cpm3rd rheumatic fever

8/15/2019 CPM3rd Rheumatic Fever

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RHEUMATIC FEVER / RHEUMATIC HEART DISEASE

PHILIPPINE SOCIETY OF PEDIATRIC CARDIOLOGY


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Santiago V. Guzman, M.D.

Wilberto L. Lopez, M.D.

Edgardo E. Ortiz, M.D.

Asuncion A. Reloza, M.D.

Luis M. Mabilangan, M.D.

Philippine Pediatric SocietyPhilippine Society of Pediatric CardiologyDepartment of HealthPhilippine Foundation for the Prevention and Controlof RF/RHD

Contributors

179


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RHEUMATIC FEVER/RHEUMATIC HEART DISEASE CPM 3RD EDITION

180

Algorithm for the Management of Cases of AcuteRheumatic Fever

N

Y

Y

Y

N

N

N

Y

1

2

3

4

5

6

78

910

11 1213

1415

Patient aged 5 to15 years with jointswelling, pain andhigh temperature

Send forconsultation

Patientsees a physician

within 48hours?

Make the patientrest and treat with

benzathine penicillinuntil physician can

see patient.

MedicalExamination

Meets the

Jones'Criteria?

Check whetherthe streptococcal

infection wastreated

Keep patientunder

observation for2 weeks

Classify the

case

Meets the

Jones'Criteria?

DischargeCarditis?

If there is nohospital, treat with

inflammatory drugs.Let patient rest and

begin secondaryprevention.

Send patientto

hospital

Send forregistry

FIGURE 1


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CPM 3RD EDITION RHEUMATIC FEVER/RHEUMATIC HEART DISEASE

181

Algorithm for the Secondary Preventionof Rheumatic Fever

N

Y

N

Y

N

Y

N

Y

Y

N

1

2 3

4 5

6 7 8

9 10 11

12

13

14 15

16

Patient with ARF in thepast and/or with RHD

seen by the physician forsecondary prevention

Hypersensitiveto penicillin?

Treatment witherythromycinby mouth for

10 days

Begin orcontinue preventive

administration ofIM penicillinevery month

Continue preventiveregimen with a dailydose erythromycin250 mg twice daily

Has RHD?

Maintainpreventiveregimen for

whole life

Keep an eyeon evolution

Over 5 yearssince acute

attack?

Has reached18 yearsof age?

Suspend thepreventiontreatment

Continue preventiveregimen until 5years have elapsed

Keep an eye onevolution

Functionallystable?

Continuepreventive

regimen

Send tospecialist for

evaluation

FIGURE 2


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182

Algorithm for the Diagnosis and Treatment of Strepto-coccal Sore Throat

Y

N

Child 5 to 15 yearsof age with an acute

infection of the upperrespiratory tract

Consultation

Examination byphysician, nurse or

trained auxiliary

Apply the clinicaland/or bacteriological

diagnosis criteria

Positive throatculture

(Typical Strepthroat)

Allergic topenicillin?

Treatmentwith

erythromycinfor 10 days

A single dose ofbenzathine penicillin

or 10 days of oralpenicillin

Healtheducation

Discharge

1

2

3

4

5

6 7

8 9

10

FIGURE 3


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183

INTRODUCTION

The RF/RHD Registry is one major component of the Programfor the Prevention and Control of RF/RHD, being spearheadedby the Philippine Foundation for the Prevention and Controlof RF/RHD in coordination with the Department of Healthand other professional organizations and societies, such as thePhilippine Heart Association, Philippine Society of PediatricCardiology and the Philippine Pediatric Society.

The RF/RHD Registry will serve at least two purposes. Firstly,it will verify referred cases of RF/RHD, which will eventuallypermit a long-term follow-up study of verified cases of RF/ RHD. Secondly, it will act as means through which secondaryprophylaxis will be administered.

SCREENING AND MANAGEMENT OF CASES OF ACUTERF

If the patient is a child aged between 5 and 15 who is sufferingfrom joint pain and high temperature, he must be sent to aphysician as soon as possible. If no physician is available,the patient is advised to rest and symptomatic treatment is

given.

