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Rheumatic Fever :

What you should know ?

Teddy Ontoseno

Division of Cardiology

Department of Child Health Dr Sutomo HospitalAirlangga University, Surabaya


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Philosophical - Practical consideration

Licks the joints and bites the heart.


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Epidemiology

* Ages 5-15 yrs

*


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R.F. can be presented in many ways:

a.Arthritiswithout cardiac involvement.

b. Rheumatic choreawithout arthritis

nor carditis.c. Carditiswith or without arthritis.

R.F.What Pediatrician should know ?


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R.F.What Pediatrician should know ?Pathogenesis

* Recent concept :abnormal humoral (acutephase) and cellular (chronic phase) immuneresponse occurs.

* Antigenic mimicry :there is certain aminoacid sequence that is similar btw GABHS andhuman tissue in individuals with genetic

predisposition.

Immunologically mediated inf lammatory


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Rheumatic

fever is a

classicexample of

molecular

mimicry

Rheumatic fever-pathogenesis


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Throat

Heart

Rheumatic Fever Pathogenesis:TissueDamage

The Recent Concept :

HUMORAL and

CELLULAR


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R.F.What Pediatrician should know ?

B. Rheumatogenic strains of

GABHS M types l, 3, 5,

6,18,19 & 24 have antigenic

domains similar to antigensin components of the human

heart

A. Only infections

GABHS of the pharynx

initiate or reactivate RF.


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1st Problem

* 50% of patients with pharyngitis will be treatedbut will not be infected with GABHS

* 30% of patients with pharyngitis will not be

treated but will be infected with GABHS

GABHS pharyngitis and non-GABHS pharyngitisSigns and symptoms overlap broadly
http://images.google.com/imgres?imgurl=www.vvh.org/health/graphics/streplg.gif&imgrefurl=http://www.vvh.org/health/topics/strep.htm&h=203&w=200&prev=/images%3Fq%3Dstrep%2Bthroat%26svnum%3D10%26hl%3Den%26lr%3D%26ie%3DUTF-8%26oe%3DUTF-8%26sa%3DG


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How do we diagnose it?

* A laboratory test

* A clinical diagnosis and offer presumptive

treatment.

so diagnosis remains a clinical decision !


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Rationale Decission Making

The WHO Acute Respiratory Infections (ARI) :

* In the absence of laboratory diagnosis forchildren under 15 years of age, acute

GABHS pharyngitis should be suspected and

presumptively treated when pharyngeal

exudate plus enlarged and tender cervicallymph nodes are found.


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Acute Pharyngitis

To treat or not to treat?.

That is the million dollar question.

WellSo what is the mostimportant goal of treatment?


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Modified CentorScore & Management Approach( McIsaac - JAMA 2007)

Criteria Points

*Temperature >38 C 1

*Absence of Cough 1

*Swollen Tender Cervical Node 1

*Tonsillar Swelling / Exudate 1*Age: 3 - 14 years 1

1544 years 0

45 years or older -1

Total Score : ( )

Management Approach:

SCORE: 0 - 1 No Further Testing or ABX Therapy.

2 - 3 Culture All

>4 Treat Empirically .


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GABHS Pharyngitis:Treatment Options

Four reasons to treat a GABHS pharyngitiswith antibiotics :

* To prevent rheumatic fever

* To prevent peritonsillar abscess* To reduce symptoms there is a modest (~ 1

day) reduction in symptoms with early treatment

* To prevent transmission this is important in

pediatrics due to extensive exposures but not in

adults


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2nd Problem

RHEUMATIC FEVER, DIAGNOSIS

It is difficult to give a satisfactory

clinical picture of the disease,

because the modes of onset are sovaried and the symptoms so diverse.


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RF, Clinical Features:

* Acute Rheumatic Fever- Acute Inflammatory Phase

- HeartPancarditis(40-50%)

- SkinErythema Marginatum/ S.nodule (10%)

- CNSSydenham Chorea (15%)

- Migratory polyarthritis(75%)

* Chronic Rheumatic Fever- Deforming fibrotic valvular disease.


