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Rheumatic Fever
Criteria for Diagnosis :
From : 1944 to 2020
ARF
( Acute Rheumatic Fever )
Safwat EL-ARABY M.D.
Rheumatology
EGYPT
Is available Criteria valid for :
High & Low
Risk Areas & Populations
Before Diagnosis :
Is manifestations of ARF are the same for :
Boys & Girls
3 / 5 / 7 / 9 / 11 / 13 / > 16 years
North & South countries
Developed & Developing countries
Crowded localities
Race : Chinese / Egyptian / European / African
This phenomenon ( of Variation ) is relevant :
Why ?
because there are
No specific diagnostic tests for ARF, and a
Diagnostic mistake has severe consequences.
Diagnosis
‫المتابينة‬ ‫االمراض‬
:
‫طبية‬ ‫معضلة‬
‫متباينة‬ ‫الروماتيزمية‬ ‫االمراض‬ ‫من‬ ‫الكثير‬ ‫هناك‬
:
‫والمخرج‬ ‫والمسار‬ ‫البداية‬ ‫متعددة‬
‫وعالجية‬ ‫تشخيصية‬ ‫معضلة‬ ‫هناك‬ ‫اذا‬
:
‫موحدة‬ ‫عالجية‬ ‫استراتيجية‬ ‫او‬ ‫موحدة‬ ‫تشخيصية‬ ‫معايير‬ ‫ايجاد‬ ‫العسير‬ ‫من‬ ‫سيكون‬
The classic Jones criteria was first published
in 1944
It is revised in 1956
1965
1992.
In 2004,
the WHO published an expert consultation report that included ARF criteria based on the previous revised Jones criteria
Diagnosis
‫المتابعة‬ ‫من‬ ‫فائدة‬ ‫هناك‬ ‫هل‬
:
‫اخرى‬ ‫الى‬ ‫مرضية‬ ‫مجموعة‬ ‫ومن‬ ‫الخر‬ ‫مكان‬ ‫من‬ ‫تتكرر‬ ‫ربما‬ ‫التى‬ ‫ظروفها‬ ‫ولها‬ ‫توقيتها‬ ‫لها‬ ‫معايير‬ ‫كل‬ ‫نعم‬
ARA
Revised Criteria :
1992
2015
2018
Diagnosis
Each criterion modification has resulted in :
More Specific and
Less Sensitive criteria,
Is it good ?
Yes & No
It is appropriate for most of the developed world
but
Not for Hot Spots ( High Risk Areas )
‫الهادئه‬ ‫لبالدهم‬ ‫مناسبة‬ ‫النها‬ ‫واالمريكية‬ ‫االوربية‬ ‫المعايير‬ ‫على‬ ‫كليا‬ ‫نعتمد‬ ‫اال‬ ‫يجب‬ ‫لذلك‬
‫وذاك‬ ‫هذا‬ ‫من‬ ‫خليط‬ ‫النها‬ ‫العالمية‬ ‫الصحة‬ ‫منظمة‬ ‫وايضا‬
‫وجنسنا‬ ‫ومنطقتنا‬ ‫وظروفنا‬ ‫باطفالنا‬ ‫الخاصة‬ ‫المعايير‬ ‫الى‬ ‫وانتظارا‬
:
‫مظاهر‬ ‫باقى‬ ‫من‬ ‫بنا‬ ‫خاصة‬ ‫اخرى‬ ‫ابعاد‬ ‫واضافة‬ ‫المعتمدة‬ ‫بالمعايير‬ ‫االستعانة‬ ‫يجب‬
‫المصرية‬ ‫والخبرة‬ ‫المرض‬
Jones Criteria
Original
‫اكتشف‬ ‫قد‬ ‫البنسلين‬ ‫يكن‬ ‫لم‬
‫المتابعة‬ ‫من‬ ‫فائدة‬ ‫هناك‬ ‫هل‬
:
‫اخرى‬ ‫الى‬ ‫مرضية‬ ‫مجموعة‬ ‫ومن‬ ‫الخر‬ ‫مكان‬ ‫من‬ ‫تتكرر‬ ‫ربما‬ ‫التى‬ ‫ظروفها‬ ‫ولها‬ ‫توقيتها‬ ‫لها‬ ‫معايير‬ ‫كل‬ ‫نعم‬
Add
It is now : Gate / Base / To categorize
If supported by preceding GAS infection
The presence of 2 major OR 1 major & 2 minor manifestations indicates a high probability of ARF
Add
ASO Titre
RA Test
Pit falls of Jones Criteria
1- It is difficult to diagnose ARF when Carditis is the only manifestation of the disease particularly in a recurrence
2- When patient has sub-clinical Carditis the clinicians fail to detect clinically
4- When previous Cardiac status is unknown , it is not possible to know in a new case whether the findings are due to :
Acute Carditis OR it is recrudescence OR it is established old case of RHD
3- Clinically apparent Carditis is present but supportive minor criteria are not fulfilled
5- In case of Polyarthralgia , which is a minor criterion , if the patient is neglected & not evaluated for ARF , they would go undiagnosed , & could
end up with RHD , allegedly without any past history of ARF : as the patient & the parents would have long forgotten the joint pain
AHA Criteria
(American Heat Association )
Diagnostic criteria for rheumatic fever – modified 1992 Jones criteria
( By AHA = American Heart Association )
Major criteria Minor criteria
Carditis
Arthritis
Chorea
Erythema marginatum
Subcutaneous nodules
Hyperpyrexia
Arthralgia, without other signs of inflammation
Laboratory indicators of acute phase:
ESR, CRP
Prolonged PR interval in ECG
And Evidence of antecedent streptococcal infection
– Throat Swab Culture or Rapid Antigen Test
– Elevated / increasing ASO antibody titer in serum
Jones Criteria ( Revised ) For Guidance in the diagnosis of RF
Clinical Laboratory
Major manifestations Minor manifestations Supporting Evidence of Strept. infection
Recommendations of the American Heart Associations
The presence of Two Major OR of One Major & Two Minor Criteria :
Indicates a high Probability of ARF : If Supported by evidence of GASBH infection
5
4
4
3
‫التوقيت‬ ‫هذا‬ ‫حتى‬
:
‫كانت‬ ‫ان‬ ‫وبعد‬ ‫المعيشة‬ ‫مستوى‬ ‫وتقدم‬ ‫البنسلين‬ ‫اكتشاف‬ ‫بعد‬ ‫ان‬ ‫االعتبار‬ ‫فى‬ ‫يؤخذ‬ ‫لم‬
