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ACUTE RHEUMATIC FEVER + INFECTIVE ENDOCARDITIS

Malak Abu-aqulah & Rahaf Hasan

6

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ACUTE RHEUMATIC FEVER

PS. At least there will be 6 questions about these two lectures inthe exam

In the past there were a lot of children have tonsillitis, their

parents may give them poached eggs or lemon or whatever and

after a period of time the child will develop joint pain, it was very

common (rheumatic fever), but now when the child have feverthey give him good antibiotic so the rheumatic fever significantly

decreased especially in Jordan.

Rheumatic feveris an acute systemic immune disease, when

bacteria attack the throat specifically tonsils the body start to

form antibodies against those bacteria and the result will be

immune complex, this immune complex has many complications,

the most important is pancarditis.

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The bacteria that is responsible to cause rheumatic fever is Group

A beta- hemolytic Streptococcal infection in the pharynx mainly

in tonsils, the same bacteria may attack the skin and cause scarlet

fever and skin lesions but it will not cause rheumatic fever, it mustbe in the throat.

There was a question about this point last year

Rheumatic fever with its immune complex has many

complications the most important one affects the heart, also

another complications on joints, skin, subcutaneous tissue and

brain.

In sum the immune complex form nodules called aschoff nodules

it depends where these nodules reach then it will cause

symptoms.

At the beginning the child have sore throat, tonsillitis and fever,

after a period of time there will be joint pain, joint pain usually

not important although it may develop to acute mono-arthritis itdoes not leave any deformities in the bone, just we are concern

about knowing that it is rheumatic fever to prevent pancarditis.

The incubation period for this disease about 2-3 weeks, the child

might have sore throat and tonsillitis and it will be subsided after

taking simple antibiotic, after 2-3 weeks incubation period joint

pain may reveal with the symptoms of rheumatic fever.

The most common ages affected by rheumatic fever are the

children between 5-15 years old, so its peak incidence 5-15 yrs

old, rarely we see patients pre to this period < 4 or after 40 yrs

old.

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Question: Why 5-15 yrs old is the most common ages to develop

rheumatic fever?

First of all we have to know that rheumatic fever cause

destruction to the main mitral (tricuspid) valve in the heart.

Answer: There are many theories to explain that, one of them

said that the structure of the mitral valve between 5-15 yrs old

exactly similar to the structure of the bacteria!, so the antibodies

attack against the bacteria and the heart. Before 4 yrs old and

after 40 yrs old the structure is different; the structure of the

tricuspid valve is different from the structure of the bacteria.

Another theory said that the immune complex end to have fibrin

and anti-inflammatory cells over it which will form aschoff

nodules which will cause many things that we will see later on.

It is not necessary that everyone have infection in beta hemolytic

anemia will develop rheumatic fever, it is just up to 3% even now

it is less than that.

Question: Does every patient or child come to us with sore throat

or tonsillitis and a little joint pain mean that he has rheumatic

fever?

Answer: no it doesnt.

We have criteria help establishing rheumatic fever called modified

JONES CRITERIA

*We have major and minor, minor criteria are not important but if

we have two major it is rheumatic fever

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MODIFIED JONES CRITERIA

Major criteria

Polyarthritis Carditis

Chorea

Subcutaneous nodules

Erythema marginatum

Polyarthritis usually happens on large joints like ankle and knee,

but it will not lead to any deformity, migratory; patient has pain inleft knee and after 2-3 days it will subside and begin in the right

ankle.

Carditis it may cause pancarditis, patient might come with severe

heart failure and it may lead to death.

Chorea involuntary coarse movement: the name refers to monk

his name is SYDENHAM CHOREA who had his own dance withstrange random movements difficult to describe and dont have

any specific characteristics.

Subcutaneous nodules: which are the aschoffs nodules

composed of immune complex with collagen, fibrin and

inflammatory cells, might be subcutaneous and we can feel it.