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Polyarthritis low grade fever, large joints,( > 75%), migratory, painful, warm and swollen

asymmetrical, no permanent dysfunction

Carditis - pericarditis, cardiomegaly, or

valvulitis (~ 50%) (valvulitis is the most serious

manifestation.)

Chorealate occurrence, 3 - 4 months after ( ~

10%) infection, self-limiting, resolves in 1- 3

months.


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Erythema Marginatumclassic truncalrash, ( ~ 10%) migratory - appears &disappears within hours. (pink rashirregular

red edgesclear center)

Subcutaneous Noduleslate occurs

(1 - 2%) ( months after infection), painless smallnodules over bony prominences - elbows,knees, spine.


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Major criteria of Jones

Help to remember :CAPOCHES

CarditisPolyarthritis

ChoreaErythemaMarginatum

Subcutannodule
http://www.emedicine.com/med/images/Large/1554ARF_MANIFESTATIONS.JPG


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http://www.emedicine.com/med/images/Large/1554ARF_MANIFESTATIONS.JPG


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The Jones Criteria for RheumaticFever, Updated 2003

Major Criteria

Carditis

Migratory polyarthritis

Sydenham's chore Subcutaneous nodules

Erythema marginatum

Minor Criteria

Clinical

fever

Arthralgia

Laboratory Elevated acute phase

reactants

Prolonged PR intervalplusSupporting evidence of a recent group A streptococcal infection

positive throat culture or

rapid antigen detection test; and/ or elevated or

increasing streptococcal antibody test

(e.g., anti-streptolysin O, anti-deoxyribonuclease B, anti-hyaluronidase).

C diti f ARF


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Carditis of ARF :What Paediatrician should know ?

* Pancarditis (endocarditis most serious, always present)* 40 and 60% of patients with ARF

* Characterised by

-persistent tachycardia

-organic cardiac murmurs not previously present

(mitral regurgitation)

- pericardial friction rub

- cardiomegaly

- prolonged PR interval and evidence of heart failure

may be presentnonspecific

Mitral regurgitation


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Mitral regurgitationWhat Paediatrician should know ?

Apical, softer and blowing holosystolic murmur

Pure rheumatic MR due

to shortening of valvecusps and of papillary

muscles, chordae

tendineae that becomematted and adherent to

the valve.


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Chronic RHD:


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Chronic RHD:What Paediatrician should know ?

Rheumatic fever cause- chronic process of

valvular fibrosis

- commissures are fused

- the cusps are severelythickened

- calcification with

shortened,thickened chordaetendineae

Subcutaneous nodules


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Subcutaneous nodulesWhat Paediatrician should know ?

Rarely seen and whenpresent

Usually associated withsevere carditis.

Painless, firm, movable,measuring around 0.5 to2 cm.

Located over extensorsurfaces of the joints,particularly knees, wristsand elbows

Erythema Marginatum


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Erythema MarginatumWhat Pediatrician should know ?

erythematous lesions

with pale centers and

rounded or serpiginous

margins

Laboratory Investigations:
http://www.emedicine.com/med/images/Large/1567ARF_Erythema_marginatum.jpg


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Laboratory Investigations:What Pediatrician should know ?

No specific laboratory investigations

1. Acute phase reactant

(CRP, SAA, SAP, Complements, CoagulationProteins)

2. Serologis and bacteriologis (ASO, Anti-

DNAse B titres, Culture)3. Electrocardiography, radiology,

echocardiograpphy

Differential diagnosis of rheumatic fever


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Differential diagnosis of rheumatic feverWhat Pediatrician should know ?

* Rheumatic fever and rheumatoid arthritis are

completely different diseases although both

are immmunologically mediated diseases.

* But remmember R.F. is more serious and

more important as it can be prevented.


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MANAGEMENT

Step 0 : Primordial preventionStep I :Primary prevention

(eradication of streptococci)

Step II :Anti inflammatory treatment(aspirin,steroids)

Step III: Supportive management &

management of complications

Step IV:Secondary prevention(prevention of recurrent attacks)


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Step 0:Primordial Prevention

* Primordial prevention of the disease- Immunization (?)

