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v
Acute (often recurrent)
inflammatory disease that follows pharyngitis with group A- β
hemolytic strep.
v
Children 5-15 yrs.
v
Associated with poor
social conditions & overcrowding.
v
Immune response to
strep. antigens à cross-reaction with tissues lined endothelium (e.g. blood vessels,
endocardium, pericardium, synovial membranes) & myocardium in
susceptible individuals.
v
2-3 weeks after
pharyngitis with strep à 3% children à RF.
Pathology
In
acute RF:
o
Aschoff
bodies in myocardium:
Fibrinoid
necrosis surrounded by lymphocytes, Macrophages, plasma cells,
histiocytes.
o
Presence
of small warty (verrucae) endocardial vegetations:
esp.
on left side valves (mitral & aortic) along chordae tendinae.
In chronic RHD:
o
Irregular thickening +
fibrosis + calcification of leaflets with thickening, shortening,
fusion of chordae tendinae.
o
50% with acute RF with
carditis à
chronic RHD after 10-20 yrs, affecting Mitral >> Aortic
valves.
Clinical
Features
Disease
presents suddenly with fever, joint pains, malaise, loss of
appetite.
Table.
Jones criteria for Diagnosis of RF.
Diagnosis is made on basis of ≥ 2 major criteria,
or, one major + ≥ 2 minor criteria.
Major
criteria
q
Carditis (50%).
q
Migratory polyarthritis of large joints.
q
Sydenham’s chorea (15%).
q
Erythema marginatum of skin (20%).
q
Subcutaneous nodules.
q
Fever
q
Arthralgia
q
Previous RF
q
↑ ESR / CRP
Carditis [myo-, peri-,
endocarditis] manifests as:
q
New / changed heart
murmurs.
q
Development of
cardiomegaly / cardiac failure.
q
Transient diastolic
mitral (Carey-Coombs) murmur; due to mitral vavulitis.
Arthritis (80%):
q
Signs of inflammation.
q
Flitting = fleeting =
migratory:
Inflammation
in affected joint à
disappears à
another joint inflames.
Sydenham’s chorea
[St. Vitus’ dance]:
q
Spasmodic involuntary
movements.
Skin
Erythema
marginatum:
q
Transient pink painless
rashes in trunk & limbs (never the face).
Subcutaneous
nodules:
q
Hard, asymptomatic,
painless, beneath skin, over tendons, joints, scalp.
Investigations
õ
Throat
swabs:
Culture
on selective BA à
β Hemolytic
colonies à
identify by Lancefield grouping.
õ
Serology:
↑ ASO, anti-streptokinase titer.
õ
Non-specific
indicators of inflammation:
↑ ESR, CRP.
Complications
of Chronic Rheumatic Heart Disease:
1.
Bacterial
endocarditis:
Scarred
valves are very susceptible to bacterial colonization i.e. after
episodes of bacteremia (e.g. during dental procedures).
2.
Mural
thrombosis à
systemic emboli.
3.
Heart
Failure.
Treatment
q
Patients with fever,
active arthritis / active carditis à
complete bed rest.
q
Salicylates &
corticosteroids.
q
Penicillin for:
§
Eradication of residual
strep. infection.
§
Prophylaxis in people
with previous RF (dental / surgical procedures)
à
to prevent bacterial endocarditis.
written
by:
Khalid Bin Yaroof. FMHS, UAE University.
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