Erythema marginatum: redness in the skin which will expand and

the center of it will disappear and improve eventually forming a

ring and the center of it will be normal skin.

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Minor criteria

Fever

Polyarthralgia

Previous rheumatic fever or heart disease Prolongation in P-R interval Increase in ESR Positive ASO Positive throat culture

Fever, anyone with infection will have fever.

Polyarthralgia,joint pain.

Previous rheumatic fever or heart disease, if we do ECG for the

patient, the PQR complex between P-R interval should be less

than 0.2 or less than large square on the ECG paper. But with

patient has rheumatic fever as a result of infection there will be

an effect on AV node which is responsible in controlling heart

rate, may cause elongation in P-R interval.

ESR: as a dentist you have to do it, any patient complain from

anything with high ESR, this is significant, so means that if ESR

high there is something wrong.

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If ESR above 100 it is alarm sign, because most of malignancies

associated with ESR above 100.

*So 40, 60 or 80 it is normal.

How do we perform the ESR test?

ESR: elimination sedimentation rate, we put a sample of blood in

a tube in the laboratory and then we see during the first hour how

much there is separation between RBCs and plasma, it is

measured by milliliter.

ASOanti-streptolysin O if it positive that means there isinfection

Positive throat culture; we take swap culture from throat, it will

give streptococcal antigen, rarely we do it because the mouth full

of flora, so with normal patient it may give positive culture.

*DR return to the major criteria.

Polyarthritis it is migratory may be sever bone for example at

day one there is severe knee pain and in the second day in the

ankle and the third day in the wrist, so it is called flitting and

fleeting; means it will not lead to any effect.

It affects mainly large joints not small ones like fingers.

*rheumatoid arthritis is another disease affect

small joints like in fingers not large ones and

cause deformity*

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It affect large joints sequentially as we said 2-3 days pain in the

right ankle then disappears completely and develop again in the

left ankle.

Usually affect just a single joint in adults 25-30 yrs old just knee

pain or ankle pain.

Pain last for 1-5 weeks then it will be disappeared there Is no

more joint pain.

Happens with 75% of patients who have rheumatoid arthritis (I

think it is rheumatic fever)

If we have 2 major criteria or more with some minor then we can

say that it is rheumatic fever.

(but may we have rheumatic fever without any arthritis)

It subsided without any residual deformity leaving normal joints.

If there is sever joint pain, Rheumatic response to aspirin and non

steroidal so the pain will completely disappear.

Carditis

Most likely happened in children, above 80 yrs old rheumatic

fever is not as harmful as it happens in the childhood, because the

structure of the valve and the myocardium differ from the that ofbacteria.

It is a difficult issue to say this myocarditis or pancarditis as simple

as that.

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It happens in one third of cases while polyarthritis happens in

75% of case two thirds or more, here (carditis) less than

polyarthritis.

We have to have one of these:

Mitral regurg MR or aortic regurgAR murmur (normal valve

but dilated ventricle)

As we know each ventricle connected by two valves (mitral

valve in the left ventricle and tricuspid valve in the right

ventricle, aortic valve from the left ventricle, and pulmonary

valve from the right. The normal blood circulation we have to

know about it. Any new murmur (abnormal heart sound) dueto dilated ventricle lead to dilated valve and mitral or tricuspid

regurg, may we have short diastolic murmur, means there are

more blood flow during the valve it may be due to high volume

state or due to mitral regurg itself.

Change in quality of heart sound (just you have to know about

it but not to practice it), you cannot gauge this is abnormal

heart sound even practitioner cannot know if it diastolic or

systolic murmur in some cases.

Tachycardia even at rest. The normal heart rate between 60-

100 if we have heart rate more than 100 this is tachycardia,

below 60 this is bradycardia.

Cardiomegaly on chest X-ray or on echo (echo-cardiogram).

Pericarditis, picture of pericarditis (retrosternal chest pain,

increase the respiration associated with diffuse ST changes in

the ECG, it is acute pericarditis and pericardial effusion.

