Rheumatic fever is an autoimmune disease that can occur as a result of a streptococcal throat infection. It affects multiple body systems but commonly involves the heart, joints, and brain. Symptoms may include heart inflammation (carditis), painful and migratory swollen joints (arthritis), jerky involuntary movements (chorea), and others. The disease is caused by an abnormal immune response that causes antibodies produced against streptococcal bacteria to also attack human tissues. Treatment involves bed rest, antibiotics to treat the initial infection, and anti-inflammatory drugs. Recurrences of the disease can be prevented with long-term antibiotic prophylaxis but cardiac damage may persist long-term in the form of rheumatic heart disease.

1. RHEUMATIC FEVER
Presented by
Intern Binod Chaudhary
MBBS 4th Batch
CMC

2. Introduction
• Acute rheumatic fever is an immunological disorder
resulting due to infection with group A streptococcus
and affecting multiple system.
• Many parts of the body may be affected and almost
all of the manifestations resolve completely except
cardiac valvular damage (Rheumatic heart disease)
which may persist after other features have
disappeared.

3. Epidemiology
• ARF is mainly a disease of children aged 5-14 years
and rare in persons age >30 yrs.
• The incidence following streptococcal throat
infection is 0.3% in general population and 1-3% in
presence of epidemics of streptococcal sore throat.
• Predisposing factors:
poor socioeconomic condition
Unhygienic living conditions
overcrowded housing conditions

4. Etiology
• Unknown
• Strong association with beta hemolytic
streptococci is indicated by number of
observations
h/o preceding sore throat is available in less tha
50% patients
Epidemics of streptococcal infection are followed
by higher incidence of RF
The seasonal variation of RF and streptoccal
infection are identical

5. In patients with established RHD streptococcal
infection is followed by recurrence of acute RF
Penicillin prophylaxis for streptococcal
infection prevents recurrences of RF in those
patients who have had it earlier.
More than 85% of the patients with acute RF
shows elevated levels of anti-streptococcal
antibody titer.

6. Pathogenesis
• Still not clear.
• Several theories have been proposed -
I. The Cytotoxicity theory and
II. The immunologic theory
The cytotoxicity theory –
GAS toxin may be involved.
GAS produces several enzymes that are
cytotoxic, most common being Streptolysin O.

7. Immunologic theory
• Most widely accepted theory.
• Based on molecular mimicry.
• Immune response targeted at streptococcal
antigens also recognizes human tissues, cross
react with endothelial cells on the heart valve,
leading to recruitment of activated
lymphocytes, release of peptides, activation of
cross reactive T cells and damage of
endothelial cells.

8. Pathogenesis of rheumatic fever

9. Clinical features
• Sore throat with fever about 2 weeks prior
• Guidelines for clinical diagnosis of acute
rheumatic fever suggested by T. Duckett Jones
consists of major, minor and essential criteria
• 2 major or 1 major+2minor criteria in addition
to essential criteria is required to diagnose
acute rheumatic fever.

11. Revised Jones criteria, 2015
Low risk population Moderate /high
risk population
Major criteria Evidence of recent
GAS infection
Carditis
Arthritis
Clinical and/or
subclinical
Polyarthritis
Chorea
Erythema marginatum
Subcutaneous nodules
Clinical and/or
subclinical
Monoarthritis,
polyarthritis and/or
polyarthralgia
Chorea
Erythema marginatum
Subcutaneous nodules
Positive throat culture
or rapid streptococcal
antigen test
Elevated or increasing
streptococcal
antibody titer.
Minor criteria
Arthralgia
Fever
Markers of
inflammation
Prolonged PR interval
Polyarthralgia
>38.5c
Peak ESR >60 mm/hr
and/or CRP >3.0
mg/dl
Prolonged PR interval
Monoarthralgia
>38c
Peak ESR >30 mm/hr
and/or CRP >3.0
mg/dl

12. Guidelines contd..
• Initial attack – 2 major or 1 major and 2 minor
manifestations, plus evidence of recent GAS infection.
• Recurrent attack – 2 major, or 1 minor and 2 major, or 3
minor manifestations( only in the moderate/high risk
population ), plus evidence of recent GAS infection.
• Low risk population is defined as ARF incidence <
2/1,00,000 school age children per year, or all age RHD
prevalence of < 1/1000 population.
• Moderate/high risk population is defined as ARF
incidence >2/1,00,000 school age children per year, or all
age RHD prevalence of >1/1,000 population.

