Transcript rheumatic fever gg

DR :
Gehan Mohammed
Understand the pathogenesis of rheumatic fever.
 Discuss the Effects of Rheumatic Fever on the three layers of
Heart(endocardium,myocardium,pericardium).
 Identify the organs other than the heart that are affected by
rheumatic fever.
 List the major and minor criteria needed for diagnosis of
rheumatic fever.
Differentiate rheumatic endocarditis from other causes of
endocarditis(Nonbacterial Thrombotic Endocarditis
NBTE, Infective Endocarditis)


Definition: is an acute, immunologically mediated, multisystem inflammatory disease that follows, after a few weeks
of an episode of group A-beta hemolytic streptococcal
infection with cardiac and extra cardiac manifestations.

It is characterized by inflammatory reaction involving heart
60% of patients affected by RF, joints in 75% , central
nervous system in 10 % and skin in 2%.
 Occurs
10 days to 6 weeks after pharyngitis caused by
strept infection so anti streptolysin O (ASO) titer will be
high.
 Peak
incidence: in children 5-15 years.
 Acute
carditis: pericardial friction rubs, weak heart sounds,
tachycardia and arrhythmias.
 Extracardiac:
fever, migratory polyarthritis of large joints,
arthralgia, skin lesions, chorea
 The
causative organisms result in the formation of
antibodies. Then These antibodies cross react with
joints and the heart which may be due to antigenic
similarity between streptococcus and heart tissue.
 Immunologic mechanism
damage to the heart .
is the most likely method of
For diagnosis of rheumatic fever need 2
major or 1 major and 2 minor criteria
Johnes Major criteria of rheumatic fever
 1- Carditis
 2- Migrating Polyarthritis
affect large joints as
knee,ankle which show redness, swollen,hot.
Inflamed joints , self limited, become normal within
1-3 days even without treatments so no chronic
deformities.
 3- Sydenhem’s chorea : a characteristic series of
rapid movements of the face and arms. This
occurs late in the disease
For diagnosis of rheumatic fever need 2
major or 1 major and 2 minor criteria
Major criteria of rheumatic fever
 4- Erythema margination:common
on trunk ,limbs
,not pruritic.
 5- Subcutaneous nodules : appear on the
extensor surface of tendons near bony
prominences.
Strep throat
Antibody
production
Antibody cross-reaction
with heart
vegetations
Aschoff body
pericarditis
Minor criteria of rheumatic fever
 Previous history of
rheumatic fever
 Arthralgia
 Fever
 Lab tests indicative of inflammation : increase
ESR (erythrocyte sedimentation rate), CRP (CReactive protein), leukocytosis
 Echocardiogram (ECG) changes
 It
is a heart disease caused by rheumatic fever.
 Rheumatic
 The
heart disease can be acute or chronic.
incidence and mortality of rheumatic fever has
declined over the past 30 years due to improved
socioeconomic condition and rapid diagnosis and
treatment of group A beta hemolytic streptococcus
infection of the Pharynx or skin.
It affects all the 3 layers of the heart;


Affect the heart during its acute phase  acute rheumatic carditis/
pancarditis (inflammation of endocardium, myocardium and
pericardium)
1- Endocarditis — vegetations due to edema, and fibrin deposits
on valve leaflets along lines of closure. Mostly mitral and aortic
valve.
2 - Myocarditis- Aschoff body which consists of a focus of
necrosis (representing the site of antibody-antigen reaction)
surrounded by activated histiocytes and lymphocytes. The
histiocytes may be mononuclear or multinuclear, and are
referred to as Anitschkow's or Aschoff cells.
3- Pericarditis — "bread and butter appearance", due to
fibrinous inflammation and deposition of fibrin on surface of
pericardium.
 Acute changes may resolve completely or progress to
scarring and development of chronic valvular deformities
many years after the acute disease
Manifests years or decades after the initial episode of
rheumatic fever.
The distribution of valve involvement is
variable:
mitral alone: most common
combined mitral/aortic
pulmonary: very rare
Associated with
usually present.
mitral valve damage is
Aortic
stenosis:
Aortic
regurgitation
Valvular Endocarditis heals by progressive fibrosis leading
to Irreversible deformity in the form of:
a- stenosis (Reduction of diameter): fish mouth (button
hole) stenosis
b- regurgitation (improper closure) : if fibrosis occurred in
chordae tendonae so leaflets are retracted.