‫العالم‬ ‫مستوى‬ ‫على‬ ‫منتشرة‬ ‫والحمرا‬ ‫الروماتيزمية‬ ‫الحمى‬
‫خطورة‬ ‫االقل‬ ‫المناطق‬ ‫وسميت‬ ‫المرض‬ ‫بها‬ ‫يندر‬ ‫وشعوب‬ ‫مناطق‬ ‫هناك‬ ‫اصبح‬
‫عالية‬ ‫مخاطرة‬ ‫ذات‬ ‫وشعوب‬ ‫ومناطق‬
-------------------------------------------------------------------------------------------------------
‫حسب‬ ‫على‬ ‫الروماتيزمية‬ ‫للحمى‬ ‫التشخيصية‬ ‫المعايير‬ ‫حسابات‬ ‫واعادة‬ ‫وتطوير‬ ‫تعديل‬ ‫الصواب‬ ‫من‬ ‫كان‬ ‫لذلك‬
‫الشعب‬ ‫وهذا‬ ‫البلد‬ ‫لهذا‬ ‫االصابة‬ ‫معدل‬
(
‫المرض‬ ‫من‬ ‫الوفيات‬ ‫ومعدل‬ ‫القلب‬ ‫واصابات‬ ‫الوماتيزمية‬ ‫الحمى‬ ‫اصابات‬ ‫معدل‬
)
-------------------------------------------------------------------------------------------------------
‫صور‬ ‫الختالف‬ ‫نظرا‬ ‫بها‬ ‫خاصة‬ ‫معايير‬ ‫وضعت‬ ‫قد‬ ‫البالد‬ ‫بعض‬ ‫ان‬ ‫الى‬ ‫الخصوصية‬ ‫من‬ ‫لمزيد‬ ‫االمر‬ ‫وصل‬ ‫بل‬
‫مواطنيها‬ ‫حماية‬ ‫من‬ ‫ولمزيد‬ ‫اخر‬ ‫الى‬ ‫شعب‬ ‫ومن‬ ‫اخر‬ ‫الى‬ ‫بلد‬ ‫من‬ ‫المرض‬ ‫ومسارات‬
(
‫ونيوزيالندا‬ ‫استراليا‬
)
‫للقلب‬ ‫االمريكية‬ ‫المنظمة‬ ‫فعلت‬ ‫هكذا‬
:
‫خطورة‬ ‫االقل‬ ‫والبالد‬ ‫المخاطر‬ ‫عالية‬ ‫للبالد‬ ‫معايير‬ ‫بوضع‬
Diagnostic criteria for RF ( AHA ) – modified 2015 Jones criteria
Major criteria
Low Risk Population High Risk Population
Carditis (clinical or subclinical)
Arthritis – only Polyarthritis
Chorea
Erythema marginatum
Subcutaneous nodules
Carditis (clinical or subclinical)
Arthritis : Monoarthritis or
Polyarthritis
Poly arthralgia
Chorea
Erythema marginatum
Subcutaneous nodules
Minor criteria
Low Risk Population High Risk Population
Poly arthralgia
Hyperpyrexia (≥ 38.5ºC)
ESR ≥ 60 mm/h and/or CRP ≥ 3.0 mg/dl
Prolonged PR interval (after taking into account the differences related to age; if
there is no carditis as a major criterion)
Mono arthralgia
Hyperpyrexia (≥ 38.0ºC)
ESR ≥ 30 mm/h and/or CRP ≥ 3.0 mg/dl
Prolonged PR interval (after taking into account the differences related to age; if
there is no carditis as a major criterion)
5
4
USA / EUROPE India / AU / NZ
Subclinical carditis :
No Symptoms / No Signs
Only ECHO (Doppler) reveals mitral or aortic valve pathology
The First Episode of the disease
Diagnosis of RF : Evidence of antecedent AGSBH infection
Confirmation with : 2 major or 1 major & 2 minor criteria –
The Subsequent Episodes of the disease
requires a confirmation of 2 major or 1 major & 2 minor or 3 minor criteria
ECHO
It is currently the main diagnostic tool
For Confirmation, Diagnosis and Monitoring of valvular lesions in the course of RF, especially in subclinical carditis.
1. In the major criteria :
1. Low risk population: clinical and/or subclinical carditis. AHA recommends that all the patients with suspected RF undergo Doppler ECHO
examination, even if no clinical signs of carditis are present
In doubtful cases it is recommended that ECHO is repeated.
2- Medium and High risk population: also clinical and/or subclinical carditis and arthritis – Monoarthritis or Polyarthritis, possibly also with
PolyArthralgia
2- In the minor criteria:
1. Low risk population : the parameters of inflammation and the level of fever were defined precisely.
2. Medium and High risk population : Monoarthralgia, also with defined parameters of inflammation and the level of fever.
The modifications introduced in 2015 in the Jones criteria are as follows:
(RF) is an autoimmune disease associated with GASBH infection, in the course of which the patient develops Carditis, Arthritis, Chorea, SC nodules and EM
RF diagnosis is based on the Jones criteria, developed in 1944, then revised twice by the American Heart Association (AHA), in 1992 and recently in 2015.
The last revision of the Jones criteria consists mainly in the supplementation of the major criteria with ECHO examination, the introduction of a concept of
Subclinical Carditis and the isolation of : Low, Medium and High Risk Populations
AHA recommends : that all the patients with suspected RF undergo Doppler ECHO after the Jones criteria have been verified, even if no clinical signs of carditis
are present.
Diagnosis of RF ; 2018
RF can also be diagnosed if the Jones criteria are not met, in the case of isolated Chorea or Carditis with an :
insidious onset, long-term course and indistinct progression of lesions, after other causes have been excluded.
