria
Low Risk Population High Risk Population
Carditis (clinical or subclinical)
Arthritis – only polyarthritis
Chorea
Erythema marginatum
Subcutaneous nodules
Carditis (clinical or subclinical)
Arthritis – monoarthritis or polyarthritis
Polyarthralgia
Chorea
Erythema marginatum
Subcutaneous nodules
Minor Criteria
Low Risk Population High Risk Population
Polyarthralgia
Hyperpyrexia (≥ 38.5ºC)
ESR ≥ 60 mm/h and/or CRP ≥ 3.0 mg/dl
Prolonged PR interval (after taking into
account the differences related to age; if
there is no carditis as a major criterion)
Monoarthralgia
Hyperpyrexia (≥ 38.0ºC)
ESR ≥ 30 mm/h and/or CRP ≥ 3.0 mg/dl
Prolonged PR interval (after taking into
account the differences related to age; if
there is no carditis as a major criterion)

27. LAB TESTS
 No single specific laboratory test can confirm the
diagnosis of acute rheumatic fever (ARF).
 Evidence of preceding group A streptococcal
infection is an integral part of the Jones criteria for
ARF diagnosis unless the patient has chorea
(which may occur months after the inciting
infection) or indolent rheumatic heart disease.
 These tasks can be helpful:
 Throat Culture
 Antibody Titer Tests
 Acute-phase Reactants, Erythrocyte Sedimentation
Rate, and C-reactive Protein
 Blood Cultures

28. LAB TESTS (CONTD..)
 Throat Culture
 Throat culture remains the criterion standard for confirmation
of group A streptococcal infection. Rapid antigen detection
tests are not as sensitive.
 If a rapid antigen detection test result is negative, obtain a
throat culture in patients with suspected rheumatic fever.
 On the other hand, because of the high specificity of these
tests, a positive rapid antigen test confirms a streptococcal
infection.

29. LAB TESTS (CONTD..)
• Antibody Titer Tests
 Antibody titer tests used include ASO test, antistreptococcal DNAse B
(ADB) test, and the antistreptococcal hyaluronidase (AH) test.
 ASO is a test used to detect streptococcal antibodies directed against
streptococcal lysin O. An elevated titer is proof of a previous
streptococcal infection. It is usually more elevated after a pharyngeal
than skin infection, while the ADB is typically elevated regardless of the
site of the infection.
 Acute and convalescent sera, if available, are helpful for proving recent
streptococcal infection.
 The antibody tests must be interpreted with caution in areas with high
rates of streptococcal infection and ARF, as relatively high titers are
commonly encountered in the population.
 These tests are of greater utility in areas with
lower prevalence (e.g., in most Western countries).

30. LAB TESTS (CONTD..)
 Acute-phase Reactants, Erythrocyte Sedimentation
Rate, and C-reactive Protein
 Acute-phase reactants, the erythrocyte sedimentation
rate (ESR), and C-reactive protein levels (CRP) are
usually elevated at the onset of ARF and serve as a
minor manifestation in the Jones criteria.
 These tests are nonspecific, but they may be useful in
monitoring disease activity.

31. LAB TESTS (CONTD..)
 Blood Cultures
 Blood cultures are obtained to help rule out infective
endocarditis, bacteremia, and disseminated gonococcal
infection.

32. TREATMENT
 Treatment will involve getting rid of all of the
residual group A strep bacteria and treating and
controlling the symptoms.
 This can include any of the following:
 Antibiotics
 Anti-inflammatory Treatment
 Anticonvulsant Medications
 Bed Rest

33. TREATMENT (CONTD..)
 Antibiotics
 Your child’s doctor will prescribe antibiotics and might
prescribe a long-term treatment to prevent it from
occurring again.
 In rare cases, your child may receive lifelong antibiotic
treatment.

34. TREATMENT (CONTD..)
 Anti-inflammatory Treatment
 Anti-inflammatory treatments include pain medications
that are also anti-inflammatory, such as aspirin (Bayer)
or naproxen (Aleve, Naprosyn).
 Though aspirin use in children with certain illnesses has
been associated with Reye’s Syndrome, the benefits of
using it in treating rheumatic fever may outweigh the
risks.
 Doctors may also prescribe a corticosteroid to reduce
inflammation.

35. TREATMENT (CONTD..)
• Anticonvulsant Medications
 Your child’s doctor might prescribe an anticonvulsant if
involuntary movements become too severe.

36. TREATMENT (CONTD..)
 Bed Rest
 Your child’s doctor will also recommend bed rest and
restricted activities until the major symptoms, such as
pain and inflammation, have passed.
 Strict bed rest will be recommended for a few weeks to
a few months if the fever has caused heart problems.

37. PREVENTION
 The only way to prevent rheumatic fever is to treat
strep throat infections or scarlet fever promptly with
a full course of appropriate antibiotics.

38. Prevention
Primary – 10 days
course of pensilin
therapy; about 30% of
patients with ARF do not
recall a preceding
episode of pharyngitis
Secondary – Secondary
prevention is directed at
preventing acute GABHS
pharyngitis in patients at
substantial risk of
recurrent ARF

39. MEDICATION
 Penicillin G benzathine (Bicillin L-A)
 Long-acting depot form of penicillin G. DOC for
prophylaxis of streptococcal pharyngitis. Avoids
compliance problems of oral regimens.
 Penicillin VK (Beepen-VK, Betapen-VK,
Pen-Vee K, Robicillin VK, V-Cillin K)
 Phenoxymethyl derivative of penicillin G is acid-
stable, enhancing oral bioavailability. Patient
compliance is essential for effectiveness.

40. MEDICATION (CONTD..)
 Erythromycin (E.E.S., E-Mycin, Eryc, Ery-Tab,
Erythrocin, E-Mycin)
 Macrolides inhibit protein synthesis, in contrast to penicillin
cell wall effects. DOC for primary treatment of
streptococcal pharyngitis in penicillin allergy. May use for
secondary prophylaxis in patients allergic to penicillin.
 Sulfadiazine (Microsulfon)
 Exerts bacteriostatic action through competitive
antagonism with para-aminobenzoic acid (PABA).
Microorganisms that require exogenous folic acid and do
not synthesize folic acid are not susceptible to the action of
sulfonamides. Used in secondary prophylaxis of ARF.

41. REFERENCES
 Rheumatic Fever: All You Need to Know | CDC
 Rheumatic fever - Symptoms and causes - Mayo
Clinic
 Rheumatic Fever: Causes, Treatment, and
Prevention (healthline.com)
 Rheumatic Fever Workup: Laboratory Studies,
Imaging Studies, Other Tests (medscape.com)
 Rheumatic fever – new diagnostic criteria (nih.gov)
 Rheumatic fever classification - wikidoc

42.  THANK YOU 