Diagnosis and management of acute Rheumatic fever

1. Presented by
Dr. Enas Fathy
Lecurer of cardiovascular medicine
Aswan university

2.  Defintion
 Etiology
 Pathophysiology
 Diagnosis
 Differential diagnosis
 management
 Prevention

3.  Acute rheumatic fever (ARF) is an
autoimmune inflammatory process that
develops as a sequela of streptococcal
infection.
 Typically following 2 to 3 weeks after group
A streptococcal pharyngitis
 occurs most commonly in children and has
rheumatologic, cardiac, and neurologic
manifestations

4.  The incidence of ARF has declined markedly
in the past 50 years in both the United States
and Western Europe
 Incidence of ARF in India is varies from
0.42–10.9 per 1,000 population
most common in children from 5 to 15
years of age
 Female > Male
 Cardiac involvement : about 60% of RF cases

5.  cytotoxicity theory :
- GAS toxin is involved in the pathogenesis of
acute rheumatic fever and rheumatic heart
disease.
- Streptolysin O has a direct cytotoxic effect
on mammalian cells in tissue culture
 Immune-mediated theory (molecular mimicry) :
- The antigenicity of GAS cellular and extracellular
epitopes and their immunologic cross reactivity
with cardiac antigenic epitopes.

7.  Family history of rheumatic fever
 Low socioeconomic status (poverty, poor hygiene,
medical deprivation)
 Age: 6-15 years

8.  Inflmmatory disorder that usually presents
with
–Fever
–Anorexia
–Lethargy
–Joint pain
 Latent period: 2–3 weeks after an episode of
streptococcal pharyngitis.
 Revised Duckett Jones criteria.

11.  MAJOR MANIFESTATIONS :
• Carditis
• Polyarthritis
• Erythema marginatum
• Subcutaneous nodules
• Chorea

12.  MINOR MANIFESTATIONS :
 Clinical features:
- Arthralgia
- Fever
 Laboratory features:
• Elevated acute phase reactants:
- ESR
- C-reactive protein
• Prolonged P-R interval

13.  SUPPORTING EVIDENCE OF ANTECEDENT
GAS INFECTION :
 Positive throat culture or
 Rapid streptococcal antigen test or
 Elevated or increasing streptococcal antibody
titer.

14.  Initial attack of ARF :
 2 major manifestations or
 1 major and 2 minor manifestations
 plus evidence of recent GAS infection.
o Recurrent attack of ARF :
 2 major manifestations or
 1 major and 2 minor or
 3 minor manifestations (in the Moderate/High-Risk
population)
 plus evidence of recent GAS infection.

16.  when chorea occurs as the only major
manifestation of acute rheumatic fever.
 when indolent carditis is the only
manifestation in patients who first come to
medical attention only months after the
apparent onset of acute rheumatic fever.

In recurrences of acute rheumatic fever in
high risk populations.

17.  Most common and early manifestation.
 Occurs in 75% of patients
 Acute painful asymmetric and migratory
inflammation of the large joints
 Typically affects the knees, ankles, elbows and
wrists.
 Pain characteristically responds to aspirin
If not, the diagnosis is in doubt
 typically not deforming

18.  Carditis occurs in approximately 50-60% of all
cases of ARF.
 Involves the endocardium, myocardium and
pericardium.
 Incidence declines with increasing age – ranging
 from 90% at 3 years to around 30% in
adolescence.
 Account for essentially all of the associated
morbidity and mortality

19.  Acute rheumatic carditis:
1- tachycardia out of proportion to fever &
cardiac murmurs, with or without
evidence of myocardial or pericardial
involvement
2- carey coombs murmer
3- Prolonged PR interval is an early sign
4- Arrythmias

20.  Occurs in < 5% of patients
 Lesions start as red
macules that fade in the
centre but remain red at
the edges.
 Occur mainly on the trunk
and proximal extremities
but not the face.

21.  Occur in 5–7% of patients.
Small (0.5–2.0 cm), firm and
painless.
 Best felt over extensor
surfaces of bone or tendons.
 Appear more than 3 weeks
after group A streptococcal
pharyngeal infection.
 A correlation between the presence
of these nodules & significant
rheumatic heart disease

22.  Late neurological manifestation.
 Appears at least 3 months after the episode of ARF.
 all the other signs may have disappeared.
 Occurs in up to 1/3rd of cases more common in
females.
 Begins with emotional lability & personality
changes (poor school performance)

23.  Typically followed by purposeless,involuntary
choreiform movements of the hands, feet or
face, hypotonia ( last 4-8 months)
 Speech may be explosive and halting.
 Spontaneous recovery usually occurs within a
few months
 Exacerbate by stress, disappear during sleep
 milkmaid's grip, spooning and pronation of the
hands, wormian darting tongue, hand writing

26.  Throat swab culture
 Antistreptolysin O
 Anti–dnase
 CXR
 ECG
 2D ECHO
 CBC
 ESR
 CRP

27.  Infective Endocarditis
 Juvenile Idiopathic Arthritis
 Mixed Connective-Tissue Disease
 Reactive Arthritis
 Rheumatoid Arthritis
 Kawasaki Disease
 Septic Arthritis

28.  Bed rest and monitored closely for evidence of
carditis.
 Antibiotic Therapy :
 Single dose of benzathine penicillin 1.2 mU I.M.
 Oral phenoxymethylpenicillin 250 mg 6-hourly for
10 days.
 Oral amoxycillin for 10 days.
Penicillin-allergic:

Erythromycin -10 days.
 azithromycin - 5 day

29.  Migratory polyarthritis :
 Aspirin is drug of choice.
 50-70 mg/kg/day in 4 divided doses PO for 3-
5 days.
 50 mg/kg/day in 4 divided doses PO for 3 weeks
 Half that dose for another 2-4 weeks.

30.  Carditis :
 prednisone is drug of choice.
2 mg/kg/day in 4 divided doses for 2-3 weeks
 1 mg/kg/day for 2-3 weeks
 tapering of the dose by 5 mg/day every 2-3 days.
 When prednisone is being tapered, aspirin should be started at 50
mg/kg/day in 4 divided doses for 6 wk to prevent rebound of
inflammation

31.  SYDENHAM CHOREA
 Phenobarbital is the drug of choice.
 16-32 mg every 6-8 hr PO.
 If phenobarbital is ineffective :
 Haloperidol (0.01-0.03 mg/ kg/24 hr divided bid
PO) OR
 Chlorpromazine (0.5 mg/kg every4-6 hr PO).

32.  The arthritis and chorea of acute rheumatic
fever resolve completely without sequelae.
 The long-term sequelae of rheumatic fever is
RHD.

33.  Primary prevention
Appropriate antibiotic therapy instituted before the 9th
day of symptoms of acute GAS pharyngitis is highly
effective in preventing first attacks of acute rheumatic
fever

34.  Secondary prevention :
 prevention of recurrent attacks of RF.
1- Benzathine penicillin G
1.2 million units IM every 3 weeks if wt > 27kg
0.6 million units IM every 3 weeks if wt < 27 kg
2- Penicillin V 250 mg twice daily orally.
3- If allergic to both – Erythromycin 250 mg twice
daily orally

35.  Rheumatic fever
without carditis
 Rheumatic fever with
carditis but without
residual heart disease
(no valvular disease)
 Rheumatic fever with
carditis and residual
heart disease
(persistent valvular disease
 5 yr or until 21 yr of age,
whichever is longer
10 yr or until 21 yr of age,
whichever is longer
10 yr or until 40 yr of age,
whichever is longer
OR lifelong prophylaxis

36. Thank you