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ACUTE RHEUMATIC FEVER
DR. HOMAYRA AKHTER
D CARD STUDENT
DMCH
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.
INCIDENCE
 Military recruits
 School aged children
 Low socio-economic condition
 Aged 5 to 15 years
 Both sex (female= chorea)
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.
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
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.
JONES CRITERIA
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
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%).
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.
MANAGEMENT
Aim of management
1.Primary prevention(Eradication of GABHS infection)
2.Suppress inflammation
3.Provide supportive treatment
4. Secondary Prevention
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
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
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
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
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
SUPPORTIVE THERAPY
 Bed rest
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.
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.
“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.
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.
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
FATE OF ACUTE RHEUMATIC FEVER
 Full recovery
 Recurrence
 Chronic carditis even heart failure
 Rheumatic valvular heart disease
Acute rheumatic fever

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Acute rheumatic fever

  • 1. ACUTE RHEUMATIC FEVER DR. HOMAYRA AKHTER D CARD STUDENT DMCH
  • 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.
  • 8.
  • 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