Содержание

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Rheumatic fever is an inflammatory disease involving the joints, the heart,


Rheumatic fever
is an inflammatory disease involving the joints, the

heart, the CNS, the skin and subcutaneous tissue.
It is:
Serious → as it leads to
permanent cardiac damage
(chronic valvular disease)
Important cause →
of acquired heart disease
in children in developing
countries.
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Etiology Non-suppurative complications of upper respiratory infections by group A-β hemolytic

Etiology


Non-suppurative complications of upper respiratory infections by
group A-β hemolytic

streptococcal (GAS)
Skin infections by GAS → acute glomerulonephritis but rarely, if ever to acute RF.
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Pathogenesis *Autoimmune theory, abnormal immune response by human host to some

Pathogenesis
*Autoimmune theory, abnormal immune response by human host to some component

of GAS.
* The resulting antibodies → immunologic damage.
Latent period is 1-3 weeks
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Epidemiology Rheumatic fever occurs at all ages peaks between 5-15 ys

Epidemiology

Rheumatic fever occurs at all ages peaks between 5-15 ys
A

high incidence in tropical and subtropical climates.
Over crowding, poor housing, lack of adequate treatment and genetic predisposition
are predisposing factors
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Epidemiology Low-Risk populations: Those with incidence ≤2 per 100,000 school-age children

Epidemiology

Low-Risk populations:
Those with incidence ≤2 per 100,000 school-age children per

year or all-age rheumatic heart disease
prevalence of ≤1 per thousand population.
Include virtually all of the United States, Canada, and Western Europe.
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Epidemiology High-Risk populations: Those with incidence >2 per 100,000 school-age children

Epidemiology

High-Risk populations:
Those with incidence >2 per 100,000 school-age children per

year or all-age rheumatic heart disease
prevalence of >1 per thousand population.
Include Maoris in New Zealand, aborigines in Australia, Pacific Islanders, and most developing countries.
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Clinical manifestations and diagnosis 2015 Revised Jones Criteria for diagnosis of Rheumatic Fever

Clinical manifestations and diagnosis 2015 Revised Jones Criteria for diagnosis of Rheumatic

Fever
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Clinical manifestations and diagnosis 2015 Revised Jones Criteria for diagnosis of Rheumatic Fever

Clinical manifestations and diagnosis 2015 Revised Jones Criteria for diagnosis of Rheumatic

Fever
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Clinical manifestations and diagnosis 2015 Revised Jones Criteria for diagnosis of Rheumatic Fever

Clinical manifestations and diagnosis 2015 Revised Jones Criteria for diagnosis of Rheumatic

Fever
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Clinical manifestations and diagnosis 2015 Revised Jones Criteria for diagnosis of

Clinical manifestations and diagnosis 2015 Revised Jones Criteria for diagnosis of Rheumatic

Fever

Subclinical carditis indicates echocardiographic valvulitis.
Erythema marginatum and subcutaneous nodules are rarely “standalone” major criteria.
Joint manifestations can only be considered in either the major or minor categories but not both in the same patient.

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Clinical manifestations and diagnosis 2015 Revised Jones Criteria for diagnosis of Rheumatic Fever

Clinical manifestations and diagnosis 2015 Revised Jones Criteria for diagnosis of Rheumatic

Fever
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Clinical manifestations and diagnosis: 2015 Revised Jones Criteria for diagnosis of Rheumatic Fever

Clinical manifestations and diagnosis: 2015 Revised Jones Criteria for diagnosis of Rheumatic

Fever
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Clinical manifestations and diagnosis 2015 Revised Jones Criteria for diagnosis of

Clinical manifestations and diagnosis 2015 Revised Jones Criteria for diagnosis of Rheumatic

Fever

CRP value must be greater than upper limit of normal for laboratory.
Because ESR may evolve during the course of ARF, peak ESR values should be used.

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Clinical manifestations and diagnosis 2015 Revised Jones Criteria for diagnosis of

Clinical manifestations and diagnosis 2015 Revised Jones Criteria for diagnosis of Rheumatic

Fever

Prolonged P-R interval [in the ECG] should not be counted as a minor manifestation in patients in whom carditis is counted as a major manifestation.
A prolonged P-R interval alone does not constitute evidence of carditis or predict long-term cardiac sequelae.

