Acute rheumatic fever (ARF) is a multisystem autoimmune disease triggered by infection with group A streptococci, predominantly affecting children aged 5-15 years, particularly in developing nations. Clinical presentations include migratory arthritis, carditis, and chorea, with diagnostics based on the presence of preceding streptococcal infection and characteristic symptoms. Treatment involves antibiotics, anti-inflammatory agents, and long-term prophylaxis to prevent recurrence and complications such as chronic rheumatic heart disease.

1. Presented by :MTUTH
Fifth year pharmacy
Group four student's
4/2/2023 1

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3. content
Introduction
Epidimology
Etiology
Pathophysiology
Clinical presentation
Diagnosis
Treatment
Progress
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4.  Multisystem disease resulting from autoimmune reaction to infection
with group A β haemolytic streptococci.
 Some children present with fever and pains in the large joints, which
may move from one joint to another.
 The infection can damage the heart valves (especially the mitral and
aortic valves), leading to respiratory distress and heart failure
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Introduction

5.  Children with mild disease may have only a heart
murmur.
 Severe disease can present with fever, fast or difficult
breathing and lethargy.
 The child may have chest pain or fainting.
 Those that present with heart failure have a rapid
heart rate, respiratory distress and an enlarged liver.
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6. Epidemiology
 In the early1960, acute rheumatic fever and its major
complication, rheumatic valvular heart disease, were
frequent worldwide.
 Nowadays it almost disappeared in the western world,
however it is still prevalent in developing countries.
 It is frequent in children aged between 5 and 15 years because
they are susceptible to Group A streptococcal pharyngitis.
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7. ♣ Approximately 20% of all sore throats are due to Group A
streptococcal and only few (0.5 to 3%) develop acute rheumatic
fever.
♣ The risk depends on the host factors (age, genetic predisposition
and carriers), if untreated, and the streptococcal strain (M type).
♣ Streptococcal skin infection does not result in acute rheumatic
fever.
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8. ♣ Major risk factor for the frequent occurrence of acute
rheumatic fever in developing countries are
 Climate (high temperature)
 poverty
 crowding
 poor housing conditions and
 inadequate health services (Shaper 1972).
♣
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9. Cytotoxicity theory-states the enzyme streptolysin O is
directly toxic to cardiac cells, but, its unable to explain the
substantial period (10-21 days) to develop ARF from GAS
pharyngitis.
Immune-mediated pathogenesis- molecular mimicry resulting
immunologic cross-reactivity between cardiac antigenic
epitopes and GAS cellular and extra cellular epitopes
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Pathogenesis

10. Antibodies to cardiac valve tissue cross-react with the N acetyl
glucosamine of group A streptococcal, and that these antibodies
may be responsible for
 valvular damage,
 muscle and pericardium (pancarditis),
 joint synovium (arthritis),
 nervous tissue (sydneham chorea),
 skin (erythema marginatum) and
 subcutaneous tissue (subcutaneous nodule).
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12.  Latent period of ~3 weeks (1–5 weeks)
 Exceptions are chorea and indolent carditis: prolonged latent periods
lasting up to 6 months
 The prevalence of chorea in ARF varies substantially between
populations, ranging from <2% to 30%
 Erythema marginatum and subcutaneous nodules are now rare, being
found in <5% of cases
 The most common clinical presentation of ARF is polyarthritis and
fever
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Clinical presentation

13. Carditis- occurs in 40 to 80% of patients
It presents with fever, dyspnea and cough and the main signs
are tachycardia, mitral regurgitation murmur and cardiac
enlargment.
In severe cases there are signs of heart failure
Aortic regurgitation may occur with the mitral regurgitation
Other signs are arrhythmia and pericarditis
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14. Arthritis is the commonest manifestation
It is migratory and involves several larger joints (knee, ankle,
elbow and wrist)
The affected joint is swollen, red warm and exquisitely tender.
These signs disappear within 24 hours if anti-inflammatory drug
(salicylates,NSAIDs)is given.
It may resolve completely without any residual sequel.
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15. Chorea: in 10-15% of patient
♣ is purposeless jerky, repetitive movements particularly
involving the hands and face.
♣ Early manifestations are emotional liability (easily induced
crying and inattention) and deterioration in handwriting.
♣ Occurs at the same time or following other features. It
usually occurs several months after the sore throat.
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16. Erythema marginatum – is a serpiginious rash over the
chest and trunk
 It disappears in hours or days.
Subcutaneous nodule – is small painless nodules which
occur more frequently on the
 extensor surface of large joints (elbow, wrist and knee)
and spine.
Other minor manifestations are fever and arthralgia.
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17. Laboratory Findings
 Laboratory findings may include:
 Increased acute phase reactants – elevated ESR and C-reactive
protein
 CXR – may show cardiomegaly
 Evidence of preceding streptococcal infection
 elevated antistreptolysin O antibody (ASO) titer
 throat culture
 Other tests - ECG, Echocardiography
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18.  Major
manifestati
ons
 Carditis
 Polyarthritis
 Chorea
 Erythema marginatum
 Subcutaneous nodules
 Minor
manifestati
ons
 fever,
 polyarthralgia
 Electrocardiogram: prolonged P-R interval
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Diagnosis