affection of the cardiac valves can also lead to cardiac
failure secondary to ventricular hypertrophy then dilatation,
thromboembolism and infective endocarditis,pulmonary
congestion and hypertension.
Signs
and symptoms depend on the valve(s)
involved: cardiac murmurs, ventricular
hypertrophy then dilation ending into heart
failure,
 arrhythmia, thromboembolism and infective
endocarditis.
Treatment may require valve surgery.
Body makes antibody to strep. bacteria that cross-
reacts with antigens in heart and joints
2-3 weeks after strep throat, patient gets:
• migratory polyarthritis
• pericardial friction rub, arrhythmias
Chronic disease can reappear decades later
• mitral stenosis, left atrial enlargement, thrombi
• increased risk of infective endocarditis
Diffferential diagnosis for
rheumatic Endocarditis
Inflammation of the endocardium can also be
caused by:
(A)Non-infective causes:
1-Non-infective thrombotic endocarditis
2- Libmann sacks verrucous endocarditis
(B)Infective causes:
1 -Acute Infective endocarditis
2 -Sub-acute Infective endocarditis

causes:
 endothelial abnormalities ,Hypercoagulability.
 Association with malignancy (50%).
Characterized by the deposition of small masses of fibrin,
platelets, and other blood components on the leaflets of the
cardiac valves (sterile thrombus). There is no infective organism.
It is aseptic.
 Aortic valve most common site.


Clinically asymptomatic, if large: may embolise, may become
infected
Less common, occur in patients with systemic
lupus erythematosus due to deposition of
circulating immune complexes in the valves.
 Definition:
infection of the cardiac valves or mural surface
of the endocardium, resulting in the formation of
vegetations (adherent mass of thrombotic debris and
organisms).
 Divided into:
 Acute infective endocarditis: caused by highly virulent
organisms (staphylococcus aureus)as apart of
septicemia, infect even normal valves, progress rapidly.
 Subacute infective endocarditis : infection of previously
abnormal valves by organisms of low virulence (hemolytic streptococci) as apart of bacteremia
complicating i.v. drug abusers, previous dental, surgical
procedure (catheterization)., progress slowly.
Persons
with cardiac abnormalities:
e.g.chronic valvular diseases, septal
defects etc.
Prosthetic
heart valves.
Intravenous
drug abusers.
 Aortic
and mitral valves are the most common sites
affected while right side valves commonly affected in
i.v.users.
 Vegetations
may be single or multiple, involve one or
more valve(s), differ in appearance according to the
causative agent.
 In
acute IE vegetations may:
a- obstruct valve orifice
b- lead to rupture of the leaflets, cordae
tendineae, or papillary muscles
c- abscess in perivalvular tissue (ring
abscess)
friable large yellow
vegetations may become systemic emboli
infarcts + abscesses.
 In subacute IE vegetations are firmer, less
destructive, and ring abscess are
uncommon.
1- rheumatic heart disease: vegetations are
marked by a row of warty, small vegetations
along the lines of closure of the valve
leaflets.
2- IE (infective endocarditis): vegetations
are characterized by large, irregular masses
on the valve cusps that can extend onto the
cords .
3- NBTE (nonbacterial thrombotic endocarditis)
typically exhibits medium sized bland vegetations,
usually attached at the line of closure.
4-LSE (Libman-Sacks endocarditis) has small or
medium-sized vegetations on either or both sides of
the valve leaflets.