A Low Risk Population : is one in which cases of
ARF occur in ≤ 2/100 000 school-age children or
RHD is diagnosed in ≤ 1/1000 patients at any age during one year
Review :
Situational Therapy
Add to your Situations
The Guideline Recommendations
For diagnosing Rheumatic Fever Recurrences are :
With a reliable past history of ARF
OR
established RHD & in the face of GAS infection
2 Major OR 1 Major & 2 Minor OR 3 Minor manifestations
may be sufficient for a presumptive diagnosis
If No Major : Only Minors :
It is recommended : Exclusion of other more likely causes of the clinical presentation before a diagnosis of an ARF recurrence is made
( Possible ) Rheumatic Fever
In some circumstances , 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 the clinicians should use their discretion & clinical acumen to make the diagnosis that they consider most likely &
manage the patient accordingly
Clinical sense should be respected
Wakefulness / Watchfulness / Clinical smell
( Possible ) Rheumatic Fever
1- Where there is Genuine Uncertainty
------------------------------------------------------------------------------
It is reasonable to consider offering 12 months of 2ry prophylaxis
Followed by : Reevaluation ( Careful History & examination + ECHO )
2- If recurrent symptoms ( particularly joints ) + adherent to prophylaxis + lacks serological evidence of GAS infection + lacks ECHO
evidence of valvulitis ,
-----------------------------------------------------------------------------------------------------------------------
it is reasonable to exclude ARF & Discontinue prophylaxis Antibiotics
To avoid lack of
sensitivity
To fill pitfalls
More addition to your
situations
For more situational
Therapy
WHO Criteria
* Major manifestations
Carditis, Polyarthritis, Chorea, Erythema Marginatum, SC Nodules
** Minor manifestations
Clinical : Fever, Polyarthralgia;
Laboratory findings : elevated acute-phase reactants (ESR or WBC count)
*** Supporting evidence of :
ECG : Prolonged PR interval ;
Preceding streptococcal infection ;
Elevated or rising ASO or streptococcal infection with another streptococcal Ab ;
Infection in the past 45 days ;
Positive throat culture ;
Rapid antigen test for group A streptococci ;
Recent Scarlet Fever.
2004 WHO Criteria for the Diagnosis of Rheumatic Fever and Rheumatic Heart Disease
WHO Criteria 2004
For More Sensitivity
5
4
7
Primary episode of RF
2 Major*
OR
1 Major and 2 Minor** manifestations Plus
evidence of a preceding group A streptococcal infection***
Recurrent attack of RF in a patient without established RHD
2 Major OR
1 Major and 2 Minor manifestations Plus
evidence of a preceding group A streptococcal infection
Recurrent attack of RF in a patient with established RHD
2 Minor manifestations Plus
evidence of a preceding group A streptococcal infection c
2004 WHO Criteria for the Diagnosis of RF and RHD
WHO Criteria
Diagnostic categories
Criteria
More addition to your situations
For more situational Therapy
‫سابق‬ ‫روماتيزمى‬ ‫قلب‬ ‫وجود‬
=
‫كبير‬ ‫معيار‬
1
2
3
Rheumatic chorea and insidious onset of rheumatic carditis
Other major manifestations OR
evidence of group A streptococcal infection are not required
Chronic valve lesions of RHD
Patients presenting for the first time with Pure mitral stenosis or mixed mitral valve disease and/or aortic valve)
Do not require any other criteria to be diagnosed as having RHD
2004 WHO Criteria for the Diagnosis of RF and RHD
WHO Criteria
More addition to your situations
For more situational Therapy
4
5
Patients may present with polyarthritis (or with only polyarthralgia or monarthritis) and with several (three or more) other minor
manifestations, together with evidence of recent GAS infection.
Some of these cases may Later turn out to be RF
It is wise to consider them as cases of “Probable RF”
(once other diagnoses are excluded) and advise regular secondary prophylaxis.
Such patients require close follow-up and regular examination of the heart.
This cautious approach is particularly suitable for patients in vulnerable age groups in high-incidence settings.
Some patients with recurrent attacks may not fulfill these criteria.
Congenital heart disease should be excluded.
2004 WHO Criteria for the Diagnosis of RF and RHD
WHO Criteria
6
7
8
Criteria for
High Risk Areas
Specificity
Sensitivity
Specificity
Sensitivity
High Risk Area
High Risk Population
Low Risk Area
Low Risk Population
f6-clep-3-067: Map showing reported worldwide prevalence of RHD from 1970 through 1990.14,21,86,90,99,102–104,111,112,118,128,132,136,201–216 (1) Barbados,104 (2) Cook Islands,21
(3) El Salvador,202 (4) Fiji,112 (5) French Polynesia,112 (6) Jamaica,202 (7) Puerto Rico,103 (8) Samoa,112 (9) Tonga,112,136 (10) Minnesota.86
Rheumatic Heart Disease : 1970 ; 1990
Map showing reported worldwide prevalence of RHD from 1991 through present.
2-6,21,111,160,168,169,176,177,189,191,192,197,217-240 (1) Fiji,169,228 (2) Samoa,192 (3) Tonga.3
Why ?
NZ & AU
Rheumatic Heart Disease : 1990 trough Present
File:Rheumatic heart disease world map-
Deaths per million persons-
WHO : 2012
NZ &
AU
RHD : Deaths/ Million persons : WHO : 2012
Why specific Criteria ?
NZ & AU
Use of Echocardiograms for the diagnosis of carditis
Aseptic monoarthritis as a major criterion
( provided NSAIDs are used in NZ and only for high-risk individuals in AU )
Use Polyarthralgia as a major criterion for high risk individuals (AU)
Use Monoarthralgia as a minor criterion for high–risk individuals (AU)
Diagnosis
These guidelines may be more applicable to high risk populations
Utilization of NZ guidelines for the diagnosis of ARF result in a modest increase (16%) in cases classified as definite
ARF compared with (AHA) 1992 Jones criteria.