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ECG changes, changes in contour of P wave due to

depolarization and contraction of both atria (p wave means

atrial contraction), if we have dilated ventricle, mitral regurgand tricuspid regurg may we have dilated atria and the P wave

will be changed in shape and size. Inversion of T-waves (it must

be positive and must go with the QRS direction, but in this case

we may have inverted T wave). Prolonged PR interval as we

said before.

SYDENHAMS CHOREA

Involuntary choreo- athetoid movements (like ballet dance he

move one hand alone then he start to move the other).

It exists in 50% of patients.

So as we said the most common is polyarthritis 75%, pancarditis

or cadiac involvement in one third, and here (Sydenhams chorea)

in 50% of cases.

Girls affected more frequently than males.

*And as we said all these manifestations are rare in adult patients

Erythema Marginatum

There is ring enlarges slowly and there is pale area in the center,

may be raised over the skin and can feel it, most likely it is

transient means after a period it will disappear but in some cases

it may be persistent and continue to be there.

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Subcutaneous Nodule

Feel it on the external joints like elbow, wrist or knee. Small fairnontender , rarely see it in adults it is childhood disease, it is not

painful important point, attached to fascia or tendon sheaths

over bony prominences so it can move, where there is

prominence bone over the joints we see nodules ,it may persist

for days or weeks, recurrent may disappear and come back again.

Indistinguishable from rheumatoid nodules, there is another

disease rheumatoid arthritis it affects the small joints and sharesome manifestations with rheumatic fever and one of these is

subcutaneous nodules.

To reach a diagnosis we have to have two major

criteria or one major with two minor

Differential diagnosis

Rheumatoid arthritis involving the small joints with deformities,patients affected by it cannot do anything by their hands

Osteomyelitis Endocarditis Chronic meningococcemia SLE systemic lupus erethromatosus, very bad disease

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Lyme disease Sickle cell disease

Surgical abdomen

The question about them in the exam may come as :All of the following is considered as differential diagnoses

except

You dont have to know the details about them just knowthem by name ^_^

Treatment

The most important thing is to eradicate the bacteria from the

pharynx, otherwise accordingly if the patient has pancarditis we

do bed rest, give him non-steroidal or steroids.

Polyarthritis: non-steroidal or high dose aspirin, their action like a

magic with it.

But again the most important thing to prevent rheumatic fever

and to prevent another attack, each attack take more risk to have

complications, because of that children with documented

rheumatic fever we should give them Benzathene penicillin 1.2

million units, sometimes in two weeks or monthly for at least 5years after the last attack or till age of 18 yrs old and in some

cases till 25 yrs old (monthly needle injection till 25 yrs old).

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*If the patient has penicillin allergy we give him erythromycin or

oral penicillin not important point

If we leave the patient without any treatment the sequence will

not affect the joint but it will destroy the heart valves.

As a result if we dont make a good treatment with good

antibiotic we may have rheumatic heart disease.

Rheumatic heart disease: destruction of the heart valves, the

bacteria beta-hemolytic streptococcus eventually the immunecomplex will destruct mainly the mitral valve (between the left

atrium and the left ventricle).

In majority of case 50% mitral valve alone involved, but mitral +

aortic in 25% of cases. Pure aortic uncommon, to have patients

with pure aortic stenosis due to rheumatic fever it is rare. If we

have patient 35 yrs old with mitral stenosis always we ask about

history of rheumatic fever or history of recurrent tonsillitis.

Patient presented with sever mitral stenosis or mitral regurg, in

about 60% they remember that they had some joint pain or

tonsillitis or recurrent tonsillitis while he/she was a child.