The physician should examine the patient before 48 hourshave elapsed and check whether he presents the symptomsmentioned in the Jones' Diagnostic Criteria (Tables 1-3 andFigure 1). If he does not, he should be kept under observationfor two weeks and then discharged if the diagnosis cannot beestablished within the period. If the Jones' criteria are fulfilled,the physician will check whether the streptococcal infectionhas been treated and will classify the case as to presence orabsence of carditis.

If the patient is not suffering from carditis, he can be treatedby a general practitioner but whenever possible, he shouldbe admitted to a hospital as an in-patient for confirmationof the diagnosis and for treatment. A patient with carditismust always be admitted to a hospital for treatment and afterhospital discharge, must be on home rest for at least six (6)weeks (Algorithm Fig. 1). Patient is also sent to a registry forsecondary penicillin prophylaxis (Algorithm Fig. 2).

Table 1: Diagnostic Criteria

Modified Jones' Criteria for Diagnosis of Rheumatic Fever

A. Major Criteria B. Minor Criteria

1. Arthritis 1. Fever

2. Carditis 2. Arthralgia (pain in

joints without objective

findings)

3. Chorea 3. Increased sedimentation

rate and presence of C- reactive protein or

leucocytosis

4. Erythema 4. Evidence of preceding

Marginatum beta-hemolytic strepto-

coccal infection

5. Subcutaneous 5. A previous history of

nodules rheumatic fever or the

presence of established

valvular disease

6. Prolonged P-R interval

in the ECG

Definite RF: Presence of two (2) major criteria, or of one (1)major and two (2) minor criteria.

Historical Note: The Jones' Criteria for guidance in thediagnosis of acute rheumatic fever were initially proposed byT. Duckett Jones in 1944. Committees of the American HeartAssociation subsequently modified (1955), revised (1965)and now updated (1992). This modified criteria should onlybe used if application of the updated criteria is not feasible

in the community.

Table 2: Jones' Criteria, Updated 1992

Major Minor

Carditis Minor

Polyarthritis Clinical

Chorea Fever

Erythema marginatum Arthralgia

Subcutaneous Nodules Laboratory

Elevated ESR

Positive CRP Prolonged P-R interval

Evidence of previous Group A β−hemolytic Streptococcalinfection either: Positive throat culture Positive rapid streptococcal antigen test for Group A

streptococcal Elevated ASO titer

Previous infection is indicated by increased anti- streptolysin

O or other streptococcal antibody and positive throat culturefor Group A streptococcus. Manifestations with a long latentperiod, such as chorea and late-onset carditis, are exemptedfrom this last requirement.

Definite RF Diagnosis: Presence of two (2) major criteria, or one(1) major and two (2) minor criteria. Plus evidence of previousGroup A β-hemolytic streptococcal infection.

Manual of Operation of the Primary and Secondary Pre-vention of Rheumatic Fever-Rheumatic Heart Disease


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intensity of exposure to streptococcal infections which isinfluenced by age and geographical area. Titers up to 2000 arecommon in healthy school-age children so that only levels of333 units or higher are considered abnormal.

*Markowitz and Gordis; W.B. Saunders, Philadelphia, 1972

PRIMARY PREVENTION

Primary prevention is the treatment of upper respiratorytract infection due to group A beta-hemolytic streptococcito prevent an initial attack of acute rheumatic fever. Studieshave demonstrated that appropriate antibiotics, given early inthe course of streptococcal infection, essentially prevents thedevelopment of rheumatic fever (Table 5).

Primary prevention should be given equal importance assecondary prevention because primary prevention has the

following advantages:

1. Permits eradication of the disease;2. Duration of treatment is brief;3. Treatment is accepted because it concerns sick

individuals;4. Prevents severe and irreversible heart damage from first

attack of RF;5. Cost-effective if prevention is restricted to the major “at

risk” group - children aged 5-15 years compared to treatingcases of RHD for life.

All physicians involved in child health care and primaryhealth workers such as municipal health officers, schoolphysicians and general practitioners should be encouragedto detect and treat streptococcal pharyngitis (Tables 6-7 andAlgorithm Fig. 3).

