Rheumatic fever

RHEUMATIC FEVER
Is immunologically mediated, multisystem
inflammatory disease that occurs a few weeks
following an episode of group A β hemolytic
streptococcal pharyngitis.
-both sexes are equally affected between 5-
15 years
The incidence and mortality rate of RF have declined
remarkably in many parts of the world over the past
30 years, owing to improved socioeconomic conditions,
and an unexplained decrease in the virulence of group
A streptococci. Nevertheless, in developing countries,
and in many crowded, economically depressed urban
areas in the Western world, RF remains an important
.public health problem
 AETIOLOGY
:Predisposing factors
Hereditary- 1
predisposition
Cold climate, low socio-- 2
economic standards
overcrowding and
malnutrition
recurrent streptococcal
tonsillitis

Exciting cause: abnormal

. immune reaction
 feat
ures
of
acut
e
rheu
mati
c
 PATHOGENESIS
Group A strep pharyngeal infection precedes clinical-
manifestations of ARF by 2 - 6 weeks
Antibodies made against group A strep cross-react-
with human tissue because of molecular mimicry. heart
valve and brain share common antigenic sequences with
.GAS bacteria starting the formation of allergic granuloma
Only a minority of infected patients develop RF, suggesting-
the role of genetic susceptibility
Pathogenesis
Most important antigenic proteins in external
layer of cell wall M, T
and R proteins
 TISSUE REACTION

Characteristic lesion known as “Aschoff’s body”.

• Central area of necrosis.

• Collection of chronic inflammatory cells with
occasional Aschoff giant cells.

Fate: Fibrosis.
Normal myocarduim Aschoff
bodies
Aschoff body
Clinical diagnosis:  
The clinical diagnosis of acute rheumatic fever is made
when two major or one major and two minor criteria -
Jones Criteria, are met.
The major clinical manifestations include - Carditis ,
Erythema marginatum ,Migratory large joint arthritis ,
Sydenham chorea (a neurologic disorder with involuntary
purposeless, rapid movements),Subcutaneous nodules.

The minor manifestations include - arthralgia ; fever ;

certain laboratory tests indicative of an inflammatory
process (Eg: , positive test for C-reactive protein,
leukocytosis) and electrocardiographic changes
 Guidelines for the diagnosis of initial attack of rheumatic fevrer
Jones Criteria

Major manifestations Minor manifestations

Carditis Fever
Arthritis Arthralgia
Sydenham’s chorea laboratory tests indicative of an
inflammatory process

 Erythema marginatum electrocardiographic changes

Subcutaneous nodules
Subcutaneous nodules are rarely seen and when
present, they are usually associated with severe
carditis. They are painless, firm, movable, measuring
around 0.5 to 2 cm. They are usually located over
extensor surfaces of the joints, particularly knees,
wrists and elbows
Erythema marginatum on the trunk, showing
erythematous lesions with pale centers and rounded
or serpiginous margins
Acute rheumatic fever

The predominant clinical manifestations are those of carditis and

arthritis

acute carditis: (pancarditis) include

Pericarditis ,myocarditis,endocarditis:
During the acute stage, The myocarditis may cause cardiac dilation
that may evolve to functional mitral valve insufficiency or even heart
failure. but with chronic disease, endocarditis with permanent valve
lesions are most serious and dangerous. The patholigical change of the
valve lead to its stenosis and or regurgitation.

Arthritis It typically begins with migratory arthritis accompanied by

fever in which one large joint after another becomes painful and swollen
for a period of days and then subsides spontaneously, leaving no residual
disability.
 PATHOLOGY
Rheumatic carditis: Pancarditis 
Rheumatic Endocarditis- 1
Rheumatic Myocarditis- 2
.Rheumatic pericarditis- 3


 RHEUMATIC ENDOCARDITIS

Valvular endocarditis: affects the valvular

endocardium especially arotic and mitral valve

Mural endocarditis: affects the mural endocardium

of the posterior wall of the left atruim
Acute Rheumatic Valvulitis

Multiple episodes of acute Rheumatic fever

Inflammation of the cardiac cusps with the formation of Aschoff’s.
nodules with edema results in swelling of the leaflets of the cusps,
friction of their free borders….injury of the endothelium……thrombosis
((vegetations