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Let me sum up with you the main points in the lecture:

Rheumatic fever is an acute systemic immune disease, affect thepharynx

Caused by Group A beta- hemolytic Streptococcus bacteriaMost important complication is pancarditis cause of formation of

aschoff nodules

Incubation period is 2-3 weeksPeak incidence 5-15 yrs old, rare 403% of people develop rheumatic feverMODIFIED JONES CRITERIA-major criteria

Polyarthrits affect75%, pain last for 1-5 weeks, 2-3 daysmigratory between large joints

Carditis affect1/3 of cases, MR or AR, tachycardia, cardiomegaly,abnormal heart sounds, pericarditis, changes in contour of Pwave, inversion of T wave, prolonged P-R interval

SYDENHAMS CHOREA, affect 50% of cases Subcutaneous Nodule, affect external joints, non painful, nontender,

recurrent, indistinguishable from those of rheumatoid arthritisnodules

Erythema marginatumMODIFIED JONES CRITERIA-minor criteria

Fever Polyarthralgia Previous rheumatic fever or heart disease Prolongation in P-R interval Increase in ESR, above 100 Positive ASO Positive throat culture, not commonly do it

To reach a diagnosis we have to have two major criteria or one majorwith two minor

Refer to page 11 to know the Differential diagnosisTreatment, Benzathene penicillin 1.2 million units, at least for 5 years

End of part 1

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Infective Endocarditis

As we said One of the causesof Infectious Endocarditis isRHEUMATIC

FEVER, when there is a destructive valve

now, let's start :

(IE): an infection of the heartsEndocarditisInfectious

endocardial surface

There are many Classification of Infectious Endocarditis

We Classify IE into four groups:firstly,

i. Native Valve IE

ii. Prosthetic Valve IEiii. Intravenous drug abuse (IVDA) IE, as Addiction on heroiniv. Nosocomial IE

Why were classified in this way?

There are different bacteria organisms

1-The patients with IV drug uses have Staphylococci auras withdestructive tricuspid valve.

2- The patient with mitral valve rarely seen severe Infectious

Endocarditis.

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3-The patient with Prosthetic Valve IE have vegetation prosthesis.

4-Patients in Nosocomial IE have very aggressive bacteria usually

MRSA.

Further Classification:

i.Acute IE

ii.Sub-acute IE

How to differentiate between Acute IE and Sub-acute IE?

Sub acute IEoften affects damaged heart valves with RF , mitral stenosis, mitral leakages thicken valve

Acute IEaffects normal heart valves Commonly Staph. Rapidlydestructive valveIf not treated, usually fatal within 6 weeks

seen patient with Acute IE when they areRarely , wecarefulBe

already taken available antibiotics like Ampicillin, Amoxicillin,

Zinnate

Etiology( Important)::

*Native valve Endocarditis- Streptococcus viridians,Staphylococci, HACEK -*

*Prosthetic Valve Endocarditis- Coagulase negativeStaphylococci, S.Aureus (very bad bacteria as when we make

gastric band patient will died from S .Aurous pneumonia

exposure)

*IV Drug abuse endocarditis:- Tricuspid valve ,MRSA

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*Nosocomial- we see in patient with Infectious Endocarditis inhospital , pacemaker lead- and/or implanted defibrillator

associated endocarditis , also in depleted patient (long time

central line patient )

5-15% may be culture negative?? Although blood culture is a

because of priorEndocardiaInfectioussitmajors criteria to say

HACEKantibiotic exposure and there's a special organism like

group need special media to growth.

Pathophysiology:

Which patient susceptibility more to have Infectious

Endocarditis?

Turbulent blood flow, when we have mitral stenosis, calcified

mitral valve or mitral leakage the blood flow from atrium to

ventricles or from ventricle to aortic will have bad way which

lead injury to valve if we have wound it will form a clot why this

clot isnt inside a vessel? To prevent bacteria to catch theinternal part of vessel, also injury to valve turbulent blood flow

make a good area to colonization a bacteria.

