Prof . El Sayed Abdel Fattah - PowerPoint Presentation

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Prof . El Sayed Abdel Fattah EidLecturer of Internal MedicineDelta University

Acute Rheumatic Fever

Rheumatic fever (RF), also known as acute rheumatic fever (ARF), is an inflammatory disease that can involve the heart, joints,

skin

, and

brain

.

The disease typically develops two to four weeks after pharyngeal infection with group A beta hemolytic Streptococci.

It continues to be a problem worldwide.

EpidemiologyEstimated 30 million people suffer from ongoing heart disease from ARF, 70% dying at average age 35 years old.Usually

occurs in people between 5 and 18 years

old.

Males

and females are equally

affected.

Overcrowding

, poverty, and lack of access to medical

care contribute

to transmission.

Pathogenesis

1- The disease typically develops two to four weeks after pharyngeal infection with group A beta hemolytic

Streptococci.

2- Antibodies made against group A beta hemolytic Streptococci cross-react with human tissue (heart valve and brain).

Clinical FeatureSudden onset of fever, pallor, malaise, fatigue.Characterized by:

Jones criteria:

Major criteria

- Arthritis

-

Carditis

- Sydenham’s chorea

-

Erythema

marginatum

- Subcutaneous

nodues

Minor manifestations- Fever. - Arthralgia. - Elevated C - reactive protein.

- High Erythrocyte sedimentation rate

. -

Prolonged PR interval on ECG.

- Evidence of Previous Infection.

ArthritisMost common feature: present in 80% of patients - Painful, migratory, short duration, excellent response of salicylates

- Usually > 5 joints affected and large joints

prefered

(Knees, ankles, wrists, elbows,

shoulders

).

- Small joints and cervical spine less commonly involved.

Carditis- The most serious manifestation. - Any cardiac tissue may be affected (endocarditis

,

pericarditis

or

myocarditis

)

i.e

(

Pancarditis

)

-

Valvular lesion most common: mitral and aortic developing murmer heart sounds.

Sydenham’s Chorea- Fast, clonic, involuntary movements (especially face and limbs)Muscular hypotonus

, emotional

lability

.

First

sign: difficulty walking, talking, writing and slipping of dishes and

spoons.

Usually

a late manifestation: months after infection

Subcutaneous Nodules

- Usually 0.5 - 2 cm long

- Firm, non-tender, isolated or in clusters.

- Last a few days only, occur in 9 - 20% of cases

Erythema Marginatum- Present in 7% of patients - Highly specific to ARF- Cutaneous

lesion: Reddish pink border with pale center, round or irregular shape

- Often on trunk, abdomen, inner arms, or thighs

- Highly suggestive of

carditis

.

Other Clinical Features (less specific to ARF) Fever, arthralgia or

epistaxis

Abdominal

pain

(5%)

due to peritonitis or

hematuria

(5%)/renal involvement

Pneumonitis

, mild

pleuritis (5 - 10%) or encephalitis (extremely rare)

Diagnois

- Probability of ARF high with evidence of previous infection with streptococcal

upper

airway infection, 2 major criteria; or 1 major criterion and 2 minor criteria.

Laboratory StudiesCBC: not very helpful CRP, ESR: non-specific indicators of inflammation

Tests

for anti-streptococcal

antibody

Chest x-ray.

Echocardiogram

: For

Valvular

lesion and HF.

Treatment Treatment

- Eradication of the group A

strept

.

-

Avoids chronic exposure of immune system to

strept

.

- Single dose IM

benzathine

penicillin G:

< 27 kg: 600,000 units>27 kg: 1,200,000 units Treatment: Arthritis

Salicylates

or NSAIDs x 3 weeks

Usually excellent response (If poor response: diagnosis in question)

TreatmentTreatment: Carditis

- Steroid (Prednisone 1 -2 mg/kg/d (max 60 mg) x 10 - 15 days)

- Taper 20-25% each week.

- For 4 weeks.

Treatment: Sydenham’ s Chorea

- Haloperidol.

- Alternate: Sodium

valproate

.

No proven benefit of steroids

Primary Prophylaxis- Antibiotic use: Systemic Benzathine Penicillin led to impressive fall incidence of ARF - Social and economic factors (improving living conditions, hygiene, overcrowding)

- Access to medical care (Education)

Secondary Prophylaxis

-

Benzathine

Penicillin given to prevent recurrences of ARF

- Prevention of chronic valve disease: <27 kg 600,000 U or> 27 kg 1,200,000 U

- Newer recommendation: every 2 wks for first 2 years then every 3 wks

- Duration: minimum 5 years after last episode or until 21 years old, whatever later

- American heart association recommends: 10 years or until “well into

Adulthood

Artificial heart valves(Prothetic Valve)If Sever Valvular Damage Occurred .Require long-term anticoagulation.

Complications:

1.

Stenosis

2. Thrombosis or calcification.

3.

Hemolysis

.

4.

Endocarditis

.

Thank You