‫الحاالت‬ ‫من‬ ‫عدد‬ ‫اكبر‬ ‫التقاط‬ ‫وبالتالى‬ ‫معاييرهم‬ ‫حساسية‬ ‫من‬ ‫ليرفعوا‬ ‫القوم‬ ‫هؤالء‬ ‫اليها‬ ‫لجأ‬ ‫التى‬ ‫واالدوات‬ ‫الوسائل‬ ‫هى‬ ‫ما‬
NZ & AU have developed their own criteria ,
the main particularities of which are :
Result
‫الحاالت‬ ‫معظم‬ ‫التقاط‬ ‫ثم‬ ‫ومن‬ ‫المعايير‬ ‫بزيادة‬ ‫وذلك‬ ‫الحساسية‬ ‫رفع‬ ‫الصواب‬ ‫من‬ ‫هل‬
‫احصائيا‬
:
‫الحساسية‬ ‫زيادة‬
:
‫التخصصية‬ ‫نقص‬ ‫يتبعه‬
:
‫العالية‬ ‫الحساسية‬ ‫ذات‬ ‫المعايير‬ ‫هذه‬ ‫ستلتقط‬ ‫اذا‬
:
‫الخاطئة‬ ‫الحاالت‬ ‫من‬ ‫مزيد‬
‫مثال‬
:
‫الحساسية‬ ‫كانت‬ ‫اذا‬
50
: %
‫فقط‬ ‫ستلتقط‬ ‫اذا‬
50
%
‫الصحيحية‬ ‫الحاالت‬ ‫من‬
:
‫خاطئة‬ ‫حاالت‬ ‫هناك‬ ‫يكون‬ ‫ال‬ ‫ربما‬
:
‫عالية‬ ‫التخصصية‬
‫التخصيصية‬ ‫رفع‬
:
‫العالج‬ ‫الى‬ ‫تحتاج‬ ‫التى‬ ‫الحاالت‬ ‫بعض‬ ‫سنفقد‬ ‫ولكن‬ ‫فقط‬ ‫الصحيحة‬ ‫الحاالت‬ ‫التقاط‬ ‫الى‬ ‫سيؤدى‬
‫الى‬ ‫الحساسية‬ ‫رفع‬ ‫اردت‬ ‫اذا‬
95
%
‫والتقاط‬
95
%
‫الحاالت‬ ‫من‬
:
‫معهم‬ ‫ستلتقط‬
10
‫خاطئة‬ ‫مثال‬ ‫حاالت‬
‫اليه‬ ‫حاجة‬ ‫فى‬ ‫ليسوا‬ ‫عالج‬ ‫سيتلقون‬ ‫العشرة‬ ‫وهؤالء‬
:
‫تقديرى‬ ‫االمر‬ ‫هنا‬
‫االمر‬ ‫هذا‬ ‫مع‬ ‫تعامل‬
:
‫الصحية‬ ‫وحالته‬ ‫مجتمع‬ ‫عن‬ ‫مسئول‬ ‫كطبيب‬ ‫مرة‬
:
‫الطفل‬ ‫لهذا‬ ‫امر‬ ‫كولى‬ ‫ومرة‬
‫امر‬ ‫ولى‬ ‫كنت‬ ‫واذا‬
:
‫موقفان‬ ‫هناك‬
:
‫المناسب‬ ‫العالج‬ ‫يتلقى‬ ‫ولم‬ ‫المعايير‬ ‫من‬ ‫سقط‬ ‫طفل‬ ‫امر‬ ‫ولى‬ ‫مرة‬
:
‫لديه‬ ‫يوجد‬ ‫ال‬ ‫طفل‬ ‫امر‬ ‫ولى‬ ‫ومرة‬
‫مدمرة‬ ‫وصمامات‬ ‫روماتيزمى‬ ‫قلب‬ ‫ماساة‬ ‫من‬ ‫اخر‬ ‫طفل‬ ‫حما‬ ‫وقد‬ ‫المفعول‬ ‫طويل‬ ‫بنسلين‬ ‫ويتلقى‬ ‫مرض‬
‫الساخنة‬ ‫للمناطق‬ ‫وخاصة‬ ‫بحثها‬ ‫يجب‬ ‫خالف‬ ‫نقاط‬
2020 Updated Australian Criteria for ARF diagnosis
High Risk Group :
Communities with high rate of
ARF : incidence
> 30/100000 / year OR
RHD > 2/1000 / year
For more Situations
Definite : Initial Definite : Recurrence
Probable : initial Probable : Recurrence
Possible : Recurrence
Possible : initial
For recurrent episode : > 90 days from previous episode of ARF
Is Polyarthritis + Monoarthritis OR Polyarthralgia = 1 major & 1 minor
Chorea : if : insidious alone exclusion of other causes : No need for other manifestations
Definite History of arthritis is sufficient for satisfying : history of ARF
Evidence of GAS infection ( even 1/5 ) + one minor or major = what is the category ?
2 major or 1 major + 2 minor + No evidence of GAS infection = what is the category ?
Such cases should be further categorized according to the level of evidence
Probable ARF ( previously : highly suspected ) – Possible ( previously : Uncertain )
1 major + 1 minor : 1 major : 2 minor : 1 minor with base of GAS
High Risk Group : > 30/100.000 / year in 5-15 years-old ( of ARF incidence )
OR ( RHD )( all age prevalence ) : > 2 / 1000
People at high risk : rural ^ remote settings
Evidence of GAS infection : ASOT or ADBT or Throat culture or Rapid Ag or Nucleic acid test
If Carditis is present as major criteria – Prolonged P-R interval is not minor
Elevated : ADBT is high than ASOT ( nearly double )
Upper Limits of Normal ( ULN ) : for ASOT & ADBT
Lowest levels : Below 5 Y & Above 25 Y ( ASOT = 170 & ADBT = 350 )
Much lower > 35 Y ( 130 & 260 )
Higher levels : Between 5 & 15 Y
In Recurrent episodes No need for majors : Minors are sufficient ( 3 )
Definitions : From NZ & AU Criteria
Prognosis
Good Prognosis
For Older Age Group & If No Carditis during initial attack
Bad Prognosis
For Younger children & those with Carditis with valvular lesions
RHD : may involve : Higher / valvular & Lowest / is infective endocarditis
Treatment of GAS infection ( 1ry prophylaxis ) : to prevent ARF
Treating episodes of ARF with long acting penicillin ( 2ry prophylaxis )
To prevent further episodes of ARF
Primordial prophylaxis : potential options for prevention
Strategies to avoid GAS infection ( e.g. good housing ) & Gas vaccine
Screening of RHD of school age By Auscultation : neither sensitive nor specific
ECHO : Sensitive & Specific : For Subclinical ( OR Latent RHD )
Is GAS infection :
Has the same effect on different age group
Has the same effect on the same individual , at different ages
Has the same reactions
Has the same target tissues
Has the same response to Penicillin ( Dose & duration )
Comparison of : PSRA & ARF
PSRA ARF
Why & How to modify 2ry Prophylaxis ?