13. Guidelines contd..
• Carditis is now defined as clinical and/or subclinical
(echocardiac valvulitis).
• Arthritis (major) refers only to polyarthritis in low risk
populations, but also to monoarthritis or polyarthralgia
in moderate/high risk population.
• Minor criteria for moderate/high risk population only
include monoarthralgia (polyarthralgia for low risk
population), fever of >38c (>38.5c in low risk
populations), ESR >30mm/hr (>60 mm/hr in low risk
population).

14. Exceptions of Jones criteria
1. When chorea occurs as the only major
manifestation of ARF.
2. When indolent carditis is the only manifestation
in patient who first come to medical attention
only months after the apparent onset of ARF.
3. In a limited number of patients with recurrences
of acute rheumatic fever in particularly high risk
populations.

15. Major manifestations
Carditis
• An early manifestation of rheumaric fever
• Echocardiographic studies indicates that it occurs in
almost 90% of the patients
• Rheumatic carditis is pancarditis.
• Severity ranges from fulminant, potentially fatal
exudative pancarditis to mild, transient cardiac
involvement.
• Endocarditis ( valvulitis ) is a universal finding.
• Most cases consist of either isolated mitral valvular
disease or combined aortic and mitral valvular disease.

16. Carditis contd..
• Signs of carditis include the development of new murmurs,
cardiac enlargement, CHF, pericardial friction rub, and/ or
pericardial effusion.
• Characteristic murmurs of acute carditis –
• High pitched blowing, holosystolic apical murmur of mitral
regurgitation
• Low pitched apical, mid-diastolic flow murmur( Carey-
Coombs murmur )
• And a high pitched, decrescendo, diastolic murmur of aortic
regurgitation .
• Murmur of MS and AS are observed in chronic cases.

17. • A major change in the 2015 revision of the
Jones Criteria is the acceptance of subclinical
carditis.
• Subclinical carditis – defined as the carditis
without a murmur of valvulitis but with a
echocardiographic evidence of valvulitis.

18.  Migratory polyarthritis
• Occurs in about 75% of patients with ARF.
• Earliest manifestation
• Typically involves large joints.
• Generally hot red, swollen, and exquisitely tender.
• Severely inflammed joint can become normal
within 1-3 days without treatment, as one or other
joints become inflammed.
• A dramatic response to even to small doses of
salicylates is characteristic
• Non deforming.

19. Sydenhams Chorea
• Occurs in 10 – 15% cases.
• Late manifestation
• Consists of semi-purposeful, jerky movements
resulting in deranged speech, muscular inco-
ordination, awkward gait and weakness.
• The affected child is emotionally disturbed.
• More common in females.
• Self limiting course.

20. Erythema marginatum
• Rare manifestation (approximately 1% of cases
of ARF).
• Consists of erythematous, serpiginous,
macular lesions, with pale centers that are
non pruritic.
• Occurs on the trunk and extremities.

22. Subcutaneous nodules
• very rare ( < 1% of cases of ARF ).
• Consists of small, firm, painless, mobile
nodules approximately 1 cm in diameter along
the extensor surfaces of tendon near bony
prominences.
• Significant correlation between subcutaneous
nodule and rheumatic heart disease.

24. Minor manifestations
• Clinical criteria
I. Fever – rheumatic fever is almost always associated with fever.
Low risk populations at least 38.5C, moderate/high risk
populations 38C.
II. Arthralgia - polyarthralgia in low risk while monoarthralgia in
moderate/high risk population.
• Laboratory manifestations –
I. Acute phase reactant – elevated ESR 60 mm/hr in low risk while
30 mm/hr in high risk populations.
CRP at least 3 mg/dl in both low and high risk.
I. Prolonged PR interval

25. Essential criteria
• Supporting evidence of a recent GAS infection.
• ARF typically develops 2-4 wk after an acute episode of GAS
pharyngitis.
• 10 - 20% of the throat culture or rapid streptococcal
antigen test shows positive result.
• 80 – 85% of patients have an elevated ASO titer.
• 95 – 100% have an elevation if 3 different antibodies ( ASO,
anti-Dnase B, antihyaluronidase) are measured.

