Rheumatic Fever

Hamzeh Al-Rashdan
Abdullah Al-Shorman


Thursday 29/11/2012
Today lecture about acute Rheumatic Fever ,its very easy lecture and won't take
more than 30 min.
The doctor was reading from the slides. Good luck.

Common streptococci:
The most common Streptococcus species isolated from humans are as follows
1) Streptococcus Pyogenes
2) Streptococcus Agalactiae
3) Streptococcus bovis
4) Streptococcus pneumoniae
5) and the Streptococcus Viridans group.
Streptococcus Pyogenes is a beta hemolytic streptococci and cause bacterial
phyrangitis.
It also causes Scarlet fever due to the erythrogenic toxin . They are also
known as "flesh eating" bacteria and can cause life-threatening disease,
necrotising facitis.it also causes Skin infections and Streptococcal toxic shock
syndrome.

this is simple phyrangitis can be caused by bacterial or viral infection.

This is pus formation from the tonsils ,most likely caused by Streptococcus Pyogenes

This is maculopapular rash caused by Streptococcus . its scarlt fever

This is Necrotising fasciitis ,the worst Streptococcal infection

and this is skin infection by Streptococcus Pyogenes

Acute rheumatic fever is a systemic disease of childhood, often recurrent that follows
group A beta hemolytic streptococcal infection
It is a delayed non-suppurative sequelae to URTI with GABH streptococci.

 It is a diffuse inflammatory disease of connective tissue,primarily involving

heart,blood vessels,joints, subcut.tissue and CNS .

Epidemiology :

Children between 5-15 years most affected

Its rare in children less than 3 years
Girls affected mora than boys
Common in developing countries because of overcrowding , poor sanitation
and poverty ,these factor increase the risk of sterp.cocus infection
Incidence more during fall, winter & early spring because recurrent infection in
these period.

Pathogenesis :
The pathogenesis of acute Rh.fever is Delayed immune response to group.A beta
hemolytic streptococci. After a latent period of 1-3 weeks, and there is antibody
induced immunological damage occur to heart valves, joints, subcutaneous tissue
& basal ganglia of brain.
GABHS strains that cause ARF are: M types l, 3, 5, 6,18 & 24
Pharyngitis- produced by GABHS can lead to:
Post sterp.coccal glomerulo niphritis
Acute Rh.fever
Rheumatic heart disease
Skin infection- produced by GABHS leads to :

post streptococcal glomerulo nephritis only.

It will not result in Rh.Fever or carditis because skin lipid cholesterol will
inhibit antigenicity.

Refer to slide 16# to see the structure of GABSH

Antigen of outer protein cell wall of GABHS induces antibody response in victim
which result in autoimmune damage to heart valves, sub cutaneous
tissue,tendons, joints & basal ganglia of brain .
The pathologic lesion of acute Rh.fever is Fibrinoid degeneration of connective
tissue,inflammatory edema, inflammatory cell infiltration & proliferation of
specific cells resulting in formation of Ashcoff nodules that can be seen in :

Pancaritis
Artharits
Ashcoff nodules in the subcutaneous tissue
Basal ganglia lesions resulting in chora

Major Clinical features of acute Rh.fever :

1) Arthritis :arthritis of acute Rh.fever is migratory fleeting polyarthritis
which mean involving major joints and transmitted from one joint to
another, the most common joint the involved is the knee, ankle, elbow and
wrist.
Occur in 80% of rh.fever,involved joints are exquisitely tender.
In children below 5 yrs arthritis usually mild but carditis more prominent.
Arthritis do not progress to chronic disease
So we have 2 features of arthritis in rh.fever :

Migratory:in first 24hr afeecting the knee,then the other knee then elbow
and its not chronic

2) carditis : cardaitis in rh.fever manifest as pancarditis(endocarditis,

myocarditis and pericarditis).
Carditis is the only manifestation of rheumatic fever that leaves a
sequelae & permanent damage to the organ.
Valvulitis occur in acute phase.
Chronic phase- fibrosis,calcification & stenosis of heart
valves(fishmouth valves).
3) Sydenham Chorea:
is a disease characterized by rapid, uncoordinated jerking movements affecting
primarily the face, feet and handswiki

o Occur in 5-10% of cases.

Mainly in girls of 1-15 yrs age
May appear even 6-12 after the attack of rheumatic fever.
Clinically manifest as-clumsiness, deterioration of handwriting, emotional
liability or grimacing of face
The best examination of Sydenham Chorea is standing and asking the patient to
put his button on/off.

 Clinical signs: pronator sign, milking sign of hands .

"milk sign", which is a relapsing grip demonstrated by alternate increases and
decreases in tension . wiki

This is the abnormal movment in Sydenham Chorea we can see while the patient
is writing or walking or putting his button on.

4) Erythema Marginatum :
o Occur in less than 5%.
Unique,transient,serpiginous-looking lesions of 1-2 inches in size
Pale center with red irregular margin
More on trunks & limbs & non-itchy
Worsens with application of heat
Often associated with chronic carditis

05/05/1999

This is Erythema Marginatum

24

5)Subcutaneous nodules :

Occur in 10%
Painless,pea-sized,palpable nodules
Mainly over extensor surfaces of joints,spine,scapulae & scalp
Associated with strong seropositivity
Always associated with severe carditis.

