RHEUMATIC FEVER RHEUMATIC FEVER

PROF.DR. AYÇA VİTRİNEL

A postinfectious, immune-mediated, inflammatory reaction that affects the connective tissue of multiple organ systems (heart, joints, central nervous system, blood vessels, subcutaneous tissue)and that follows infection with certain strains of group A beta-hemolytic streptococci(GABHS).

In the developing world, acute rheumatic fever and rheumatic heart diseases are the leading causes of cardiovascular death during the first five decades of life

ETIOLOGY GROUP A ß HEMOLYTIC STREPTO-

COCCUS. Certain serotypes ( M-types 1,3,5*,6,14,18,

19,24) are more frequently isolated from patients with ARF than other serotypes.

ARF; most frequently observed in the age group susceptible to GABHS infections, children from 5-15 years of age. Increased numbers of cases also occur in socially and economically disadvantaged group

ETIOLOGY

The increased incidence of GABHS pha-rangitis in the fall, winter and early spring is associated with an increased number of cases of ARF during these same periods of the year.

GABHS impetigo doesn’t result in ARF the reasons for this aren’t fully understood;

Differences of rheumatogenic potential of skin strains and throat strains as well as observed difference in the immunologic response to GABHS impetigo compared with URI with GABHS

Group A ß HS

PATHOLOGY

1) Exudative, degenerative-» Reversible.fibrinoid,T-lenf,B-lenf,macrophage,mast cells

2) Proliferative-» Aschoff nodules.Fibrinoid center+multinuclear cells

Attack rate of ARF after URI ; individual of untreated, inadequately treated, strain of infecting organism, certain host factors.

PATHOLOGY Aschoff nodules

Aschoff nodules

PATHOLOGY

Two basic theories for development of ARF:

-TOXIC EFFECT: produced by an extracel-lular toxin of GABHS on target organs; myocardium, valves, synovium, brain.(Streptolysin S and O)

-ABNORMAL IMMUNE RESPONSE: The resulting Abs cause the immunological damage ; clinical manifestations.

PATHOLOGY Polisaccaride/group spesific carbohydrates have

similarities with valves ( endocardium).

The possibility of abnormal immune response is

based on cross reactivity between group A streptococcus M protein and human tissue

Hyaluranate has a similar antigenic properties with cartilage tissue.

M protein shares certain aminoacid sequences with some human tissues and this has been proposed as a possible source of cross reactivity between the microorganism and its host leading to the abnormal immune response .(myocardium)

PATHOLOGY Some people appear to be susceptible to

developing ARF.(genetic predisposition) A spesific alloantigen is present on the

surface of non T lymphocytes in 70-90% of rheumatic individuals but fewer than 30% of control nonrheumatic individuals.

The marker is more common in families in which there’s an index case of RF than in nonaffected members of control families.

In Turkey ARA is mostly seen in patients with HLA A20 and HLA DRw 11

CLINICAL MANIFESTATIONS

CARDITIS: PANCARDITIS; pericardium, epicardium, myocardium and endocardium

Residual of ARF -» chronic changes.Valvular insufficiency = most frequently mitral valve, aortic valve.---» later stenosis of the valve.

Occurs 40-80% of patients with RF. Pericarditis. Arrythmias Cardiomegaly

New murmurs appear within the first 2 weeks in 80% of patients, and they rarely occur after the second month of illness.

Hence, during an episode, one normal echocardiogram in the first 2 weeks should be sufficient to eliminate carditis

Rheumatic carditis (verrucae)

Mitral stenosis

Rheumatic carditis (before treatment)

Rheumatic carditis (posttreatment-4 wk later)

CLINICAL MANIFESTATIONS

POLYARTHRITIS: Tender, joints are red, warm and swallen. Migratory, several dif-ferent joints; elbows, knees, ankles, wrists. Need not be symmetric. Doesn’t result in chronic joint disease

untreated it may persist for a week.

CLINICAL MANIFESTATIONS

CHOREA: Occurs much later than other manifestations. (as long as several months)

Marked deterioration is handwriting. Emotional lability Affect all four extremities or may be unila-

teral. Frequently the only symptom of RF Usually disappears within weeks to

months. Dissapears during sleeping

CLINICAL MANIFESTATIONS

ERYTHEMA MARGINATUM: Occurs very infrequently, nonspesific pink macules

over the trunk can be made worse with application of

heat migratuar.

ERYTHEMA MARGINATUM

CLINICAL MANIFESTATIONS

SUBCUTAN NODULES: Infrequently and are most commonly observed in patients with severe carditis.

Nontender No inflammation Seen on the extansor surfaces of the joints

such as knees, elbows, over the spine

SUBCUTAN NODULES

CLINICAL MANIFESTATIONS

MINOR MANIFESTATIONS: FEVER: 37.8 C- 40 °C

ARTHRALGIA

DIAGNOSIS

DIAGNOSIS

Two major criteria OR one major + two minor criteria ; + evidence of preeceding strep infection.

Indicate high probability of RF

In three situtations ARF may be diagnosed in the absence of these rules:

1) Chorea (if other cause have been pre-clude.

2) Insidious or late onset of carditis with no other explanation

3) Rheumatic reccurence

DIAGNOSIS

WHO CRITERIA (2002-2003) FIRST ATTACK: JONES CRITERIA RECURRENCE: R.H.D. (-): first attack criteria R.H.D. (+): 2 minor criteria + eviden-

ce of preceeding strep. İnfection.

Differential diagnosis of rheumatic fever

Juvenile rheumatoid arthritis Systemic lupus erythematosus Infective endocarditis Reactive arthritis Sickle cell disease Drug reactions Other connective tissue diseases Septicaemia Leukaemia Gonoccocal arthritis Tuberculosis Lyme disease Serum sickness

LABORATORY FINDINGS

Throat culture ASO, reaches its peak 3-6 weeks after

infection. ESR CRP ECG Chest Roentgram ECHO

Rheumatic carditis (ECG)

COMPLICATIONS

Development of rheumatic valvular heart disease.

TREATMENT

1) Treatment of GABHS 2) Antiinflamatuar agents 3) Supportive therapy (therapy of conges-

tive heart failure)

TREATMENT

1) Ten full days of an appropriate oral agent

A single IM injection of 1.200.000 U of benzathin penicillin G

2) Arthritis salicylates 75-120 mg/kg/day in 4 divided doses

Mild carditis: salicylates

TREATMENT

2)Congestive hearth failure/significant manifestations of carditis

CORTICOSTEROIDS ( 2 mg/kg/day prednisone (d2) 2-3 wk and taper the drug (add salicylates during tapering)

3)SYDENHAM CHOREA: Phenobarbital, cholorpromazine, benzodiazepine, halope-ridol.

PREVENTION

PRIMARY PROPHYLAXIS: Antibiotic treatment of strep URI.

SECONDARY PROPHYLAXIS: Prevention of colonisation or infection of URI with GABHS:

IM B.pen. Every 3-4 wk Oral pen V 250 mg twice daily Sulfadiazine 500 mg daily Erytromycin 250 g twice daily

PREVENTION

The necessary duration of secondary prophylaxis--» 5 years or reaching 21. birthday

Carditis without residual heart disease-10 yrs or 21 years age

Residual rheumatic heart disease--» 10 years or until age 40.

Severe heart disease or risc of continous exposure- lifelong.