- Socio economic

- Nutrition

- Public education (school going age, parents,teachers, all personel involve with children, etc)

* Control spread of disease to others

- Reduce risk of cross-transmission of organisms

- Infection control policies

- Handwashing

- Overcrowding

- Availability to prompt medical care

STEP I: P i P ti f


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STEP I:Primary Prevention ofRheumatic Fever

* The most important way to prevent

rheumatic fever is by proper and

prompt treatment of GABHS throat.

* Identification & Eradication of

GABHS

Step II Id tifi ti d t t t


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Anti inflamatory* Definite CarditisNo cardiomegaly

Salicylates 100 mg/kg/day

-In one or two weeks, reduce to 75 mg/kg/day

-Continue for 6 - 8 weeks

-Shift to prednisone if cardiomegaly develops

* Severe CarditisCardiomegaly or CHF

Prednisone 1 - 2 mg/kg/day for 2 - 4 weeks

-Begin Salicylates in final weeks of prednisone

and continue for 68 weeks

Step II: Identification and treatmentof ARFand RHD

Step II : Identification and treatment


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Step II: Identification and treatmentof ARFand RHD

Antibiotic regimensANTIBIOTIC ERADICATION REGIMEN

Benzathine penicillin,

imBodyweight < 27 kg 600,000 IU x 1

Bodyweight < 27 kg 1.2 MIU x 1

Penicillin V, oral 100,000 IU/kg/day for 10 daysin 3 doses/day

Erythromycin 50 mg/kg/day

in 3 doses/day for 10 days

The roles for antibiotics


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The roles for antibioticsin Rheumatic Fever

(1) initially treat GABHS pharyngitis

(2) prevent recurrent streptococcal

pharyngitis, RF, and RHD

(3) provide prophylaxis against bacterial

endocarditis.

Supportive & management ofStep III:


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Bed rest

Treatment of congestive cardiac failure:

-digitalis,ace inhibitor, diuretics

Treatment of chorea:

-diazepam or haloperidol Rest to joints & supportive splinting

Supportive & managementofcomplications

Step III:


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VariableAfter 6

months

After 10

weeks

After 6

8

weeks

All

Activties

3 months or

longer

3 months4 weeks3 weeksOutdoor

3 months6 weeks3 weeks2 weeksIndoor

36 months6 weeks3 weeks2 weeksBed Rest

Carditis; withenlargementCarditis; NoenlargementMinimalCarditisArthritis

Bed rest in Rheumatic Fever


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Activity :

->Initially, on bed rest, a period of indoor activity

,permitted to return to school.

->Do not allow full activity directly.

->Patients with chorea may require a wheelchair.



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Recommendat ions of American Heart Ass ociat ion



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Treatment of congestive cardiac failure:Recommendat ions of American Heart Ass ociat ion

STEP IV : Secondary Prophylaxis of RF


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STEP IV:Secondary Prophylaxis of RF(Prevention of Recurrent Attacks)

Agent Dose Mode

Benzathine penicillin G 1 200 000 U every 4 weeks* Intramuscular

or

Penicillin V 250 mg twice daily Oral

or

Sulfadiazine 0.5 g once daily for patients 27 kg (60 lb Oral1.0 g once daily for patients >27 kg (60 lb)

For individuals allergic to penicillin and sulfadiazine

Erythromycin 250 mg twice daily Oral

*In high-risk situations, administration every 3 weeks is justified andrecommended


F ti f S d P h l i


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Function of Secondary Prophylaxis

The regular administration of antibiotics

Prevents GABHS infections

(which can result in recurrent ARF)

Reduces the severity of RHD

Helps prevent death from severe RHD.


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Prevention of bacterial endocarditis


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What Paediatrician should know ?

* Patients who had RF without valve damagedo not need endocarditis prophylaxis.

* Do not use penicillin, ampicillin, or amoxicillinfor endocarditis prophylaxis in patients alreadyreceiving penicillin for secondary RF

prophylaxis (relative resistance of oralstreptococci to penicillin and aminopenicillins).