Table 5: Recommended Treatment for Group A StreptococcalPharyngitis

Mode of

Antibiotics Administration Dose

-1,200,000 units foradults & children more

than 30 kg

Benzathine Intramuscular 600,000 units for

Penicillin children weighing

less than 30 kg

(given as a single

injection)

-250 mg 3-4 times a

day for 10 days. Very

small children

Phenoxymethyl Oral weighing less than

Penicillin (V) 20 kg may be given125 mg 4 times a day.

Penicillin V (phenoxymethyl penicillin) is the preferred oralformulation because of its more reliable absorption. PenicillinG may also be used if available, although its absorption isless predictable.

For patients allergic to penicillin, oral erythromycin is anaccepted alternative. The recommended dose of erythromycinis 250 mg 4 times a day. For children weighing less than 25 kg,the recommended dose is 40 mg/kg of body weight per dayin 2-4 divided doses. The total daily dose should not exceed1 gram.

SECONDARY PREVENTION OF RHEUMATIC FEVER ORPREVENTION OF RECURRENCES

Secondary prevention will be prescribed for rheumatic feverpatients for all ages, but more frequently in school childrenand young adults who have had one or more attacks ofrheumatic fever.

It is essential to check whether the patients are hypersensitiveto penicillin, a sensitivity test must be done before the first

injection.

* Those not hypersensitive to penicillin will be given a 21-28days preventive dose of 1,200,000 u of benzathine penicillinby the intramuscular route.

* In special circumstances or high-risk patients, the injectionmay be given every 3 weeks (21 days).

* Those who are hypersensitive but not undergoing apreventive regimen will have to be given an eliminatorycourse of treatment initially with erythromycin by mouthfor 10 days: 125 mg four times a day for those under six yearsand 250 mg four times a day for older patients.

* They will be maintained on Erythromycin 250 mg 2x daily,as in the penicillin maintenance schedule.

* Patients who are not suffering from RHD and have onlyhad an attack of acute rheumatic fever will follow the sametreatment schedule for 10 years until they are 18 years of age,whichever is longer (Algorithm Fig. 2).

* When they have fulfilled these two requirements, i. e. thatthey should be 18 years of age or over and that 10 yearsor more should have elapsed since the last acute attack,prevention will be suspended and they will be kept undersurveillance.

* The preventive regimen will continue throughout life forthose with cardiac lesions, but care must be taken to ensurethat the condition remains stable. If symptoms of fatigueappear and if there is a change of functional category, theperson concerned will again be sent to the physician forexamination.

* Patients who have undergone heart surgery will be includedin the registry to prevent bacterial endocarditis.

Rheumatic fever once diagnosed by appropriate criteriais considered clinically active if any one of the followingfeatures is found:

1. Joint symptoms 2. New organic murmurs 3. Changing heart size 4. Congestive heart failure (in the absence of long-standing

* Stollerman G. H., Rheumatic Fever and Streptococcal Infection: Gruneand Stratton 1975


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186

severe valvular disease). 5. Subcutaneous nodules 6. A sleeping pulse rate greater than 100/minute 7. Erythema marginatum 8. Chorea 9. A positive test for C-reactive protein 10. Unexplained fever with rectal temperature of 100.40F for

at least 3 consecutive days. The patient when confirmed to have reactivation of RF willbe treated accordingly as outlined in Table 4*.Administration of Secondary Prophylaxis

Antibiotic regimen for secondary prophylaxis of RF/RHD isshown in Table 6. Benzathine penicillin shall be providedfree of charge for RF-RHD enrolled in the Registry. Thechemoprophylaxis may be administered at the source of referralby trained primary health workers after initial injection in theReferral Center has been found safe. Patients shall be advised

to see to it that their visits for injection of benzathine penicillinshall be properly recorded in their appointment card. Also, aPatient Data Record Form should be filled up and kept in theunit where chemoprophylaxis is given.The Philippine Foundation for the Prevention and Controlof RF-RHD DOH shall issue benzathine penicillin at theRegional Registry Center. The quantity shall be based onthe list of registered cases submitted by the various ReferralCenters. Since the monthly administration of prophylaxisshall be carried out at the peripheral level, the regionalhospitals should be responsible in sending the drugs to thereferral centers which shall in turn dispose the same to thevarious units where the enrolled RF-RHD patients are beingfollowed-up.