From 600000 to 1200000
From 1200000 to 600000
From 2 ro 3 weeks
From 3 to 4 weeks
From 4 to 3 weeks
From parenteral to Ora
Support with Oral
Is aging require modification
Is Successful prophylaxis require modification
Rheumatic fever ( criteria for diagnosis )

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Rheumatic fever ( criteria for diagnosis )

  • 1. Rheumatic Fever Criteria for Diagnosis : From : 1944 to 2020 ARF ( Acute Rheumatic Fever ) Safwat EL-ARABY M.D. Rheumatology EGYPT
  • 2. Is available Criteria valid for : High & Low Risk Areas & Populations
  • 3. Before Diagnosis : Is manifestations of ARF are the same for : Boys & Girls 3 / 5 / 7 / 9 / 11 / 13 / > 16 years North & South countries Developed & Developing countries Crowded localities Race : Chinese / Egyptian / European / African
  • 4. This phenomenon ( of Variation ) is relevant : Why ? because there are No specific diagnostic tests for ARF, and a Diagnostic mistake has severe consequences. Diagnosis ‫المتابينة‬ ‫االمراض‬ : ‫طبية‬ ‫معضلة‬ ‫متباينة‬ ‫الروماتيزمية‬ ‫االمراض‬ ‫من‬ ‫الكثير‬ ‫هناك‬ : ‫والمخرج‬ ‫والمسار‬ ‫البداية‬ ‫متعددة‬ ‫وعالجية‬ ‫تشخيصية‬ ‫معضلة‬ ‫هناك‬ ‫اذا‬ : ‫موحدة‬ ‫عالجية‬ ‫استراتيجية‬ ‫او‬ ‫موحدة‬ ‫تشخيصية‬ ‫معايير‬ ‫ايجاد‬ ‫العسير‬ ‫من‬ ‫سيكون‬
  • 5.
  • 6. The classic Jones criteria was first published in 1944 It is revised in 1956 1965 1992. In 2004, the WHO published an expert consultation report that included ARF criteria based on the previous revised Jones criteria Diagnosis ‫المتابعة‬ ‫من‬ ‫فائدة‬ ‫هناك‬ ‫هل‬ : ‫اخرى‬ ‫الى‬ ‫مرضية‬ ‫مجموعة‬ ‫ومن‬ ‫الخر‬ ‫مكان‬ ‫من‬ ‫تتكرر‬ ‫ربما‬ ‫التى‬ ‫ظروفها‬ ‫ولها‬ ‫توقيتها‬ ‫لها‬ ‫معايير‬ ‫كل‬ ‫نعم‬ ARA Revised Criteria : 1992 2015 2018
  • 7. Diagnosis Each criterion modification has resulted in : More Specific and Less Sensitive criteria, Is it good ? Yes & No It is appropriate for most of the developed world but Not for Hot Spots ( High Risk Areas ) ‫الهادئه‬ ‫لبالدهم‬ ‫مناسبة‬ ‫النها‬ ‫واالمريكية‬ ‫االوربية‬ ‫المعايير‬ ‫على‬ ‫كليا‬ ‫نعتمد‬ ‫اال‬ ‫يجب‬ ‫لذلك‬ ‫وذاك‬ ‫هذا‬ ‫من‬ ‫خليط‬ ‫النها‬ ‫العالمية‬ ‫الصحة‬ ‫منظمة‬ ‫وايضا‬ ‫وجنسنا‬ ‫ومنطقتنا‬ ‫وظروفنا‬ ‫باطفالنا‬ ‫الخاصة‬ ‫المعايير‬ ‫الى‬ ‫وانتظارا‬ : ‫مظاهر‬ ‫باقى‬ ‫من‬ ‫بنا‬ ‫خاصة‬ ‫اخرى‬ ‫ابعاد‬ ‫واضافة‬ ‫المعتمدة‬ ‫بالمعايير‬ ‫االستعانة‬ ‫يجب‬ ‫المصرية‬ ‫والخبرة‬ ‫المرض‬
  • 10. ‫المتابعة‬ ‫من‬ ‫فائدة‬ ‫هناك‬ ‫هل‬ : ‫اخرى‬ ‫الى‬ ‫مرضية‬ ‫مجموعة‬ ‫ومن‬ ‫الخر‬ ‫مكان‬ ‫من‬ ‫تتكرر‬ ‫ربما‬ ‫التى‬ ‫ظروفها‬ ‫ولها‬ ‫توقيتها‬ ‫لها‬ ‫معايير‬ ‫كل‬ ‫نعم‬ Add
  • 11. It is now : Gate / Base / To categorize
  • 12. If supported by preceding GAS infection The presence of 2 major OR 1 major & 2 minor manifestations indicates a high probability of ARF Add ASO Titre RA Test
  • 13. Pit falls of Jones Criteria 1- It is difficult to diagnose ARF when Carditis is the only manifestation of the disease particularly in a recurrence 2- When patient has sub-clinical Carditis the clinicians fail to detect clinically 4- When previous Cardiac status is unknown , it is not possible to know in a new case whether the findings are due to : Acute Carditis OR it is recrudescence OR it is established old case of RHD 3- Clinically apparent Carditis is present but supportive minor criteria are not fulfilled 5- In case of Polyarthralgia , which is a minor criterion , if the patient is neglected & not evaluated for ARF , they would go undiagnosed , & could end up with RHD , allegedly without any past history of ARF : as the patient & the parents would have long forgotten the joint pain
  • 15. Diagnostic criteria for rheumatic fever – modified 1992 Jones criteria ( By AHA = American Heart Association ) Major criteria Minor criteria Carditis Arthritis Chorea Erythema marginatum Subcutaneous nodules Hyperpyrexia Arthralgia, without other signs of inflammation Laboratory indicators of acute phase: ESR, CRP Prolonged PR interval in ECG And Evidence of antecedent streptococcal infection – Throat Swab Culture or Rapid Antigen Test – Elevated / increasing ASO antibody titer in serum
  • 16. Jones Criteria ( Revised ) For Guidance in the diagnosis of RF Clinical Laboratory Major manifestations Minor manifestations Supporting Evidence of Strept. infection Recommendations of the American Heart Associations The presence of Two Major OR of One Major & Two Minor Criteria : Indicates a high Probability of ARF : If Supported by evidence of GASBH infection 5 4 4 3