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Clinical manifestations and diagnosis 2015 Revised Jones Criteria for diagnosis of Rheumatic Fever

Clinical manifestations and diagnosis 2015 Revised Jones Criteria for diagnosis of Rheumatic

Fever
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Clinical manifestations and diagnosis 2015 Revised Jones Criteria for diagnosis of Rheumatic Fever

Clinical manifestations and diagnosis 2015 Revised Jones Criteria for diagnosis of Rheumatic

Fever
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Guidelines for the diagnosis of initial or recurrent attack of RF Revised Jones criteria, Updated 2015

Guidelines for the diagnosis of initial or recurrent attack of RF

Revised Jones criteria, Updated 2015
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Major Jones criteria

Major
Jones
criteria

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Minor Jones criteria

Minor
Jones
criteria

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Rheumatic Carditis Most serious manifestations of ARF Occurs in about 50-60%

Rheumatic Carditis
Most serious manifestations of ARF
Occurs in about 50-60% of all

cases of ARF
Pancarditis involves endocardium, myocardium and pericardium
Result in residual chronic valvular lesion
Rheumatic Subclinical Carditis is carditis without a murmur of valvulitis but with echocardiographic evidence of valvulitis.
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Endocarditis (valvulitis) * Numerous small rheumatic sterile vegetation on the line

Endocarditis (valvulitis)
* Numerous small rheumatic
sterile vegetation on the line

of closure of the valves
* Mitral valve is commonly
affected followed by the
Aortic valve
* Valvular regurge is characteristic of ARF
* Valvular stenosis usually appears several years after
the ARF
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Murmurs in patients with endocarditis soft - musical - not associated

Murmurs in patients with endocarditis
soft - musical - not associated with

thrill
1- Apical pansystolic murmur, musical
soft, radiating to axilla, changeable, not associated with thrill, caused by mitral valvitis → (MR)
disappears within 6 months if not associated with chronic MR
2- Apical low-pitched mid-diastolic murmur → (MS)
(Carey Coombs murmur).
3- Early diastolic murmur over the aortic area → (AR)
4- Appearance of new murmurs.
5- Change in character of previous murmurs.
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Myocarditis Cardiac dilatation Congestive heart failure Tachycardia disproportionate to fever Arrhythmias Muffled Heart sounds

Myocarditis

Cardiac dilatation
Congestive heart failure
Tachycardia disproportionate to fever
Arrhythmias
Muffled Heart sounds

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Pericarditis Dry pericarditis precordial pain and friction rub Wet Pericarditis mild to moderate effusion

Pericarditis
Dry pericarditis
precordial pain and friction rub
Wet Pericarditis
mild to moderate

effusion
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Pericarditis with massive pericardial effusion: Weak pulse. Pulsus paradoxus is an

Pericarditis with massive pericardial effusion:
Weak pulse.
Pulsus paradoxus
is an abnormally large

decrease in systolic BP during inspiration. The normal fall in systolic BP is <10 mmHg. When the drop is >10mmHg, it is referred to as pulsus paradoxus. 
Congested non pulsating neck veins.
Weak apical pulsation
Heart sounds are distant and muffled.
Dullness outside the apex
Ewart’s sign
compression of the left lung produces
dullness and bronchial breathing at the
lung base posteriorly.
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DD of rheumatic carditis: Other causes of myocarditis such as viral

DD of rheumatic carditis:
Other causes of myocarditis such as viral myocarditis.
Other

causes of pericarditis.
Infective endocarditis.
Congenital heart disease.
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Treatment of carditis * Mild cases without HF or cardiomegaly Salicylates

Treatment of carditis
* Mild cases without HF or cardiomegaly
Salicylates

50-70mg/kg/day orally after meal divided into 4 doses for 3-5 days, then 50 mg/kg/day divided into 4 doses for 3 weeks, then 25 mg/kg/day for 3 weeks
* Moderate and severe cases with cardiomegaly and/or HF:
Bed rest
- Prednisone 2 mg/kg/day divided in 4 doses for 2-3 wk., then half the dose for 2-3 weeks
When the patient responds clinically & on lab tests (ESR, CRP), the dose should be tapered by reducing 5 mg/day every 2-3 days.
-Salicylates 50mg/kg/day in 4 divided doses for 6 weeks at the beginning of tapering steroid dose to prevent rebound
-Supportive therapies include digoxin, fluid, salt restriction, diuretics and O2.
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Rheumatic Polyarthritis -Migratory (from one joint to another)& affects several joints.

