rheumatic fever....

1. ACUTE RHEUMATIC FEVER
Dr kishore parthav
Asst.prof,Dept of General
medicine

2. Acute rheumatic fever is immunological
disorder intiated by group A beta hemolytic
streptococcus.
Antibodies produced against some
streptococcal proteins ,sugars react with
connective tissue and heart result in multisystem
disorder,ARF.
• It affects children and young adults ages of 5
and 15 years,peak age 8y.
• More common in developing countries.
• Most common cause of acquired heart disease
in childhood and adolescence.
• Latency period;2-6wks

3. Predisposing factors :
Poor nutrition
Poor hygiene
Low SES
Over crowding
Genetic factors:HLA-DR class 2.

4. Pathogenesis:
• Immune-mediated delayed response to infection
with specific (pharyngitis)strains of group A
streptococci, which have antigens that cross-
react with cardiac myosin and sarcolemmal
membrane proteins.
• Antibodies produced against the streptococcal
antigens cause inflammation in the
endocardium,myocardium and pericardium, as well
as the joints and skin.
• Hyaluronic acid capsule of streptococcus prevents
phagocytosis,NAG –cell wall component
participates in immunological process.
• Aschoff nodules are pathognomonic.Seen only in

5. Clinical features:
• Presents with fever, anorexia, lethargy and joint
pain, 2–3 weeks after an episode of
streptococcal pharyngitis. There may be no
history of sore throat.
• Acute rheumatic fever is a multisystem
disorder,Arthritis occurs in 75% of patients
.Other features include rashes, subcutaneous
nodules, carditis and neurological changes .
Revised Jones criteria used for diagnosis ;5
major ,4 minor and required criteria.
ARF Diagnosis is made by the presence of :
 One required criteria,two major criteria,and zero

6. Required criteria
• Evidence of streptococal infection (e.g:increased titer of anti-
streptococal antibodies [ASO,others];
• Positive throat culture for group A streptococus;
• Recent scarlet fever)
Major diagnosis criteria:
• Carditis
• Polyarthritis
• Chorea
• Erythema marginatum
• Subcutaneous nodules
Minor diagnostic criteria:
• Fever
• Arthralgia
• Previous rheumatic fever or rheumatic heart disease
• Acute phase reactions (ESR/CRP/leukocytosis)
• Prolonged PR interval

7. Carditis :
• Rheumatic fever causes a pancarditis
involving the endocardium,myocardium and
pericardium to varying degrees.
• Incidence more at younger age
• It manifest as breathlessness,palpitations or
chest pain and tachycardia, cardiac
enlargement,edema and new or changed
murmurs in established RHD
• Mostly Left side valves involved ,Mixed valvular
involvement more common than individual
valves.

8. • Mitral valve more commonly regurgitation than
stenosis
• A soft pansystolic murmur due to mitral
regurgitation
• Carey Coombs murmur-mid daistolic murmur
is typical seen in RHD ,due to mitral valvulitis.
• Aortic regurgitation occurs in 50% of cases
but the tricuspid and pulmonary valves are
rarely involved.
• Pericarditis present with severe precordial
pain,friction rub on auscultation
• ECG :ST and T wave changes. Conduction
defects, including AV block.

9. Arthritis
• Arthritis is most common major and early
manifestation and occurs early when
streptococcal antibody titres are high.
• Clinically this arthritis-Acute,painful
,asymmetric and migratory polyarthritis
,typically affects large joints knees, ankles,
elbows and wrists.
• The joints are red, swollen and tender for
between a day and 4 weeks
• Arthritis do not progress to chronic disease.
• Occurs in 80% of cases,

10. Erythema marginatum:
• Erythema marginatum occurs in less than 5% of
patients.
• The lesions start as red macules that fade in
the centre but remain red at the edges,non
pruritis and not raised above the skin.
• Sites mainly on the trunk and proximal
extremities but not the face. The resulting red
rings or ‘margins’ may coalesce or overlap .
• Rash disappears on exposure to cold and
reappers after hot shower.

11. Subcutaneous nodules:
• occur in 5–7% of patients.
• Nodules are firm,painless,mobile,non
pruritic and best felt over extensor surfaces
of bone or tendons,spine.
• They are small (0.5–2.0 cm),symmetrical single
or in clusters.
• They typically appear more than 3 weeks after
the onset of other manifestations and therefore
help to confirm rather than make the diagnosis.
• Strong association with severe carditis .

12. Sydenham’s chorea:
• Neuropsychiatric disorder , It occurs in up to one-
third of cases and is more common in females.
• Sydenham’s chorea, also known as St Vitus
dance, is a late neurological manifestation that
appears at least 3 months after the episode of
acute rheumatic fever.Chorea occurs due to
lesions in Basal ganglia.
• Emotional lability may be the first feature and is
typically followed by purposeless, involuntary,
Choreiform movements of the hands, feet or face.
• Neurologic –choric movements and hypotonia
• Psychiatric- Emotional lability
,hyperactivity,separation anxiety,obcessions and
compulsions.
• Speech may be explosive and halting.
Spontaneous recovery usually occurs within a few

13. • Carditis can cause permanent damage,
• Arthritis subsides within days to weeks without
rx
• Chorea subsides gradually takes 6-7months
Other features :
 Pleural effusion and pneumonia
 Anemia
 Epistaxis
 Abdominal pain

14. Investigations :
CBC
Acute phase reactants- CRP,ESR;for monitoring
progression of disease
ASO titres provides supportive evidence for
diagnosis,normal in one fifth cases
Throat culture- should be taken but positive
results seen in 10–25% of cases
Ecg – Prolonged PR interval,ST depression ,T
inversion,2,3rd heartb block.
2d Echo- Valves edema,LA,LV
dilatation,pericardial effusion,decreased
contractility.
Chest xray

15. D/D:
Juvenile RA
Collagen vascular diseases
Virus associated arthritis
Hematological disorders

16. Treatment :
Bed rest
Aspirin :
Gives symptomatic relief of pain and confirms diagnosis.
60-80mg/kg per day in divided doses for arthritis for 2-3
weeks and
4-6 weeks for carditis.
Antibiotics :
• A single dose of benzathine benzylpenicillin (1.2
million U IM) or oral phenoxymethylpenicillin250 mg 4
times daily for 10 days)after diagnosis .
• If the patient is penicillin-allergic, erythromycin or a
cephalosporin can be used
Heart failure drugs –Furosemide ,digoxin..
Glucocorticoids- When aspirin fails and severe carditis
present.Prednisolone 2mg/kg in divided doses then

17. Long-term prophylaxis :
• INJ.benzathine benzylpenicillin (1.2 million U
IM monthly), oral phenoxymethylpenicillin (250
mg twice daily)
• If the patient is allergic to penicillin; Sulfadiazine
1g daily or erythromycin 250mg BD may be
used.

18. Duration of prophylaxis:
1) Rheumatic fever without carditis- Atleast 5 yrs
or until 21 years,whichever is longer
2) Rheumatic fever with carditis-At least 10 yrs or
until 21 yrs ,whichever is longer
3) Rheumatic fever with carditis but with residual
heart disease-At least 10 yrs since last
episode and at least upto 40 yrs ,sometime
lifelong

19. PROGNOSIS :
• The more severe 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.

20. THANK YOU