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 515 years

RHEUMATIC FEVER

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

:Predisposing factors Hereditary- 1 predisposition Cold climate, low socio-- 2 economic standards overcrowding and malnutrition recurrent streptococcal tonsillitis

AETIOLOGY

Exciting cause: abnormal . immune reaction

feat ures of acut e rheu mati c

Group A strep pharyngeal infection precedes clinicalmanifestations of ARF by 2 - 6 weeks Antibodies made against group A strep cross-reactwith 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, suggestingthe role of genetic susceptibility

PATHOGENESIS

Pathogenesis
layer of cell wall M, T and R proteins

Most important antigenic proteins in external

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 bodies

Aschoff

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 Arthritis Sydenham’s chorea Fever Arthralgia 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 serofibrinous 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
incompetence

contraction of the cusps result in inability of the valve to close properly…….

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 hypertrophiesand dilates and its pressure increase. It leads to pulmonary venous hypertension and oedema increased pulmonaryarterial 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 thevalve 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 amicrobiological 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 infarctsin 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 ofextremities and internal organs

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