Acute rheumatic fever is an autoimmune disorder caused by an untreated streptococcal throat infection. It commonly affects children ages 5-15 and can cause long-term heart damage through inflammation of the heart valves and muscles. Symptoms include migratory joint pain, skin rashes, and involuntary movements. Treatment involves bed rest, antibiotics to treat strep, aspirin or steroids to reduce inflammation, and lifelong antibiotic prophylaxis to prevent recurrence and further heart damage. Without proper treatment and prevention, it can lead to serious and permanent heart valve problems.

1. ACUTE RHEUMATIC
FEVER

2. INTRODUCTION
 Acute rheumatic fever is an immunological disorder
initiated by group A beta hemolytic streptococcus
 Antibodies produced against some streptococcus cell
wall proteins and sugars react with the connective
tissue and heart and result in rheumatic fever

3. EPIDEMIOLOGY
 It constitutes 17 – 50 % of all cardiac patients in
hospital
 Prevalence rate : 0.55 – 0.67 /1000
 Incidence rate : 5.3 / 1000 ( ICMR survey 2010)
 Age : 5 to 15 years
 Both sex are equally affected but mitral valve disease
and chorea are common in female , and aortic valve
involvement is more in male

4. PREDISPOSING FACTORS
 Poor socioeconomic status
 Overcrowding
 Under and poor nutrition
 Family history of rheumatic disease
 Age group 5 – 15 yrs ( peak incidence at 8 yrs)

5. ETIOPATHOGENESIS
 Etiology is unknown
 Strong association with beta hemolytic streptococcus
is indicated by :
 History of preceding sore throat in 50% of cases
 Epidemics of streptococcus infection are followed
by higher incedence
 Seasonal variation of both are identical
 Penicillin prophylaxis prevents recurrence
 > 85% patients show eleveted anti streptococcal
antibody titer

6.  Following streptococcal sore throat there is latent
period of 10 days to several weeks
 Streptococci have never isolated from rheumatic
lesions in joints, heart or blood stream
 Streptococcal products against which antibodies
produced are streptolysin , hyluronidase , erythrogenic
toxins , deoxyribonuclease
 Association with HLA – DR3 and B cell antigen serum
883

7.  Patient of rheumatic fever produce antibody against
streptococcal cell wall and membrane protiens
 Streptococcal antigen and human myocarium
appears to be identical antigenically
 These antibodies react with human connective tissue
mainly cardiac , striated and vascular smooth muscle
 Immunoflurescent techniques – antibodies atteched
to sarcolemma of cardiac muscle

8.  Streptococcus has hyaluronic acid capsule that
prevents phagocytosis
 N-acetyl glucosamine is component of cell wall
carbohydrate which is immunologically active
 That is also present in human connective tissue
 N-acetyl glucosamine cross react with antiserum
against human connective tissue

9. DIAGNOSIS CRITERIA

10. CARDITIS
 Pancarditis
 50 – 60 % of patients
 Early manifestation , around 80 % of patients
developed carditis in first 2 weeks
 PERICARDITIS :
 Present in 15 % patients of carditis
 Severe precordial pain
 Friction rub
 ECG : ST and T changes

11.  MYOCARDITIS :
 Cardiac enlargement
 Soft first heart sound
 Protodiastolic gallop
 CCF
 Carey coomb’s murmur
 ENDOCARDITIS :
 Pansystolic murmur of MR and AR

12. POLYARTHRITIS
 Flitting & fleeting migratory polyarthritis, involving
major joints
 Commonly involved large joints-knee, ankle, elbow &
wrist
 Occur in 80%
 In children below 5 yrs arthritis usually mild
 Arthritis do not progress to chronic disease
 Rheumatic joints are generally hot, red, swollen,
and exquisitely tender.

13.  A dramatic response to even small doses of
salicylates .
 The absence of such a response should
suggest an alternative diagnosis.
 Rheumatic arthritis is typically not deforming.
 Arthritis is the earliest manifestation of acute
rheumatic fever.

14. ERYTHEMA MARGINATUM
 Nonpruritic serpiginous or annular erythematous
rash more prominent on the trunk & inner
proximal portions of the extremities.
 Rash is faintly reddish, not raised above the skin
and non itching
 Rash disappears on exposure to cold & reappears
after hot shower.