Acute phase subsides then fibrosis alters leaflet and cusp -

structure results in leaflet or cuspal thickening along valvular
margins of closure, commissural fusion and shortening, and thickening
..and fusion of the tendinous cords
Valves affected. Most often mitral valve alone .Then most often
mitral and aortic together .Lastly aortic alone
Acute
Rheumatic
Valvulitis
Valve cusps are swollen
and red
- Vegetations are
found near the free
margin of cusps
( small, beaded, pale
and adherent)
Mitral valve vegetations
RF
Vegetation, fibrin,
platelets
 RHEUMATIC MYOCARDITIS
Aschoff's bodies are seen in interstitial tissue of
the myocardium and associated with interstitial
edema and mild inflammation, sometimes
with muscle fiber necrosis.

- The condition is usually mild, but may

.produce left ventricular failure
 Rheumatic
myocarditis
Aschoff giant
cells
 RHEUMATIC PERICARDITIS
Rheumatic fever is the commonest cause of sero-
fibrinous pericarditis mainly at the heart base,
the pericardial sac is filled with serous fluid
and fibrin is deposited on both visceral and
parietal pericardium. Pericarditis heals by
organization (fibrosis) which can result in…
Adhesions between the visceral and parietal
pericardium. Separation of which produces
.bread and butter appearance
.Aschoffs' bodies may be seen
, Serofibrinous pricarditis
, Serofibrinous pericarditis
,Serofibrinous pericarditis
Serofibrinous pricarditis, fibrin
threads
Pericarditis, fibrin at the
 COMPLICATIONS OF RF

-Valvular lesions.

- Infective endocarditis

Heart failure
Valvular damage
Healing of acute valvular lesion by fibrosis with fusion
of the cusps result in inability of the valve to open
properly…….
Stenosis

Healing of acute valvular lesion by fibrosis with

contraction of the cusps result in inability of the
valve to close properly…….
incompetence
Stenotic mitral valve
seen from left atrium.
Both commissures are
fused; the cusps are
. severely thickened
This view of the atrioventricular valves shows marked stenosis of
the mitral valve, and less severe involvement of the tricuspid
..valve
Thickened mitral valve, fibrotic chordae tendinae
Effect of Mitral
Stenosis
On Heart

Left atrium hypertrophies-

and dilates and its pressure
increase. It leads to
pulmonary venous
hypertension and oedema
increased pulmonary-
arterial pressure and
pulmonary vascular
resistance . Right ventricle
dilates from pressure
overload .. Right heart
failure develop
Effect of Mitral Stenosis
On Heart
-Atrial fibrillation and thrombosis may occur.
-Left ventricles “protected” by stenotic mitral
valve
-LV usually normal in size and contour

-
 Effect of Mitral Stenosis On Lung
Chronic edema of alveolar walls ,
pulmonary hemosiderin deposited in
lungs, fibrosis
Valvular damage
Mitral incompetance
-may occur alone or associated
with mitral stenosis.
- Blood accumulates in the left
side of the heart …… hypertrophy
then dilatation of both the left
ventricle and atrium and then left
side heart failure.
Aortic Regurgitation

the leaflets of the-

valve do not fit
together properly lead
to left ventricular
dilatation and failure
Valvular damage
•Aortic Stenosis
-Fusion, thickening of the cusps leading to concentric left ventricular
hypertrophy. Inadequate coronary perfusion lead to syncope and angina.
Eventually. Left ventricular failure develop.
�
 INFECTIVE ENDOCARDITIS
Infection of the endocardium (esp. heart valves) by a-
microbiological agent, with the formation of
vegetations of fibrin, inflammatory cells, & bacteria
.or other organisms
Vegetations located most commonly on heart valves,-
. esp. aortic & mitral

Vegetation may produce emboli that produce infarcts-

in brain, kidney, myocardium, & other organs
Vegetation of infective endocarditis
Heart failure
Heart failure (HF) is a condition in which
the heart is unable to provide the body
with enough blood and nutrients to meet
its metabolic needs. Heart failure is
usually caused by failure of the heart to
function efficiently as a pump
-It may be acute or chronic.
-Chronic may be right or left sided or
.total heart failure
Blood backs up causing congestion of neck veins and swelling of- 
extremities and internal organs