With the private hospitals, the drug may be directly issuedto them at the RF-RHD Foundation Office at the PhilippineHeart Center.

Frequency and Duration of Prophylaxis

1. Low-risk group - every 4 weeks 1.1 Arthritis - minimum of 5 years (symptom/

recurrence free) 1.2 Carditis - minimum of 10 years or up to age18, whichever is longer, if recurrence free andnon-high-risk group

2. High-risk group - every 3 weeks 2.1 Carditis with previous history of RF 2.2 Severe attack of carditis with or withoutmultiple valve involvement 2.3 Development of recurrence of RF despiteregular monthly benzathine penicillin. 2.4 Cardiomegaly

2.5 Congestive heart failure

2.6 RHD - every 4 weeks 2.6.1 Mild - up to age 25 (single valve, without

recurrences)* (Class II-III) 2.6.2 Severe - every 3 weeks up to age 25;

(decrease unless recurrences occur) - every 4 weeks for life if recurrence

free (Class II-III)

- Class IV-Oral Prophylaxis recommended 2.6.3 RHD with surgical intervention - forprolonged period/s for life.

Note: Diagnosis of carditis should be documented by echocardiographywhen feasible.* Patients dealing with children, working in health services orcirculating in population crowded areas are at higher risk ofcontracting streptococcal infection and must continue the secondaryprophylaxis while working or living in these conditions.

Table 6: Antibiotic Regimens for Secondary (Continuous)Prophylaxis for Rheumatic Fever and RheumaticHeart Disease Prevention

Mode of Penicillin ErythromycinAdministration

Benzathine penicillin G Single inj once a month* Intramuscular For children


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Table 7: Clinical Presentation of StreptococcalTonsillopharyngitis

Common Findings Non-Strep

Symptoms

Pain on swallowing Coryza

Fever Hoarseness

Headache Cough

Abdominal pain Diarrhea

Nausea and vomiting

Signs

Tonsillopharyngeal erythema Conjunctivitis

Tonsillopharyngeal exudate Anterior stomatitis

Soft palate petechiae Discrete ulcerative lesions

("doughnut" lesions)

Beefy red, swollen ovula

Anterior cervical adenitis Scarlantiniform rash

Appendix 1

RF-RHD Registry Center

CodeNumber Region Center00000 NCR00101 Philippine Heart Center00102 St. Luke's Medical Center00103 UERMMC00104 Phil. Children's Medical Center00105 Quezon City General Hospital00201 UP-PGH00202 UST00301 Makati Medical Center00401 MCU

01000 Region I San Fernando, La Union02000 Region II Tuguegarao, Cagayan03000 Region III San Fernando, Pampanga04100 Region IV Lipa City05100 Region V Naga15200 Legaspi05300 Sorsogon06100 Region VI Iloilo07100 Region VII Cebu07200 Bohol08000 Region VIII Tacloban, Leyte09100 Region IX Zamboanga City

10100 Region X Cagayan de Oro City11100 Region XI Davao City12000 Region XII Cotabato City

References:

1. Rheumatic Fever. Markowitz and Gordis. W.B. Saunders,Philadelphia, 1972

2. Rheumatic Fever and Streptococcal Infection. Gene H.

Stallerman. Grine and Stratton, New York, 19753. Rheumatic Fever. Angelo Toronta and Milton Markowitz.MTP Press Limited, Boston, 1981

4. Prevention and Control of Rheumatic Fever in theCommunity. Pan American Health Organization.Washington, D.C., 1985

5. Rheumatic Fever and Rheumatic Heart Disease. B.L.Agarwal. Arnold Publishers. Bombay, 1988

6. Streptococcal Sore Throat Rheumatic Fever/RheumaticHeart Disease. Achutti, Kaplan, Nordet and Vynckt.UNESCO, WHO and ISFC. 1992

7. Treatment of Acute Strep Pharyngitis and Prevention of

RF: Statement for Health Professionals. Committee onRheumatic Fever, Endocarditis and Kawasaki Diseaseof the Council on Cardiovascular Diseases in the Young,American Heart Association Rajumi, et al. Pediatrics 95(96) 4: 758-68