Prevention of bacterial endocarditis


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* Alternate drugs recommended by the

American Heart Association for these

patients include oral clindamycin

(children: 20 mg/kg; adults: 600 mg)

and

oral azithromycinor clarithromycin

(children: 15 mg/kg; adults: 500 mg)

Prevention of bacterial endocarditisWhat Paediatrician should know ?


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Surgical vs Non Surgical Care:

* Surgery for patients who remain symptomatic

despite medical management.

* Critical MS : valvotomy / valve replacement(


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Diet:

* Without restrictions except in patientswith CHF, who should follow a fluid-restricted and sodium-restricted diet.

* Potassium supplementation(mineralocorticoid effect of corticosteroid

and the diuretics)


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Patient Education:

->Timely evaluation and treatment ofpharyngitis in children.

->Secondary prophylaxis.

->Additional prophylactic antibiotics prior to

dental and surgical procedures.

Complications:


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Complications:

* CHF from valve insufficiency (acute RF) or

stenosis (chronic RF).

* Atrial arrhythmias

* Pulmonary edema

* Recurrent pulmonary emboli

* Infective endocarditis * Thrombus formation

* Systemic emboli.

Rheumatic Fever Prognosis


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Rheumatic Fever - Prognosis

Is good if recurrence is prevented by continuous

antibiotic prophylaxis-particularly if no carditis in theinitial attack.

Can recur if not on prophylactic medicines.

Good prognosis for older age group & if no carditis

during the initial attack.

Bad prognosis for younger children & those with

carditis with valvar lesions.


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Is It Possible to Prevent Rheumatic Fever ?

In the future

Primary prevention will have to wait

till a safe and effective GABHSvaccine becomes available.


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PRIORITY ISSUES TO BE COVERED

SOCIO ECONOMIC STATUS OF THE PEOPLE

EARLY DIAGNOSIS OF THE STREPTOCOCAL THROAT

CASE DETECTION OF ARF

PRIMARY PREVENTION (3 to 15 YEARS OF AGE).

SECONDARY CONTINOUS PROPHYLAXIS (3 to 21/35

years).

REFERRAL SYSTEM, FOLLOW UP AND ADHERENCE


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Rheumatic Fever

A post-infectious connective tissue disease

Follows GAS pharyngitis by 3 weeks (vs. nephritogenic strains ofGAS)

Injury by GAS antibodies cross-reacting with tissue

DxJONES criteria (major and minor)

TestsThroat Cx, ASO titer, CRP, ESR, EKG, +/- ECHO

RxPCN x10 days and high-dose ASA or steroids

2oProphylaxisdaily po PCN or monthly IM PCN


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Jones Modified Criteria


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Rheumatic Carditis Present in 50% cases

Sleeping tachycardia is an early sign

Mitral and aortic valves most commonly involved

Rheumatic Arthritis Most common manifestation Pain, swelling and erythema

Resolves within 1 week

Rheumatic Feverorgans


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gaffected

1. Heart muscle & valvesmyocarditis & endocarditis(pericarditis rare w/o the others)

2. Jointspolyarthritis

3. BrainSydenhams Chorea (milkmaids grip or better yet,

motor impersistance)

4. Skinerythema marginatum (serpiginous border) due to

vasculitis

5. Subcutaneous nodulesnon-tender, mobile and on extensor

surfaces

Rheumatic Fever: The Problem


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Rheumatic Fever:The Problem

#1 Cause of Acquiredheart disease in children.

( world-wide but not in USA )

- AHA: >3200 deaths in US, related to RF/RHD in 2004.

Sequelae of inadequately treatedstrep. pharyngitis.( strep throat )

Highly Uncommon- < 1% of untreated infections.

- Gp A beta-hemolytic - rheumatogenic strainsM proteins.

- 1/3rd

of cases follow inapparent strep infections. A Non-SuppurativeSystemic Inflammatory illness

occuring 1 - 2 wks following a Strep.Infection.

Pathogenesis - Autoimmune mediated.

Multiple systems affected. (Joints, Skin, CNS & Heart !)

Primarily affects: 515 year old age group.


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Rheumatic Fever: Clinical FeaturesPolyarthritis w/ low grade fever, large joints, ( > 75%)

migratory - often 1 at a time, w/ no permanent dysfx.