  • 17. ‫التوقيت‬ ‫هذا‬ ‫حتى‬ : ‫كانت‬ ‫ان‬ ‫وبعد‬ ‫المعيشة‬ ‫مستوى‬ ‫وتقدم‬ ‫البنسلين‬ ‫اكتشاف‬ ‫بعد‬ ‫ان‬ ‫االعتبار‬ ‫فى‬ ‫يؤخذ‬ ‫لم‬ ‫العالم‬ ‫مستوى‬ ‫على‬ ‫منتشرة‬ ‫والحمرا‬ ‫الروماتيزمية‬ ‫الحمى‬ ‫خطورة‬ ‫االقل‬ ‫المناطق‬ ‫وسميت‬ ‫المرض‬ ‫بها‬ ‫يندر‬ ‫وشعوب‬ ‫مناطق‬ ‫هناك‬ ‫اصبح‬ ‫عالية‬ ‫مخاطرة‬ ‫ذات‬ ‫وشعوب‬ ‫ومناطق‬ ------------------------------------------------------------------------------------------------------- ‫حسب‬ ‫على‬ ‫الروماتيزمية‬ ‫للحمى‬ ‫التشخيصية‬ ‫المعايير‬ ‫حسابات‬ ‫واعادة‬ ‫وتطوير‬ ‫تعديل‬ ‫الصواب‬ ‫من‬ ‫كان‬ ‫لذلك‬ ‫الشعب‬ ‫وهذا‬ ‫البلد‬ ‫لهذا‬ ‫االصابة‬ ‫معدل‬ ( ‫المرض‬ ‫من‬ ‫الوفيات‬ ‫ومعدل‬ ‫القلب‬ ‫واصابات‬ ‫الوماتيزمية‬ ‫الحمى‬ ‫اصابات‬ ‫معدل‬ ) ------------------------------------------------------------------------------------------------------- ‫صور‬ ‫الختالف‬ ‫نظرا‬ ‫بها‬ ‫خاصة‬ ‫معايير‬ ‫وضعت‬ ‫قد‬ ‫البالد‬ ‫بعض‬ ‫ان‬ ‫الى‬ ‫الخصوصية‬ ‫من‬ ‫لمزيد‬ ‫االمر‬ ‫وصل‬ ‫بل‬ ‫مواطنيها‬ ‫حماية‬ ‫من‬ ‫ولمزيد‬ ‫اخر‬ ‫الى‬ ‫شعب‬ ‫ومن‬ ‫اخر‬ ‫الى‬ ‫بلد‬ ‫من‬ ‫المرض‬ ‫ومسارات‬ ( ‫ونيوزيالندا‬ ‫استراليا‬ ) ‫للقلب‬ ‫االمريكية‬ ‫المنظمة‬ ‫فعلت‬ ‫هكذا‬ : ‫خطورة‬ ‫االقل‬ ‫والبالد‬ ‫المخاطر‬ ‫عالية‬ ‫للبالد‬ ‫معايير‬ ‫بوضع‬
  • 18. Diagnostic criteria for RF ( AHA ) – modified 2015 Jones criteria Major criteria Low Risk Population High Risk Population Carditis (clinical or subclinical) Arthritis – only Polyarthritis Chorea Erythema marginatum Subcutaneous nodules Carditis (clinical or subclinical) Arthritis : Monoarthritis or Polyarthritis Poly arthralgia Chorea Erythema marginatum Subcutaneous nodules Minor criteria Low Risk Population High Risk Population Poly arthralgia Hyperpyrexia (≥ 38.5ºC) ESR ≥ 60 mm/h and/or CRP ≥ 3.0 mg/dl Prolonged PR interval (after taking into account the differences related to age; if there is no carditis as a major criterion) Mono arthralgia Hyperpyrexia (≥ 38.0ºC) ESR ≥ 30 mm/h and/or CRP ≥ 3.0 mg/dl Prolonged PR interval (after taking into account the differences related to age; if there is no carditis as a major criterion) 5 4 USA / EUROPE India / AU / NZ
  • 19. Subclinical carditis : No Symptoms / No Signs Only ECHO (Doppler) reveals mitral or aortic valve pathology The First Episode of the disease Diagnosis of RF : Evidence of antecedent AGSBH infection Confirmation with : 2 major or 1 major & 2 minor criteria – The Subsequent Episodes of the disease requires a confirmation of 2 major or 1 major & 2 minor or 3 minor criteria ECHO It is currently the main diagnostic tool For Confirmation, Diagnosis and Monitoring of valvular lesions in the course of RF, especially in subclinical carditis.
  • 20. 1. In the major criteria : 1. Low risk population: clinical and/or subclinical carditis. AHA recommends that all the patients with suspected RF undergo Doppler ECHO examination, even if no clinical signs of carditis are present In doubtful cases it is recommended that ECHO is repeated. 2- Medium and High risk population: also clinical and/or subclinical carditis and arthritis – Monoarthritis or Polyarthritis, possibly also with PolyArthralgia 2- In the minor criteria: 1. Low risk population : the parameters of inflammation and the level of fever were defined precisely. 2. Medium and High risk population : Monoarthralgia, also with defined parameters of inflammation and the level of fever. The modifications introduced in 2015 in the Jones criteria are as follows:
  • 21. (RF) is an autoimmune disease associated with GASBH infection, in the course of which the patient develops Carditis, Arthritis, Chorea, SC nodules and EM RF diagnosis is based on the Jones criteria, developed in 1944, then revised twice by the American Heart Association (AHA), in 1992 and recently in 2015. The last revision of the Jones criteria consists mainly in the supplementation of the major criteria with ECHO examination, the introduction of a concept of Subclinical Carditis and the isolation of : Low, Medium and High Risk Populations AHA recommends : that all the patients with suspected RF undergo Doppler ECHO after the Jones criteria have been verified, even if no clinical signs of carditis are present. Diagnosis of RF ; 2018 RF can also be diagnosed if the Jones criteria are not met, in the case of isolated Chorea or Carditis with an : insidious onset, long-term course and indistinct progression of lesions, after other causes have been excluded. A Low Risk Population : is one in which cases of ARF occur in ≤ 2/100 000 school-age children or RHD is diagnosed in ≤ 1/1000 patients at any age during one year Review :
  • 22. Situational Therapy Add to your Situations
  • 23. The Guideline Recommendations For diagnosing Rheumatic Fever Recurrences are : With a reliable past history of ARF OR established RHD & in the face of GAS infection 2 Major OR 1 Major & 2 Minor OR 3 Minor manifestations may be sufficient for a presumptive diagnosis If No Major : Only Minors : It is recommended : Exclusion of other more likely causes of the clinical presentation before a diagnosis of an ARF recurrence is made
