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
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.
15.
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
17.
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
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
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
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