19.  Supporting evidence of a
preceding streptococcal
infection within the last 45 days
 Elevated or rising anti-
streptolysin O or other
streptococcal antibody, or
 A positive throat culture, or
 Rapid antigen test for group A
streptococcus, or
 Recent scarlet fever
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20. Diagnostic Categories Criteria
 Primary episode of RF  Two major or one major and two
minor manifestations plus
evidence of preceding group A
streptococcal infection
 Recurrent attack of RF in a
patient without established
RHD
 Two major or one major and two
minor manifestations plus
evidence of preceding group A
streptococcal infection
 Recurrent attack of RF in a
patient with established RHDb
 Two minor manifestations plus
evidence of preceding group A
streptococcal infectionc
DIAGNOSIS: MODIFIED JONE’S CRITERIA
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21.  Rheumatic chorea  Other major manifestations or
evidence of group A
streptococcal infection not
required
Insidious onset rheumatic
carditisb
 Chronic valve lesions of RHD
(patients presenting for the
first time with pure mitral
stenosis or mixed mitral valve
disease and/or aortic valve
disease)
 Do not require any other
criteria to be diagnosed as
having RHD
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Algorithm for diagnosis

23. Treatment of ARF
Antibiotic Therapy:Treatment with of Group A
streptococcal pharyngitis
10 days of orally administered penicillin or erythromycin
or a single IM injection of benzathine penicillin if<30kg
600,000IU and if >30kg 1.2M IU to eradicate GABHS from the
upper respiratory tract
Afterwards, the patient should be started on long-term
antibiotic prophylaxis
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24. Anti-inflammatory Therapy:
 Anti-inflammatory agents (salicylates, corticosteroids) should
be withheld if arthralgia or atypical arthritis is the only clinical
manifestation of presumed acute rheumatic fever
 Acetaminophen can be used
Patients with typical migratory polyarthritis & with carditis
without cardiomegaly or congestive heart failure:
treatment with oral salicylates, 100 mg/kg/day in 4 divided doses
PO for 3-5 days, followed by 75 mg/kg/day in 4 divided doses
PO for 4-8 wk
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25. Patients with carditis & cardiomegaly or congestive heart
failure:
 treatment with corticosteroids
 Prednisone 2 mg/kg/day in 4 divided doses for 2-6 wk
followed by a tapering of the dose that reduces the dose by
5 mg/24 hr every 2-3 days.
 At the beginning of the tapering of the prednisone dose,
aspirin should be started at 75 mg/kg/day in 4 divided doses to
complete 12 wk of therapy
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26. Supportive therapies for patients with moderate to severe
carditis include digoxin, fluid & salt restriction, diuretics
& oxygen
The cardiac toxicity of digoxin is enhanced with
myocarditis
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27. Sydenham Chorea
Occurs after the resolution of the acute phase of the disease
Anti-inflammatory agents are usually not indicated
Sedatives: phenobarbital (16-32 mg every 6-8 hr PO) is the
drug of choice
If phenobarbital is ineffective, then 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
Long-term antibiotic prophylaxis
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28. PREVENTION
 PRIMARY-10 days course of amoxicillin or single i.m. injection
of Benzathine penicillin.; Early (within 1 week) treatment of
streptococcal pharyngitis to prevent an initial attack of acute
rheumatic fever.
 SECONDARY-Prevent recurrences so as to reduce the damage to
heart valves and the risk of chronic rheumatic heart disease.
 Indicated for children who have had acute rheumatic fever or have
rheumatic heart disease.
 Monthly single i.m. benzathine penicillinor daily oral penicillin
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29. Complications
 Recurrence of rheumatic fever
 Chronic rheumatic valvular heart disease
 Infective endocarditis
 Arrhythmia
 Stroke ( Thromboembolic )
 Heart failure
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30. Prognosis
♣ Untreated, ARF lasts on average 12 weeks.
♣ With treatment, patients are usually discharged from hospital within
1–2 weeks
♣ Markers should be monitored every 1–2 weeks until they have
normalized (usually within 4–6 weeks), and an echocardiogram
should be performed after 1 month to determine if there has been
progression of carditis.
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31. Category of Patient Duration of Prophylaxis
Patient without proven carditis For 5 years after the last attack or 18 years
of age (whichever is longer)
Patient with carditis (mild mitral
regurgitation or healed carditis)
For 10 years after the last attack, or 25
years of age (whichever is longer)
More severe valvular disease Lifelong
Valvular surgery Lifelong
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32. Reference
1. Nelson Behrman, Kliegman, Text Book of pediatrics; 2000.
2. Jordan, Scott. Heart disease in pediatrics.1989.
3. Levy, Sheldon, Sulyman. Diagnosis and Management of the
hospitalized child. 1984 aver
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