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بسم الله الرحمن الرحيم Rheumatic Fever

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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 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 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 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 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 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

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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

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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

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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 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 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

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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

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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 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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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

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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 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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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. -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 & 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 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 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: 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

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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 attributed to the long latent period (2-6 months), when elevated loog for carditis 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 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

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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 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 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

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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.