Bacteremia, the most important sources of Bacteremia a tooth

brush, mouth bacteria that go inside blood stream iftheres no

suitable area to attack, it will go to spleen and destructed. ,but

if have suitable area to attack like (mitral ring, aortic ring ),itwill adhesive and form vegetation with bacteria, fibrin,

inflammatory cell ,WBC SO we have amass called vegetation

with Eventual invasion of the valvular leaflets

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:So,, Pathophysiology

We have Turbulent blood flow disrupts the endocardium makingit sticky

Bacteremia delivers the organisms to the endocardial surfaceAdherence of the organisms to the endocardial surfaceEventual invasion of the valvular leaflets

As the result

Turbulent blood flow causes endothelial injury- direct infection

theres

OR

Nonbacterial thrombotic endocarditic s as in- platelet fibrin thrombus

which seen in some cases of vegetation without endocarditic mainly in

CLEpatient

Epidemiology

Much more common in males than in femalesMay occur in persons of any age and increasingly common in

elderly

Mortality ranges from 20-30%

Risk Factors

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Intravenous drug abuse, with recurrent skin infection and

destructive tricuspid valve

Artificial heart valves , (prostheses valve ) and pacemakers

Acquired heart defects, especially with Turbulent blood flow

and injury to intimae of the valveCalcific aortic stenosis

Mitral valve prolapsed with regurgitation up to 50%Congenital heart defects, patient with VCD more common

associated with Infectious Endocarditic while patient with ACD

must associated with other Congenital heart defects to cause

Infectious EndocarditisIntravascular catheters

Symptoms:

Symptoms of infection fever, myalgia, Abdominal pain, joint pain,

back pain, Leukocytosis without obvious source, the onset of

symptoms is usually ~2 weeks or less from the initiating bacteremia,

and not responding to antibiotic

Signs

(the doctor search it)

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Cardiac Manifestations

New regurgitant murmurs-

CHF- valvular damage (aortic), myocarditis-

Perivalvular abscess-

-Fistulae connection between cardiac chambers right and left

-Pericarditis

Heart block/ MI due to embolic phenomena-

-Anemia ,it a chronic process with

more than 2 week fever for

unknown origin so we chickInfectious Endocarditis

-Microscopic hematuria

Elevated ESR, CRP-

-Decreased serum complement

-Immune complexes

-Rheumatoid factor in patient with

RHEUMATIC ARTHRITIS will be

positive while in patient with

Infectious Endocarditic it will be

positive due to Immune complexes

-Fever

-Clubbing

Splenomegaly-

Neurological manifestations-

-Heart murmur abnormal heart

sound

-Peripheral manifestations-

Oslers nodes, Subungual

hemorrhage, Janeway lesions

- Leukocytosis

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in chest x ray Septic Pulmonary Emboli with some infraction in lung

manifestationsNon cardiac

In general in asystemic dieses when there are vegetation with

abesses in heart and showing Emboli ,they will go to brain with

nurigical manifestations, to lung with abscess and cause ischemic

toes subungual hemorrhageJaneway noduls and glomunthrits in

kidney(if it immune complex or vegetation)

Petechiae

Nonspecific (without any causes )Often located on extremities or mucous membranes mainly hard

palate ,skin, congigtiva

plinter HemorrhagesS

NonspecificNon blanching, dont disappear while we pressesLinear reddish-brown lesions found under the nail bedUsually do NOT extend the entire length of the nail

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Mechanism:

-Vessel damage from swelling of the blood vessels (vacuities)

from immune complex

-Tiny clots that damage the small capillaries (micro emboli) from

vegetation (source infection).