26. Differential diagnosis
Arthritis Carditis Chorea
Juvenile idiopathic arthritis
Reactive arthritis
Serum sickness
Sickle cell disease
Malignancy
SLE
Pyogenic arthritis
Post streptococcal reactive
arthritis
Viral myocarditis
Viral pericarditis
Infective endocarditis
Kawasaki disease
Congenital heart disease
Mitral valve prolapse
Innocent murmurs
Huntingtons chorea
Wilson disease
SLE
Cerebral palsy
Hyperactivity

27. Treatment
 Bed rest –
• Recommended for acute rheumatic fever.
• When carditis is present, immobilization may be needed for 1 – 3
months.
• When carditis is not present, patient can ambulate in 2 – 3 wks.
 Antibiotics -
• Single injection of intramuscular benzathine penicillin(60,000 IU in a
child <27 kg and 1.2 million IU in child >27 kg) or
• 10 days of oral penicillin v( 250 mg bid/tid for child <27 kg, 500mg
bid/tid for child >27 kg) or
• 10 days of oral amoxicillin 50 mg/kg once daily.
• 5 days of oral azithromycin 12mg/kg once daily.
• Oral clindamycin and erythromycin for 10 days.

28. Treatment contd..
• Anti-inflammatory agents -
Patients with typical migratory polyarthritis and with
carditis but without cardiomegaly or CHF.
• Aspirin 50-70 mg/kg/day in four divided doses PO for 3 –
5 days, followed by 50 mg/kg/day in 4 divided doses PO
for 3 wk then half that dose for 2-4 wk.
Patients with carditis and cardiomegaly or CHF
• Prednisone 2mg/kg/day in 4 divided doses for 2-3 wks
followed by half of that dose for 2-3 wk then tapering by
5mg/24hr every 2-3 days.

29. Treatment contd..
• At the beginning of the tapering of prednisone
dose, aspirin should be started at 50
mg/kg/day in 4 divided dose for 6 wk.
• Supportive therapies for patients with
moderate to severe carditis include digoxin,
fluid and salt restriction, diuretics, and
oxygen.

30. Treatment contd..
Sydenhams chorea
• Sedatives may be helpful early in the course of
chorea.
• Phenobarbitol is the drug of choice.
16-32mg every 6-8 hr PO.
• Haloperidol - 0.01-0.03 mg/kg/day divided bid PO.
• Chlorpromazine – 0.5 mg/kg every 4-6 hr PO.

31. Prognosis
• Depends on the clinical manifestations at the time of initial
presentation, the severity of initial episode, and the presence
of recurrences.
• About 50 – 70% of carditis during the initial episode recover
without residual heart disease.
• Approximately 20% of patients who present with pure chorea
who are not given secondary prophylaxis develop rheumatic
heart disease within 20 year.

32. prevention
• Primary prevention
• Appropriate antibiotic therapy initiated before
9th day of acute GAS pharyngitis is highly
effective in preventing 1st attack of acute
rheumatic fever.

33. Secondary prophylaxis
Drug Dose Route
Penicillin G
benzathine
6,00,000 U for children < 27 kg,
1.2 million U for children > 27 kg,
every 4 wk*
Intramuscular
Penicillin V 250 mg, twice a day Oral
Sulfadiazine or
sulfisoxazole
0.5 gm once a day for patients < 27
kg
1.0 gm once a day for patients > 27
kg
Oral
Macrolide or azalide Variable Oral
*In high risk situations, administration every 3 wk is recommended

34. Duration of prophylaxis
Category Duration
Rheumatic fever without carditis 5 yr or until 21 yr of age, whichever is
longer
Rheumatic fever with carditis but
without residual heart disease ( no
valvular heart disease )
10 yr or until 21 yr of age, whichever
is longer
Rheumatic fever with carditis and
residual heart disease( persistant
valvular heart disease)
10 yr or until 40 yr of age , whichever
is longer, sometimes lifelong