16. SUBCUTANEOUS NODULES
 Hard, painless, nonpruritic, freely mobile, 0.2 to 2cm
in diameter.
 Found symmetrically, single or in clusters, on
extensor surfaces of both large & small joints, over
the scalp or along the spine.
 Lasts for weeks.
 Always associated with severe carditis

18. SYDENHAM’S CHOREA
 Neuropsychiatric disorder
 10 – 15 % of patients
 More often in pre-pubertal girls than in boys.
 Characterized by involuntary movements specially of
the face and limbs, muscle weakness, disturbances of
speech and gait, poor scholastic performance
 Neurologic Signs : Choric Movement & Hypotonia
 Psychiatric Signs : Emotional Liability,
Hyperactivity, Separation Anxiety, Obsessions &
Compulsions

19.  Exceptions to the Jone’s criteria :
 Chorea may occur as the only manifestation
 Indolent carditis may be the only manifestation if
patient come to medical attentio after months of
onset
 Patients with rheumatic fever recurrence may not
fulfill the jone’s criteria

20. OTHER CLINICAL FEATURES
 Abdominal pain
 Rapid sleeping HR
 Tachycardia out of proportion of fever
 Malaise
 Anemia
 Epistaxis
 Precordial pain

21. DIFFERENTIAL DIAGNOSIS
 Juvenile rheumatoid arthritis
 Collagen vascular diseases
 Virus associated acute arthritis
 Hematologic disorder

22. CLINICAL COURSE
 Carditis can cause permanent cardiac damage, signs
of mild carditis disappear in weeks but in severe
carditis it may last for 6 months
 Arthritis subsides within a few days to weeks without
treatment
 Chorea gradually subsides in 6 to 7 months and
usually does not cause permanent neurologic
sequelae

23. MANAGEMENT
 Investigation :
 CBC
 Acute phase reactant
 Throat culture
 ASO titer
 Chest x ray
 ECG
 2D echo

24. BED REST
ARTHRITIS
ALONE
MILD
CARDITIS
MODERATE
CARDITIS
SEVERE
CARDITIS
BED REST 1-2 wk 3-4 wk 4-6 wk As long as CCF
present
INDOOR
AMBULATION
1-2 wk 3-4 wk 4-6 wk 2-3 months

25. ANTIBIOTICS
 Benzathine penicillin G 0.6 to 1.2 million units IM
 This serves as first dose of penicillin prophylaxis
 In patients allergic to penicilline :
Erythromycine 40 mg/kg/day in 2 to 4 doses for 10
days

26. ANTI INFLAMMATORY AGENTS
 mild to moderate carditis : aspirine 90-100
mg/kg/day in 4 to 6 divided doses for 4 to 8 weeks,
after improvement therapy is withdrawn over 4 to 6
weeks
 Arthritis : aspirin is continued for 2 weeks and
gradually withdrawn over 2 to 3 weeks
 Severe carditis : prednisone 2 mg/kg/day in four
divided doses for 2 to 6 weeks

27. Anti inflammatory agents
ARTHRITIS
ALONE
MILD
CARDITIS
MODERATE
CARDITIS
SEVERE
CARDITIS
PREDNISONE - - - 2 – 6 wk
ASPIRIN 1 – 2 wk 3 – 4 wk 6 – 8 wk 2 – 4 momths

28. TREATMENT OF CHF
 Complete bed rest with orthopneic position
 Moist and cool oxygen
 Prednisone
 Digoxine
 Furosemide if indicated

29. MANAGEMENT OF SYDENHM’S CHOREA
 Reduce physical and emotional stress
 Phenobarbital ( 15 – 30 mg every 6 to 8 hrs)
 Haloperidol ( 2 mg every 8 hrs )
 Valproic acid
 Chlorpromazine
 Diazepam
 Steroids
 Plasma exchange and IVIG

30. PROGNOSIS
 The more sever the cardiac involvement at the time
the patient first seen, greater the incidence of
residual heart disease.
 The severity of valvular involvement increases with
each recurrence.
 Valvular disease resolve more frequently when
prophylaxis is followed.

31. PREVENTION
 PRIMARY PREVENTION :
 10 days course of penicillin therapy for streptococcus
pharyngitis
 Not possible in all patients :
 30% patients have subclinical phryngitis
 30% patients developed rheumatic fever without
symptoms of streptococcal pharyngitis

32.  SECONDARY PREVENTION :
 Benzathine penicillin G 1.2 million units IM every 28
days
 Oral penicillin V 250 mg BD
 Oral sulfadiazine 1 g or sulfisoxazole 0.5 g daily
 Oral erythromycin ethyl succinate 250 mg BD

33.  Recommended duration of prophylaxis :
CATEGORY DURATION
Rheumatic fever without carditis At least 5 yrs or until 21 yrs, whichever is
longer
Rheumatic fever with carditis
But without residual heart disease
At least 10 yr or well into adulthood,
whichever is longer
Rheumatic fever with carditis
But with residual heart disease
At least 10 yr since last episode and at
least up to 40 yr , sometime lifelong

34. THANK YOU