2. DEFINITION
New Zealand Guidelines for Rheumatic Fever (UPDATE 2014)
Acute rheumatic fever (ARF) is thought to be
an auto-immune consequence of infection
with the bacterium group A streptococcus (GAS).
It causes an acute generalized inflammatory response and
an illness
that affects only certain parts of the body, mainly the heart, joints, brain and
skin.
3. INCIDENCE
Military recruits
School aged children
Low socio-economic condition
Aged 5 to 15 years
Both sex (female= chorea)
4. CONT….
There is association between the presence of specific HLA markers
and susceptibility to acute rheumatic fever.
In first half century decreased incidence of RF due to
Improve public health
Living condition
Developed modern antibiotic.
5. PATHOPHISIOLOGY
2 types- immunologic response and genetic
predisposition
History of GAS tonsillo-pharyngitis/ scarlet fever 3 wks
before
About 30% of patients with acute rheumatic fever do not
recall a preceding episode of pharyngitis.
Why not present before 3 years?
GAS infection = 3% dev RF
50% Recurrent RF dev
6. DIAGNOSIS
Importance of Accurate Diagnosis
It is important that an accurate diagnosis of ARF is made as:
Over-diagnosis will result in the individual receiving benzathine penicillin
injections unnecessarily every four weeks for a minimum of ten years
Under-diagnosis of ARF may lead to the individual suffering a further attack
of ARF, cardiac
damage and premature death.
9. Cont…
Exception in Jones criteria: when diagnosis of RF can be
made without strict adherence to Jones criteria
Indolent carditis( carditis is acute onset and slow
progression) or recurrent carditis.
Isolated chorea
10. Cont…..
Most common major manifestations during the
first episode of ARF remain:
Carditis (50%–70%)
Arthritis (35%–66%)
Chorea (10%–30%)
Subcutaneous nodules (0%–10%)
Erythema marginatum (<6%).
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16. INVESTIGATION
Evidence of Preceding Streptococcal Infection
1.Increased or rising anti-streptolysin O titer or other streptococcal antibodies
(anti-DNASE B). A rise in titer is better evidence than a single titer result.
2. A positive throat culture for group A β-hemolytic
streptococci.
3. A positive rapid group A streptococcal carbohydrate
antigen test in a child.
17. MANAGEMENT
Aim of management
1.Primary prevention(Eradication of GABHS infection)
2.Suppress inflammation
3.Provide supportive treatment
4. Secondary Prevention
18.
19. Cont…
For patients allergic to penicillin
1.Narrow-spectrum cephalosporin (cephalexin ,cefadroxil)
Varies dosage and duration.
2.Azithromycin-
12 mg per kg (maximum, 500 mg) orally once daily for 5 days
3.Clarithromycin
15 mg per kg orally per day, divided into 2 doses (maximum, 250 mg twice
daily), for 10 days
4.Clindamycin
20 mg per kg orally per day (maximum, 1.8 g per day), divided into 3 doses,
for 10 days
20. Anti-inflammatory Therapy:
Anti-inflammatory agents (salicylates, corticosteroids)
should be withheld if arthralgia or atypical arthritis is the only
clinical manifestation of presumed acute rheumatic fever
Acetaminophen can be used
Patients with typical migratory polyarthritis & carditis
without cardiomegaly or congestive heart failure:
treatment with oral salicylates,100 mg/kg/day in 4 divided
doses PO for 3-5 days, followed by 75 mg/kg/day in 4
divided doses PO for 4-8 wk
21. Cont…
Patients with carditis & cardiomegaly or congestive heart
failure:
treatment with corticosteroids
Prednisone 2 mg/kg/day in 4 divided doses for 2-6 wk
followed by a tapering of the dose that reduces the dose by
5 mg/24 hr every 2-3 days. At the beginning of the tapering of
the prednisone dose, aspirin should be started at
75 mg/kg/day in 4 divided doses to complete 12 wk of
therapy
22. Cont…
Supportive therapies for patients with moderate to severe
carditis include digoxin, fluid & salt restriction, diuretics &
oxygen
The cardiac toxicity of digoxin is enhanced with myocarditis
23. Sydenham Chorea
Occurs after the resolution of the acute phase of the disease
Anti-inflammatory agents are usually not indicated
Sedatives: phenobarbital (16-32 mg every 6-8 hr PO) is the
drug of choice
If phenobarbital is ineffective, then haloperidol (0.01-
0.03 mg/kg/24 hr divided bid PO) or chlorpromazine
(0.5 mg/kg every 4-6 hr PO) should be initiated
Long-term antibiotic prophylaxis
25. SECONDARY PREVENTION
Who should receive prophylaxis?
Patients with documented history of rheumatic fever,
Including those with isolated chorea & those without
evidence of rheumatic heart disease MUST receive
prophylaxis.
Why should receive prophylaxis ?
1.Prevent GABHS infection sequele .
2.Prevent the repeated development of ARF.
3.Prevent the development of RHD.
4.Reduce the severity of RHD.
5. Reduce the risk of death from severe RHD.
26.
27. How long ?
CATEGORY Duration after last attack
Rheumatic fever without carditis At least for 5 yr or until age 21 year,
whichever is longer.
Rheumatic fever with carditis but
without residual heart disease (no
valvular disease)
At least 10 yr or until age 21 year ,
whichever is longer.
Rheumatic fever with carditis &
residual heart disease (persistent
valvular disease)
At least 10 yr or until age 40 yr,
whichever is longer; sometime
lifelong.
28. “Possible” Rheumatic Fever
A given clinical presentation may not fulfill these
updated Jones criteria, but the clinician may still have good
reason to suspect that ARF is the diagnosis. This may occur
in high-incidence settings.
In such situations, clinicians should use their
discretion and clinical acumen to make the diagnosis that
they consider most likely and manage the patient
accordingly.
29. AHA recommendations for management of
''possible'‘rheumatic fever are:
1. In genuine uncertainty, 12 months of secondary prophylaxis
followed by reevaluation including careful history and
physical examination with repeat echo.
2. In recurrent symptoms (particularly involving the joints) who
has been adherent to prophylaxis recommendations but lacks
serological evidence of group A streptococcal infection and
lacks echo evidence of valvulitis, it is reasonable to conclude
that the recurrent symptoms are not likely related to ARF, and
discontinuation of antibiotic prophylaxis may be appropriate.
30. Secondary prevention: Adherence
How can we reduce the pain associated with IM Penicillin?
Use a 23-gauge needle- deeper is better
Local pressure to area for 10 secs
Warm syringe to room temperature
First allow alcohol to dry or use ethylchloride spray
Deliver injection very slowly(over 2-3mins)
Distraction techniques
Good rapport with the case, is a significant aid to injection comfort,
compliance and understanding
Use 0.5-1ml of 1% lignocaine. Reduces pain significantly and excellent
for younger patients
31. FATE OF ACUTE RHEUMATIC FEVER
Full recovery
Recurrence
Chronic carditis even heart failure
Rheumatic valvular heart disease