Minor features of rh.fever :

A.
B.
C.
D.
E.

Fever-(up to 38-39 )
Arthralgia
Pallor
Anorexia
Loss of weight

Laboratory finding :

High ESR
Anemia, leucocytosis
Elevated C-reactive protien
ASO titre >200 Todd units.
Anti-DNAse B test (+)
Throat culture-GABHstreptococci (+)because this is delayed inflammatory
reaction after 1-3 weeks.
ECG- prolonged PR interval, 2nd or 3rd degree blocks,ST depression,T
inversion
2-dimention Echo cardiography- valve edema,mitral regurgitation, LA & LV
dilatation,pericardial effusion,decreased contractility.

How to diagnos rh, fever?

Mainly clinical diagnosis, No single diagnostic sign or specific laboratory
test available for diagnosis.
Diagnosis based on MODIFIED JONES CRITERIA

Jones Criteria (Revised) for Guidance in

the Diagnosis of Rheumatic Fever*
Major Manifestation
Carditis
Polyarthritis
Chorea
Erythema Marginatum
Subcutaneous Nodules

Minor
Manifestations
Clinical
Previous
rheumatic
fever or
rheumatic
heart disease
Arthralgia
Fever

Laboratory
Acute phase
reactants:
Erythrocyte
sedimentation
rate,
C-reactive
protein,
leukocytosis
Prolonged PR interval

Supporting Evidence
of Streptococal Infection
Increased Titer of AntiStreptococcal Antibodies ASO
(anti-streptolysin O),

others
Positive Throat Culture
for Group A Streptococcus
Recent Scarlet Fever

*The presence of two major criteria, or of one major and two minor
criteria, indicates a high probability of acute rheumatic fever, if
supported by evidence of Group A streptococcal nfection.

Exceptions to Jones Criteria:

1) Chorea alone, -if other causes have been excluded-as we said chorea can
happen after 6-12 months of rh.fever.
2) Insidious or late-onset carditis with no other explanation

3) Patients with documented RHD or prior rheumatic fever,one major

criterion,or of fever,arthralgia or high CRP suggests recurrence

Differential Diagnosis :

1. Juvenile rheumatiod arthritis

2. Septic arthritis
3. Sickle-cell arthropathy
4. Kawasaki disease
5. Myocarditis

6. Scarlet fever
7. Leukemia

Treatment:

we have 4 steps :

Step 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).

STEP I: Primary Prevention of Rheumatic Fever (Treatment of Streptococcal

Tonsillopharyngitis)
agent
Benzathine penicillin
G

Penicillin V,

(phenoxymethyl penicillin)

For individuals

allergic to penicillin
Erythromycin:estolate

Dose

600 000 U

for patients 27 kg
(60 lb)

mode
IM

1 200 000 U
for patients >27 kg
Children:

oral
250 mg 2-3 times
daily
Adolescents

and adults: 500 mg

2-3 times daily

duration
once

10 d

20-40

oral

mg/kg/d
2-4 times

daily (maximum 1
g/d)
Ethylsuccinate

40 mg/kg/d
oral

2-4 times daily

(maximum
1 g/d)
This can prevent rh.fever after streptococcus pharyngitis .

10 d

10 d

Step II: Anti inflammatory treatment

If we have Arthritis only

For carditis

Aspirin:75-100mg/kg/day
Gives as 4 divided doses for 6 week
Attain blood level 20-30kg/dl
Predinsolone : 2-2.5mg/kg/day give as
two divided dose for 2 weeks
Taper for 2 week &while tapering add
aspirin 75mg/kg/day for two weeks,
continue aspirin alone 100mg/kg/day for
4 weeks

3.Step III: Supportive management & management of complications

Bed rest : if there is carditis with congestive heart failure we must put the
patient in bed rest
If there with congestive heart failure beside giving pre we must treat
symptom of congestive heart failure by digitalis, diuretics and bed rest .
For chorea we 3 drugs used now : diazepam, haloperidol and valproic acid
We use one of them.
Also Rest to joints & supportive splinting is important.
STEP IV : Secondary Prevention of Rheumatic Fever (Prevention of Recurrent
Attacks)
agent
Benzathine penicillin G
Penicillin V
Sulfadiazine

For individuals allergic to

penicillin and sulfadiazine
Erythromycin

dose
1 200 000 U every 4
weeks*
250 mg twice daily
0.5 g once daily for
patients 27 kg (60 lb)
1.0 g once daily for
patients >27 kg (60 lb)

mode
IM

250 mg twice daily

oral

Oral
oral

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

recommended, such as recurrent pharyngitis and rheumatic heart disease .
*Sulfadiazine can cross allergy with penicillin in 15-20%.

Duration of Secondary Rheumatic Fever Prophylaxis:

category
Rheumatic fever with carditis and
residual heart disease (persistent valvar
disease*)
Rheumatic fever with carditis but NO
residual heart disease (no valvar
disease*)
Rheumatic fever without carditis

duration
At least 10 y since last episode and at
least until age 40 y, sometimes lifelong
prophylaxis
10 y or well into adulthood, whichever
is longer
5 y or until age 21 y ,whichever is
longer

Prognosis :
Rheumatic fever can recur whenever the individual experience new GABH
streptococcal infection, 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
Finally a Student ask if the patient didn't take long acting penicillin for one or two
months, would he develop symptom?
Dr : no he won't develop symptom unless he have another attack of
streptococcus infection this will result in recurrent acute Rh.fever.

THE END

Done by : Hamzeh Al-Rashdan