  • 24. ( Possible ) Rheumatic Fever In some circumstances , 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 the clinicians should use their discretion & clinical acumen to make the diagnosis that they consider most likely & manage the patient accordingly Clinical sense should be respected Wakefulness / Watchfulness / Clinical smell
  • 25. ( Possible ) Rheumatic Fever 1- Where there is Genuine Uncertainty ------------------------------------------------------------------------------ It is reasonable to consider offering 12 months of 2ry prophylaxis Followed by : Reevaluation ( Careful History & examination + ECHO ) 2- If recurrent symptoms ( particularly joints ) + adherent to prophylaxis + lacks serological evidence of GAS infection + lacks ECHO evidence of valvulitis , ----------------------------------------------------------------------------------------------------------------------- it is reasonable to exclude ARF & Discontinue prophylaxis Antibiotics
  • 26. To avoid lack of sensitivity To fill pitfalls More addition to your situations For more situational Therapy
  • 28. * Major manifestations Carditis, Polyarthritis, Chorea, Erythema Marginatum, SC Nodules ** Minor manifestations Clinical : Fever, Polyarthralgia; Laboratory findings : elevated acute-phase reactants (ESR or WBC count) *** Supporting evidence of : ECG : Prolonged PR interval ; Preceding streptococcal infection ; Elevated or rising ASO or streptococcal infection with another streptococcal Ab ; Infection in the past 45 days ; Positive throat culture ; Rapid antigen test for group A streptococci ; Recent Scarlet Fever. 2004 WHO Criteria for the Diagnosis of Rheumatic Fever and Rheumatic Heart Disease WHO Criteria 2004 For More Sensitivity 5 4 7
  • 29. Primary episode of RF 2 Major* OR 1 Major and 2 Minor** manifestations Plus evidence of a preceding group A streptococcal infection*** Recurrent attack of RF in a patient without established RHD 2 Major OR 1 Major and 2 Minor manifestations Plus evidence of a preceding group A streptococcal infection Recurrent attack of RF in a patient with established RHD 2 Minor manifestations Plus evidence of a preceding group A streptococcal infection c 2004 WHO Criteria for the Diagnosis of RF and RHD WHO Criteria Diagnostic categories Criteria More addition to your situations For more situational Therapy ‫سابق‬ ‫روماتيزمى‬ ‫قلب‬ ‫وجود‬ = ‫كبير‬ ‫معيار‬ 1 2 3
  • 30. Rheumatic chorea and insidious onset of rheumatic carditis Other major manifestations OR evidence of group A streptococcal infection are not required Chronic valve lesions of RHD Patients presenting for the first time with Pure mitral stenosis or mixed mitral valve disease and/or aortic valve) Do not require any other criteria to be diagnosed as having RHD 2004 WHO Criteria for the Diagnosis of RF and RHD WHO Criteria More addition to your situations For more situational Therapy 4 5
  • 31. Patients may present with polyarthritis (or with only polyarthralgia or monarthritis) and with several (three or more) other minor manifestations, together with evidence of recent GAS infection. Some of these cases may Later turn out to be RF It is wise to consider them as cases of “Probable RF” (once other diagnoses are excluded) and advise regular secondary prophylaxis. Such patients require close follow-up and regular examination of the heart. This cautious approach is particularly suitable for patients in vulnerable age groups in high-incidence settings. Some patients with recurrent attacks may not fulfill these criteria. Congenital heart disease should be excluded. 2004 WHO Criteria for the Diagnosis of RF and RHD WHO Criteria 6 7 8
  • 33. Specificity Sensitivity Specificity Sensitivity High Risk Area High Risk Population Low Risk Area Low Risk Population
  • 34. f6-clep-3-067: Map showing reported worldwide prevalence of RHD from 1970 through 1990.14,21,86,90,99,102–104,111,112,118,128,132,136,201–216 (1) Barbados,104 (2) Cook Islands,21 (3) El Salvador,202 (4) Fiji,112 (5) French Polynesia,112 (6) Jamaica,202 (7) Puerto Rico,103 (8) Samoa,112 (9) Tonga,112,136 (10) Minnesota.86 Rheumatic Heart Disease : 1970 ; 1990
  • 35. Map showing reported worldwide prevalence of RHD from 1991 through present. 2-6,21,111,160,168,169,176,177,189,191,192,197,217-240 (1) Fiji,169,228 (2) Samoa,192 (3) Tonga.3 Why ? NZ & AU Rheumatic Heart Disease : 1990 trough Present
  • 36. File:Rheumatic heart disease world map- Deaths per million persons- WHO : 2012 NZ & AU RHD : Deaths/ Million persons : WHO : 2012