Carditis - pericarditis, cardiomegaly, or valvulitis ( ~ 50%)

(valvulitis is the most seriousmanifestation.)

Chorealate occurrence, 3 - 4 months after ( ~ 10%)

infection, self-limiting, resolves in 1- 3 months.

Erythema Marginatumclassic truncal rash, ( ~ 10%)

migratory - appears & disappears within hours.

(pink rashirregular red edgesclear center)

Subcutaneous Noduleslate occurs late (1 - 2%)

( months after infection), painless small nodules

over bony prominences - elbows, knees, spine.


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Rheumatic Fever: Jones Criteria*

(Reqs: 2 Major or1 Major & 2 Minor)

Major MinorPolyarthritis Arthralgia

Carditis Prolonged PR interval

Chorea Elevated CRP, ESR

Erythema marginatum Fever (1012 F)

Subcutaneous Nodules Elevated WBC

* with (+) evidence of a prior strep. infection

( incrd ASO or anti-DNAse AB)

or Hx of (+) C/S or Rapid Strep Test

Rheumatic Fever: Prevention


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Rheumatic Fever: Prevention

ANTIBIOTICS - 2 TYPES of USE:

PrimaryPrevention - Appropriate detection

& treatment of Strep. Pharyngitis.

SecondaryPrevention - Patients with Hx

of Rheumatic Fever require continuous

prophylactic antibiotics due to:

1) increased susceptibilityto recurrences

2)increased severityof recurrences, &

3) asymptomaticnature of Strep. Infections


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Rheumatic FeverPrimaryPrevention:

ANTIBIOTICS: Therapeutic Course

IM - Benzathine Penicillin Drug of Choice !0.6 MU IM 1Time (< 27 Kg or60 lbs)

1.2 MU IM 1Time ( >27 Kg or60 lbs)

PO - Phenoxymethyl Penicillin (Pen VK)

Children ( 40mg/kg/day ) 250 mg B-TID x10 days

Adolescent /Adult 500 mg B-TID x10 days

( See Strep.Pharyngitis for alternatives )

ANTI-INFLAMMATORY AGENT: ASA or CCS

SYMPTOMATIC TX: for CHF or Chorea

BEDREST: Limited physical activity


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( )


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Acute Pharyngitis (AP): Background

GaBHS *- most common Bacterialcause of AP.

Majority of AP cases are Viral. Age Relationship: GaBHS accounts for ONLY;

20 - 30 % of AP in children 5 - 15 yrs old

10 - 20 % in adoles./adults 15 - 35 yrs

5 - 10 % after 35 yrs

Seasonal: winter and early spring.

~ 75% of patients seen in primary care settings

receive ABX Rxs for AP. ( 6.7 million visits/yr )

Also causes Skin InfectionsImpetigo/Pyoderma

( * GaBHS = Gp A beta-hemolytic strep. )aka: strep pyogenes


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Nimishikavi S, Stead L Streptococcal PharyngitisImages in Clinical Medicine.

NEJM 2005: 352:e10.


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Acute Pharyngitis:Clinical Features

Suggest Bacterial Suggest Viral

( GaBHS )

Sudden Onset Sore throat Conjunctivitis

Pain on Swallowing Runny Nose

Fever ( 101-104 F) Cough

Headache DiarrheaN/V & Abdominal Pain Hoarseness

(+) Cervical Nodes

Pharyngeal / Tonsillar

Erythema & Exudates


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Strep. Pharyngitis: Diagnostic Options

Throat Culture- Gold Standard ( read at 24 & 48 hrs )

High Specificity & High Sensitivity

- True positives / Few Falsenegatives

Rapid Antigen Detection Test(RADT) - Detects GaBHS CHO.

High Specificity & but only Good Marginal Sensitivity.

- True positives / Many Falsenegatives

Strept Antibody test - detects ASO & anti-DNAse ABs.

- No immediate value in deciding treatment.

Recommended approach:

(+) C/S or RADT confirms presence of GaBHS = Treat !

In child or adoles.- (-) RADTneeds C/S confirmation.

