RHEUMATIC FEVER

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INTRODUCTION

• Rheumatic fever is an inflammatory disease that may develop

after an infection with Streptococcus bacteria (such as strep

throat or scarlet fever).

• The disease is a multisystem inflammatory disease which can

affect the heart, joints, skin, and brain.

• It is an immunologically mediated.

• It can be acute and chronic.

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ETIOLOGY

• Rheumatic fever results from an inflammatory reaction to certain Group A Streptococcus bacteria.

• CAUTION! Monitor That Sore Throat• Pay attention to sore throats, especially in children. If your

child has a severe sore throat without other cold symptoms, accompanied by a fever higher than 101 degrees, or a milder sore throat that persists for more than two or three days, see a doctor. It may be strep throat, which should be treated with antibiotics.

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EPIDEMIOLOGY

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PATHOGENESIS

• Group A Beta Hemolytic Streptococcus:

– Strains that produces rheumatic fever - M types l, 3, 5, 6,18 & 24

– Pharyngitis : produced by GABHS can lead to- acute rheumatic

fever, rheumatic heart disease & post strept. Glomerulonepritis

– Skin infection- produced by GABHS leads to post streptococcal

glomerulo nephritis only. It will not result in Rh.Fever or carditis as

skin lipid cholesterol inhibit antigenicity.

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PATHOGENESIS

• Delayed immune response to infection with group.A beta hemolytic

streptococci.

• After a latent period of 1-3 weeks, antibody induced immunological

damage occur to :

– heart valves

– joints

– subcutaneous tissue

– Basal ganglia of brain

•

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CLINICAL MANIFESTATION

• Following upper airway infection

• Silent period of 2 - 6 weeks

• Sudden onset of fever, pallor,

malaise, fatigue, arthralgia,

leucocytosis.

• Characterized by:– Arthritis– Carditis– Sydenham’s chorea– Erythema marginatum– Subcutaneous nodules– Called “major

manifestations” of Jones criteria

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ARTHRITIS

• Most common feature: present in 80% of patients

• Painful, migratory, short duration, excellent response of salicylates

• Usually >5 joints affected and large joints preferred

• Knees, ankles, wrists, elbows, shoulders

• Small joints and cervical spine less commonly involved

• Arthritis do not progress to chronic disease

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CARDITIS

• Manifest as pancarditis (endocarditis, myocarditis and pericarditis),occur in 40-50% of cases

• Carditis is the only manifestation of rheumatic fever that leaves a sequelae & permanent damage to the organ

• Chronic phase- fibrosis, calcification & stenosis of heart valves(fishmouth valves)

• PATHOLOGIC LESIONS:– Fibrinoid degeneration of connective tissue , inflammatory edema,

inflammatory cell infiltration & proliferation of specific cells resulting in formation of Ashcoff nodules mainly found in myocardium and subcutaneous.

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Ashcoff nodules

• Ashcoff nodules:– Pathogonomonic for RF.– Consist of a central zone of degenerating hypereosinophilic

extracellula rmatrix infiltrated by lymphocytes, plasma cells, plump activated macrophages which is know as ANTISCHKOW CELLS.

• Antischkow cells:– Abundant cytoplasm– Central nuclei with chromatin Arrayed in a slender, wavy ribbon( so

called caterpillar cells)– These cells can fuse and form GIANT cell.

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SYDENHAM’S CHOREA• Causes

– Sydenham chorea is one of the major signs of acute rheumatic fever.– It is because of the damage in the BASAL GANGLIA of the brain.

Sydenham chorea occurs most often in girls before puberty, but may be seen in boys. Resolve completely with no cerebral damage.

• Symptoms– Changes in handwriting– Jerky, uncontrollable, and purposeless movements in different

muscle groups (looks like twitching)– Loss of fine motor control, especially of the fingers and hands– Loss of emotional control, with bouts of inappropriate crying or

laughing

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ERTHYEMA MARGINATUM

• There are light pink macules spreading outwards with a serpiginous, well-demarcated edge and clearing central portion.

• Pale center with red irregular margin. More on trunks & limbs & non-itchy.

• The rash changes from hour to hour and may seem to appear, disappear or move so rapidly that it can almost be seen doing so.

• It often involves multiple areas, usually on the trunk and occasionally over the proximal parts of the limbs.

• It is exacerbated by heat and fades when the patient is cool.

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SUBCUTANEOUS NODULES

• Occur in 10%• Painless , pea-sized , palpable nodules• Usually 0.5 - 2 cm long• Firm, non-tender, isolated or in clusters• Most common: along extensor surfaces of joint, Knees, elbows, wrists• Also: on bony prominences, tendons, dorsi of feet, or cervical spine• Last a few days only, with complete resolution

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LABORATORY FINDINGS

• High ESR

• Anemia, leucocytosis

• Elevated C-reactive protien

• ASO titre >200 Todd units.(Peak value attained at 3 weeks , then comes

down to normal by 6 weeks)

• Anti-DNAse B test

• Throat culture-GABHstreptococci

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DIAGONOSIS

• Jones criteria:– Criteria developed to prevent over diagnosis– Still important as guidelines– Probability of Acute Rheumatic Fever is high with:-– Evidence of previous infection with streptococcal upper airway

infection and– 2 major criteria or 1 major criteria and 2 minor criteria

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TREATMENT

• Treatment of Streptococcal Tonsillopharyngitis:– Penicillin– Erythromycin

• Anti inflammatory treatment:– Arthritis

• Aspirin– Carditis

• Prednisolone– Chorea

• diazepam or haloperidol • Prevention of Recurrent Attacks:

– Penicillin– Erythromycin

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