  • 38. Use of Echocardiograms for the diagnosis of carditis Aseptic monoarthritis as a major criterion ( provided NSAIDs are used in NZ and only for high-risk individuals in AU ) Use Polyarthralgia as a major criterion for high risk individuals (AU) Use Monoarthralgia as a minor criterion for high–risk individuals (AU) Diagnosis These guidelines may be more applicable to high risk populations Utilization of NZ guidelines for the diagnosis of ARF result in a modest increase (16%) in cases classified as definite ARF compared with (AHA) 1992 Jones criteria. ‫الحاالت‬ ‫من‬ ‫عدد‬ ‫اكبر‬ ‫التقاط‬ ‫وبالتالى‬ ‫معاييرهم‬ ‫حساسية‬ ‫من‬ ‫ليرفعوا‬ ‫القوم‬ ‫هؤالء‬ ‫اليها‬ ‫لجأ‬ ‫التى‬ ‫واالدوات‬ ‫الوسائل‬ ‫هى‬ ‫ما‬ NZ & AU have developed their own criteria , the main particularities of which are : Result
  • 39. ‫الحاالت‬ ‫معظم‬ ‫التقاط‬ ‫ثم‬ ‫ومن‬ ‫المعايير‬ ‫بزيادة‬ ‫وذلك‬ ‫الحساسية‬ ‫رفع‬ ‫الصواب‬ ‫من‬ ‫هل‬ ‫احصائيا‬ : ‫الحساسية‬ ‫زيادة‬ : ‫التخصصية‬ ‫نقص‬ ‫يتبعه‬ : ‫العالية‬ ‫الحساسية‬ ‫ذات‬ ‫المعايير‬ ‫هذه‬ ‫ستلتقط‬ ‫اذا‬ : ‫الخاطئة‬ ‫الحاالت‬ ‫من‬ ‫مزيد‬ ‫مثال‬ : ‫الحساسية‬ ‫كانت‬ ‫اذا‬ 50 : % ‫فقط‬ ‫ستلتقط‬ ‫اذا‬ 50 % ‫الصحيحية‬ ‫الحاالت‬ ‫من‬ : ‫خاطئة‬ ‫حاالت‬ ‫هناك‬ ‫يكون‬ ‫ال‬ ‫ربما‬ : ‫عالية‬ ‫التخصصية‬ ‫التخصيصية‬ ‫رفع‬ : ‫العالج‬ ‫الى‬ ‫تحتاج‬ ‫التى‬ ‫الحاالت‬ ‫بعض‬ ‫سنفقد‬ ‫ولكن‬ ‫فقط‬ ‫الصحيحة‬ ‫الحاالت‬ ‫التقاط‬ ‫الى‬ ‫سيؤدى‬ ‫الى‬ ‫الحساسية‬ ‫رفع‬ ‫اردت‬ ‫اذا‬ 95 % ‫والتقاط‬ 95 % ‫الحاالت‬ ‫من‬ : ‫معهم‬ ‫ستلتقط‬ 10 ‫خاطئة‬ ‫مثال‬ ‫حاالت‬ ‫اليه‬ ‫حاجة‬ ‫فى‬ ‫ليسوا‬ ‫عالج‬ ‫سيتلقون‬ ‫العشرة‬ ‫وهؤالء‬ : ‫تقديرى‬ ‫االمر‬ ‫هنا‬ ‫االمر‬ ‫هذا‬ ‫مع‬ ‫تعامل‬ : ‫الصحية‬ ‫وحالته‬ ‫مجتمع‬ ‫عن‬ ‫مسئول‬ ‫كطبيب‬ ‫مرة‬ : ‫الطفل‬ ‫لهذا‬ ‫امر‬ ‫كولى‬ ‫ومرة‬ ‫امر‬ ‫ولى‬ ‫كنت‬ ‫واذا‬ : ‫موقفان‬ ‫هناك‬ : ‫المناسب‬ ‫العالج‬ ‫يتلقى‬ ‫ولم‬ ‫المعايير‬ ‫من‬ ‫سقط‬ ‫طفل‬ ‫امر‬ ‫ولى‬ ‫مرة‬ : ‫لديه‬ ‫يوجد‬ ‫ال‬ ‫طفل‬ ‫امر‬ ‫ولى‬ ‫ومرة‬ ‫مدمرة‬ ‫وصمامات‬ ‫روماتيزمى‬ ‫قلب‬ ‫ماساة‬ ‫من‬ ‫اخر‬ ‫طفل‬ ‫حما‬ ‫وقد‬ ‫المفعول‬ ‫طويل‬ ‫بنسلين‬ ‫ويتلقى‬ ‫مرض‬ ‫الساخنة‬ ‫للمناطق‬ ‫وخاصة‬ ‫بحثها‬ ‫يجب‬ ‫خالف‬ ‫نقاط‬
  • 40. 2020 Updated Australian Criteria for ARF diagnosis High Risk Group : Communities with high rate of ARF : incidence > 30/100000 / year OR RHD > 2/1000 / year
  • 41. For more Situations Definite : Initial Definite : Recurrence Probable : initial Probable : Recurrence Possible : Recurrence Possible : initial For recurrent episode : > 90 days from previous episode of ARF Is Polyarthritis + Monoarthritis OR Polyarthralgia = 1 major & 1 minor Chorea : if : insidious alone exclusion of other causes : No need for other manifestations Definite History of arthritis is sufficient for satisfying : history of ARF Evidence of GAS infection ( even 1/5 ) + one minor or major = what is the category ? 2 major or 1 major + 2 minor + No evidence of GAS infection = what is the category ? Such cases should be further categorized according to the level of evidence Probable ARF ( previously : highly suspected ) – Possible ( previously : Uncertain ) 1 major + 1 minor : 1 major : 2 minor : 1 minor with base of GAS
  • 42. High Risk Group : > 30/100.000 / year in 5-15 years-old ( of ARF incidence ) OR ( RHD )( all age prevalence ) : > 2 / 1000 People at high risk : rural ^ remote settings Evidence of GAS infection : ASOT or ADBT or Throat culture or Rapid Ag or Nucleic acid test If Carditis is present as major criteria – Prolonged P-R interval is not minor Elevated : ADBT is high than ASOT ( nearly double ) Upper Limits of Normal ( ULN ) : for ASOT & ADBT Lowest levels : Below 5 Y & Above 25 Y ( ASOT = 170 & ADBT = 350 ) Much lower > 35 Y ( 130 & 260 ) Higher levels : Between 5 & 15 Y In Recurrent episodes No need for majors : Minors are sufficient ( 3 ) Definitions : From NZ & AU Criteria
  • 44. Good Prognosis For Older Age Group & If No Carditis during initial attack Bad Prognosis For Younger children & those with Carditis with valvular lesions RHD : may involve : Higher / valvular & Lowest / is infective endocarditis Treatment of GAS infection ( 1ry prophylaxis ) : to prevent ARF Treating episodes of ARF with long acting penicillin ( 2ry prophylaxis ) To prevent further episodes of ARF Primordial prophylaxis : potential options for prevention Strategies to avoid GAS infection ( e.g. good housing ) & Gas vaccine Screening of RHD of school age By Auscultation : neither sensitive nor specific ECHO : Sensitive & Specific : For Subclinical ( OR Latent RHD )
  • 45. Is GAS infection : Has the same effect on different age group Has the same effect on the same individual , at different ages Has the same reactions Has the same target tissues Has the same response to Penicillin ( Dose & duration )
  • 46. Comparison of : PSRA & ARF PSRA ARF
  • 47. Why & How to modify 2ry Prophylaxis ? From 600000 to 1200000 From 1200000 to 600000 From 2 ro 3 weeks From 3 to 4 weeks From 4 to 3 weeks From parenteral to Ora Support with Oral Is aging require modification Is Successful prophylaxis require modification