-Oslers Nodes

More specific rarely seen with other dieses so if we have mostlyassociated with Infectious Endocarditis

Painful and erythematous nodulesLocated on pulp of fingers and toesMore common in subacute IEMechanism :: immune complexJaneway Lesions

More specific-Erythematous, blanching macules -painful-Located on palms and soles--Mechanism: micro abscess of the dermis with marked necrosis and

inflammatory infiltrate not involving the epidermis ( vegetation)

yesthe eretina ofRoth Spots mainly seen in the**

http://en.wikipedia.org/wiki/Dermishttp://en.wikipedia.org/wiki/Necrosishttp://en.wikipedia.org/w/index.php?title=Epidermis(skin)&action=edit&redlink=1http://en.wikipedia.org/w/index.php?title=Epidermis(skin)&action=edit&redlink=1http://en.wikipedia.org/wiki/Necrosishttp://en.wikipedia.org/wiki/Dermis

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The Essential Blood Test

Blood Cultures

Minimum of three separate blood cultures-

-Three separate venipuncture sites (not in same syringe and then

disrupted in bottles)

- At least 1 hour apart- over 24 hours from certain person from

hospital they can deal with it (you have to have sterile gloves, gown,

and bottles aerobic or non-aerobic with specific media for bacteria)-Serology- bacteria dont deal with them -Imaging

*Chest x-ray as we said,more commonly, the chest x-ray may

reveal septic pulmonary emboli in a patient

*ECG Rarely diagnostic prosthetic valve but if we have

complete heart block or bradycardiain in infectious

Endocarditic may a signe of abbesses (infection) spread to AV

node so may have bradycardiain or tachycardia arrhythmias

Echocardiography*

Indications for Echocardiography

simple, non invasion,:thoracic echocardiography (TTE)Trans*

cheap test ,TTE has superior sensitivity, especially in detecting

native valve vegetations no need to further test , while in the

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prosthetic valve vegetation with echogenicity have a difficulty to

judge normal or not because of their artificial prosthetic valve so

we do what called Trans-esophageal echocardiography (TEE)

:esophageal echocardiography (TEE)-Trans*

- High risk patients, we have highly subspecialty of prosthetic

valve but in Trans-esophageal Echocardiograph there no more

important information

-Intra-cardiac complications, we can't say never ever this is

abbesses, fistulaetc. If there is any suspicion of IE, get a TTE.

-Inadequate TTE if we have patient with long standing

smoking, inflated lung (difficulty in window), obesity >100 kg

-Fungal or S.aureus or bacteremia , in Fungal infection there's a

difficulty in differentiation between thrombus and vegetations, he

saw thrombus with 4x4 cm so if I have fever or suspicion Infectious

Endocarditic, I can't say its thrombus or Fungal infection with larger

vegetation so we need Transthoracic echocardiography(TTE) to

judge .

Modified Duke Criteria (important)

These criteria are sensitive and specific and very rarely reject a

true endocarditis:

Definite IE*

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-Microorganism (via culture or histology) in a valvular vegetation,

embolized vegetation, or intra-cardiac abscess

Histologic evidence of vegetation or intra-cardiac abscess-

2 Major

1 Major + 3 minors

5 Minor

Possible IE*

1 major and 1 minor

3 minor

Treatment

6 weeks (long stay in hospital)forparenteral antibiotics-

-Prolonged treatment to kill dormant bacteria clustered in

vegetation (broad spectrum)

Costly-

So you need to be accurate in Infectious Endocarditis**

Poor Prognostic Factors

Female, more aggressive in male than female-.S.aureus ,you will have small abscess with very aggressive

bacteria

Vegetation size, when you have just 0.8 cm it easier to deal withmore than when we have multiple vegetation valves.

Aortic valve, more dangerous than mitral and tricuspid valvebecause its out flow tract so the blood will take fewer bacteria to

the body to brain.

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Prosthetic valve because there's no blood flow in Prosthetic valveso most likely we need to put another prostheses with a result

increasing in mortality rate

Low serum albumen they happen with liver problem , Diabetes,can't even eat patient

Paravalvular abscess, as a rule if we have abcsses and giveantibiotic we will never cure we need to drain it, so in

Paravalvular abscess when the abscess between valve and wall

most likely to do incision.

Embolic eventsOlder age

Good luck ^_^Malak Abu-aqulah

Rahaf Hasan