Rheumatic Polyarthritis

-Migratory (from one joint to
another)& affects several joints.
-Involves large

joints.
-Mainly symmetrical.
-The joints are tender, red, warm and swollen.
-Effusion may be present.
-Does not result in chronic joint disease.
-Aspirin or NSAI give dramatic response
in 12-24 hours.
-Arthralgia may occur in some joints
and frank arthritis in others.
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Differential diagnosis: Other causes of arthritis - Juvenile rheumatoid arthritis &

Differential diagnosis:

Other causes of arthritis
- Juvenile rheumatoid arthritis & other collagen

diseases.
- Infective endocarditis.
- Arthritis. Of infection
- Malignancy as Leukemia.
- Sickle cell disease.
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Treatment of arthritis Salicylates 50-70mg/kg/day orally after meal divided into 4

Treatment of arthritis

Salicylates 50-70mg/kg/day orally after meal divided into

4 doses for 3-5 days, then 50 mg/kg/day divided into 4 doses for 3 weeks, and 25 mg/kg/day for 3 weeks.
Early administration of salicylates to a patient before diagnosis is established may obscure the diagnosis.
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Rheumatic Chorea (Sydenham chorea) Has long latent period (2-6 months), the

Rheumatic Chorea (Sydenham chorea)

Has long latent period (2-6 months), the

onset is usually insidious.
May be the only sign of rheumatic fever (pure chorea) or it may be associated with carditis.
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Clinical manifestation: - Characterized by involuntary movements, emotional liability & hypotonia.

Clinical manifestation:
- Characterized by involuntary movements, emotional liability & hypotonia.
-

Purposeless, irregular, rapid, jerking movements of the limbs and grimacing of the face
- Exaggerated by emotional stress and disappear during sleep.
- Drop things, spill from a cup and handwriting deteriorates.
- Speech is commonly slurred.
- May affects one side of the body (hemichorea).
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Sydenhams chorea watch please

Sydenhams chorea watch please

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Clinical tests for detection of chorea Marked fluctuation in muscle tone

Clinical tests for detection of chorea

Marked fluctuation in muscle tone


(felt by asking the patient to squeeze the examiner’s hand).
Spooning:
When the tongue is protruded
it is rapidly withdrawn to prevent being bitten by involuntary jaw movements.
The knee jerk:
either of pendulum type(due to hypotonia) or more commonly is sustained or “hung up”.
Pronation sign:
on elevation of the upper limbs above the level of the head with the palms of hands facing each other, there is pronation in the forearms and the limbs fall down gradually.
Examination of hand writing for fine motor movement
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In pure chorea the ESR and ASOT are normal. This is

In pure chorea the ESR and ASOT are normal. This is

attributed to the long latent period (2-6 months), when elevated loog for carditis
DD
Postencephalitic chorea
Cerebral palsy
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Prognosis Chorea is a self limited condition. Mild cases subside within

Prognosis

Chorea is a self limited condition.
Mild cases subside within few weeks

- 3 months
Severe cases may progress and require a padded cot.
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Treatment of rheumatic chorea - Anti inflamatory - Phenobarbital - Haloperidol - Chlorpromazine

Treatment of rheumatic chorea

- Anti inflamatory
- Phenobarbital
-

Haloperidol
- Chlorpromazine
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Subcutaneous nodules Bilaterally symmetrical firm nodules varying in diameter from few

Subcutaneous nodules

Bilaterally symmetrical firm
nodules varying in diameter from

few millimeters to centimeter.
Movable painless and not tender.
Occur over the bony prominences.
Best demonstrated by fully flexing
the joint and stretching the skin over
the extensor surface.
When occurs, usually severe carditis
is present.
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Erythema marginatum Red, raised, non pruritic macules extend to form wavy

Erythema marginatum

Red, raised, non pruritic macules extend to form wavy lines

or rings with pale centers .
Coalesce forming irregular patterns, which vary in shape, and site from hour to hour.
Usually seen over the trunk.
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Complications of Acute Rheumatic fever Chronic valvular heart disease (RHD) after

Complications of Acute Rheumatic fever

Chronic valvular heart disease (RHD) after

an attack of rheumatic carditis.
Severe acute carditis is the commonest cause of death of rheumatic fever.
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Prevention of rheumatic fever can be divided into three approaches General measures Primary prevention Secondary orevention

Prevention of rheumatic fever can be divided into three approaches

General measures
Primary prevention
Secondary

orevention
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1. Treatment (eradication ) of GAS infection Treatment of streptococcal upper

1. Treatment (eradication ) of GAS infection
Treatment of streptococcal upper respiratory

tract infection must be within 9 days to prevent an initial attack of rheumatic fever.
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Primary prevention eradication of GAS all patients with ARF should receive

Primary prevention eradication of GAS all patients with ARF should receive

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Secondary prevention (for recurrences of acute rheumatic fever)

Secondary prevention (for recurrences of acute rheumatic fever)

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Duration of Secondary prevention

Duration of Secondary prevention