8. Guidelines of the Diagnosis of Rheumatic Fever. Jones Criteria, 1992 update by Special Writing Groupof Committee on Rheumatic Fever. Endocarditis andKawasaki Disease of the Council on CardiovascularDiseases in the Young. American Heart Association, JAMA92 October 21; (268) 15:2069-73


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PHILIPPINE FOUNDATION FOR THE PREVENTION AND CONTROLOF RHEUMATIC FEVER/RHEUMATIC HEART DISEASE

INITIAL REGISTRATION FORMRheumatic Fever Prophylaxis

Regional Registry Center: [ ] Registration No.: [ ] Hospital No.: [ ]

Name of Patient: Sex: [ ] 1-Male 2-FemaleAddress:School:Year of Birth: [ ] [ ] [ ] Date of Registration: [ ] [ ] [ ] day month year day month yearSource of Notification to Registry: [ ] 1 - Hospital in-patient department 5 - School health service 2 - Hospital out-patient clinic 6 - Mass examination 3 - Private physician 7 - Rural Health Unit/Barangay Health Station 4 - Laboratory 8 - Others, Specify:

Active Rheumatic Fever: [ ] Diagnosis 1 - positive 2 - suspected 3 - negative 4 - not determined

Major Manifestation: Minor Manifestation: Evidence of previous Group A Clinical Laboratory Beta-hemolytic Strep infection: [ ] Carditis [ ] Fever [ ] Elevated ESR [ ] Positive Throat Culture [ ] Polyarthritis [ ] Arthralgia [ ] Positive CRP [ ] Positive Rapid Antigen

[ ] Chorea [ ] Prolonged P-R Test [ ] Erythema Marginatum Interval [ ] ASO Titer Elevation [ ] Subcutaneous Nodules

[ ] Initial attack (1 - yes; 2 - no; 3 - not known) [ ] Severity of heart damange (0 - None; 1 - Mild; 2 - Moderate; 3 - Severe; 4 - not determined) [ ] ASO Titer (1 - 800 units; 4 - not determined)

Chronic Rheumatic Cardiopathy: [ ] 1 - positive 2 - suspected 3 - negative 4 - not determined

Diagnosis [ ] Mitral Stenosis [ ] Aortic Stenosis [ ] Aortic Insufficiency [ ] Organic Lesion of the Tricuspid Valve [ ] Mitral Insufficiency [ ] Heart Failure

Year of Initial Attack: [ ] [ ] [ ] Year of last Attack: [ ] [ ] [ ] day month year day month year [ ] Number of Recurrences 1 - One 2 - Two 3 - More 4 - Not known 5 - None 6 - Initial Attack

[ ] Preventive Regimen in Previous Year

PENICILLIN 1 - Regular Intramuscular 5 - Sulphonamides 9 - None 2 - Irregular Intramuscular 6 - Erythromycin

3 - Occasional Intramuscular 7 - Any Combination 4 - Oral 8 - Initial Attack

Source of Information:Address: Telephone No.:


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Drugs Mentioned in the Treatment GuidelineThis index lists drugs/drug classifications mentioned in the treatment guideline. Prescribing Informationof these drugs can be found in the Philippine Pharmaceutical Directory (PPD) 7th edition. Opposite thebrand name is its page number in the PPD 7th edition.


189

ErythromycinAm-Erythromycin 42Ditron 42DLI-Erythromycin 43DrugmakersErythromycin 43

Ery-Max 43Erybron* 43Erycin 43Erythrocin/Erythrocin DS 43

Erythrolan NP9, 43

Ethiocin 43Gentrocin 43Ilosone 43

J. McKnoll Erythromycin 44Macrocin 44Pharex-Erythromycin 44Sarazine 44Sefavex 44Servitrocin 45UL Erythromycin 45

Pen G benzathine Penadur 6-3-3/

Penadur L-A* 52

PhenoxymethylpenicillinCentrapen 48

Cimpicillin 48 Megapen 51 Mipacin 52 Pentacillin 53 Sumapen 54 UL Phenoxymethyl

Penicillin K 55

cpm3rd rheumatic fever

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