In adults, (-) RADT doesnt require C/S confirmation.

No Method Identifies GaBHS Carriers with Viral AP

$$$$$$ - Lots of testing ???


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Strep. Pharyngitis:First Line Treatment Options

Child - Penicillin VK 250 mg B-TID x 10 daysAmoxicillin 20-40 mg/kg/day divd TID x 10 days

Benzathine Pen G 600,000 units IM 1X ( 60lbs)

Erythomycin 250mg QID / 500mg BID x 10 days

STREP. PHARYNGITIS:


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DOSE MAX/DAY

Cefixime 8 mg/kg/day div q1224h 400 mg

Cephalexin 25 - 50 mg/kg/day div B-QID 2000 mg

Cefadroxil 30 mg/kg/day div BID 2000 mg

Clindamycin 20-30 mg/kg/day div T-QID 1800 mg

Azithromycin 12 mg/kg/day QD X5 days 500 mg

(>2y/o)

Cefdinir 14 mg/kg/day div BID X5 days 600 mg

Cefpodoxime 10 mg/kg/day div BID X5 days 400 mg

STREP. PHARYNGITIS:AlternativeTreatment Options

Pedi


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STREP. PHARYNGITIS:AlternativeTreatment Options

Adults/ Adolescents >12 yoa or > 40Kg

DOSE MAX/DAY

Cefixime 400 mg QD 400 mg

Cephalexin 250-500 mg QID 2000 mg

Cefadroxil 1-2 gms/day div Q12-24hr 2000 mgClindamycin 300-450 mg T-QID 1800 mg

Azithromycin 500 mg day 1, then

250 mg QD X4 days

Cefdinir 300 mg BID X5 days 600 mgCefpodoxime 200400 mg BID X5 days 800 mg


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Antibiotics NOTRecommendedfor Strep. Pharyngitis:

Sulfonamides

Trimethoprim / Sulfamethoxazole

Fluoroquinolones

Tetracyclines / Doxycycline /

Minocycline

References


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References

Endocarditis:

-Wilson W et al. AHA GuidelinePrevention of Infective Endocarditis.(A guideline from the AHA Rheumatic Fever, Endocarditis, &

Kawasaki Disease Committee, Council on Cardiovascular Disease

in the Young, & Council on Clinical Cardiology, Council on

Cardiovascular Surgery & Anesthesia, & the Quality of Care &

Outcomes Research Interdisciplinary Working Group.)

Circulation 2007;116:1736-54.

-Dajani AS et al. Prevention of Bacterial EndocarditisRecommendations

by the American Heart Association. JAMA 1997; 277: 1794-1801.

- Brook MM. Pediatric bacterial endocardiotis: treatment and prophylaxis.

Pediatric Clinics of North America 1999; 46: 275-87.

References


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References

Rheumatic Fever:

-Dajani AS et al. Guideline for the Diagnosis of Rheumatic Fever: JonesCriteria, Updated 1992. The Committee on Rheumatic Fever,

Endocarditis, & Kawasaki Disease of the Council on Cardiovascular

Disease in the Young. Amer Heart Ass. Circulation 1993;87:302-7.

- Ferrieri P. AHA Scientific Statement - Proceedings of the Jones Criteria

Workshop. Circulation 2002;106:25213.

- Saxena A. Treatment of Rheumatic CarditisSymposium on Pediatric Cardiology. Indian J

of Peds 2002;69:513-6.

References


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Streptococcal Pharyngitis:

- Bisno AL et al. Practice Guidelines for the Diagnosis and Managementof Group A Streptococcal Pharyngitis. (IDSA Guidelines)

Clinical Infectious Diseases 2002; 35:113-25.

- The American Heart Association. Treatment of Acute Streptococcal

Pharyngitis and Prevention of Rheumatic FeverA Statement for

Health Professionals. Pediatrics 1995;96:758-764.

- McIsaac WJ et al. Empirical Validation of Guidelines for the management

of Pharyngitis in children and adults. JAMA 2004;291:1587-95.

- Shulman ST et al. So whats wrong with penicillin for strep throat?

Pediatrics 2004; 113: 1816-19.

References


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