CVS Module-Lecture 10

Lecture: 10 Prof. Magdy ElShamy 1

Rheumatic Fever &

Rheumatic Heart Disease
Definition::
Definition
Rheumatic fever (RF) is an acute
acute,, immune mediated,, multisystem
immune--mediated
inflammatory disease that occurs few weeks after upper
respiratory infection with group A β-
β-hemolytic
streptococci..
streptococci

Acute rheumatic heart disease (RHD)

is the cardiac manifestation of RF and is associated with
inflammation of the valves, myocardium,
myocardium, or pericardium.

Lecture: 10 Prof. Magdy ElShamy 2

1 Prof. Magdy ElShamy-Rheumatic Fever

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Pathogenesis Of
Acute Rheumatic
Heart Disease.

Acute rheumatic
fever causes
changes in the
endocardium,
myocardium, and
pericardium.

Chronic
rheumatic heart
disease is almost
always caused by
deformity of the heart
valves, particularly
the mitral and aortic
valves.

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Incidence:
- affects children & young adults (ages between 5-15 years).
- relatively high in low socioeconomic people
- It is a complication of less than 2% of streptococcal pharyngitis

Pathogenesis:
- In genetically susceptible individuals, upper respiratory tract
infection with group A β-hemolytic streptococci → production
of antibodies to streptococcal antigen (M protein) →
cross-react with glycoprotein antigens in the heart, joints, other
tissues ( = Type II hypersensitivity reaction)

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Clinical Course Of Rheumatic Fever:

1- Acute Phase: It involves

a- Heart (Cardiac):
→ Acute rheumatic heart disease → Pancarditis

b- Extracardiac Sites:
(joints, subcutaneous tissue, brain, pleura, blood vessels).

2- Chronic Phase:
- It involves the heart only → chronic rheumatic heart disease

- manifested after ~10 years by fibrosis affecting

the cardiac valves (mostly, mitral stenosis)

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Pathological Lesions of Rheumatic Fever

I- Rheumatic Heart Disease (RHD)
- All heart layers are affected (Pancarditis)

- Characteristic lesions called Aschoff bodies (pathognomonic for RF)

Gross: multiple tiny nodules, 1-2 mm (discovered by Ludwig Aschoff (1904)

Mic:
center of fibrinoid degeneration (or necrosis)
surrounded by
- lymphocytes, few plasma cells, macrophages and
- large activated macrophages called Anitschkow cells (also
called caterpillar cells ? abundant cytoplasm & central nuclei with
chromatin arrayed in a slender, wavy ribbon),
these cells can also fuse to form multinucleated giant cells termed
Aschoff giant cells.
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Acute rheumatic heart disease.

An Aschoff body is located interstitially in the myocardium.
Note collagen degeneration, lymphocytes, and a multinucleated
Aschoff giant cell.
(Inset) Nuclei of Anitschkow cells, showing owl-eye appearance in cross-
section and caterpillar shape longitudinally.
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Lecture: 10 Prof. Magdy ElShamy

Lecture: 10 Prof. Magdy ElShamy 8

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 CVS Module-Lecture 10

A) Rheumatic Myocarditis:
- It mainly affects the myocardium of left side of heart.
(→ scattered Aschoff bodies within the interstitial connective tissue).

- is mostly the common cause of death in acute disease

(Less than 1% of patients may die during the acute phase due to
moycarditis?? → CHF)

B) Rheumatic Pericarditis:
- shows a fibrinous or serofibrinous inflammation
(generally resolves without sequelae)

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C) Rheumatic Endocarditis:

1- Mural Endocarditis: Left sided

- Acute phase:
Several Aschoff bodies in posterior wall of left atrium.

- Chronic phase:
Healing of Aschoff bodies by fibrosis → white thick patch
at the posterior wall of left atrium called "Mac Callum's patch"

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2- Valvular Endocarditis:
A) Acute Rheumatic Valvulitis:
- Left sided valves (mitral alone → 70% & with aortic → ~25%)
- tricuspid valve is usually less frequently & less severely involved,
- pulmonary valve almost always escapes injury.
- Valve cusps are swollen with multiple, small (1-3 mm) firm,
adherent Vegetations (fibrin + platelets) at the lines of
closure of the cusps. (Embolism is uncommon).

B) Chronic Rheumatic Valvulitis:

- Fibrosis of the inflamed cusps → Valve incompetence or
Valve stenosis (fish mouth or buttonhole stenoses)

- The chordae tendineae become fibrosed, short, thickened

& fused
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Aschoff body
Small vegetations
There is central fibrinoid necrosis
(verrucae or warty projections)
surrounded by mononuclear
seen along the line of closure of
inflammatory cells, with some
the mitral valve + fibrous
activated macrophages (Anitschkow
thickening and fusion of the
cells) with prominent nucleoli
tendinous cords
(arrowheads).

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Rheumatic vegetations of Aortic valve cusps

sterile vegetations appear along the line of closure of the aortic valve.

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Rheumatic vegetations of Mitral valve cusps

sterile vegetations appear along the line of closure of the mitral valve.

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Chronic rheumatic valvulitis.

An example of severe rheumatic aortic stenosis.
The cusps are rigidly fibrotic and calcified, and extensive fusion of the
commissures has narrowed the orifice into a fixed slitlike configuration
that does not change during the cardiac cycle.

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II- Extracardiac Lesions Of Rheumatic Fever

1- Joints: → Rheumatic Migratory Polyarthritis
affects large joints → Fleeting arthritis (migratory polyarthritis)
then subsides spontaneously, leaving no residual disability.

2- Brain: → Rheumatic chorea (Sydenham chorea)

characterized by involuntary purposeless, rapid movements
affecting all muscles (Saint Vitus Dance)
3- Skin:
→ Rheumatic subcutaneous nodules: small non-tender on
extensor surfaces of the forearms
→ Erythema marginatum circular rings of erythema on trunk

4- Pleura: → Rheumatic Pleurisy (serofibrinous inflammation)

may occur.
5- Arteries: → Rheumatic arteritis
Lecture: 10
may affect coronary, renal, mesenteric and cerebral arteries
Prof. Magdy ElShamy 16

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Lecture: 10 Prof. Magdy ElShamy 17

Erythema marginatum Rheumatic Migratory Polyarthritis

circular rings of erythema on trunk (painful & swollen)
one large joint after another becomes
painful and swollen for a period of
days, followed by spontaneous
resolution with no residual disability

Rheumatic subcutaneous
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nodules: on extensor surfaces 18
of forearm Prof. Magdy ElShamy

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Clinical Features:

- Acute RF appears mostly in children aged 5 - 15 years,

- Symptoms occur 2-3 weeks after streptococcal pharyngitis.

- Fever, polyarthritis, functional heart murmurs (MR), ± pericardial friction rubs

a pericardial friction rub is caused by the
rubbing together of two surfaces of the
pericardial sack = rubbing sounds: best
heard between the apex and sternum
ECG: increased PR interval,
Laboratory:
- Elevated Antistreptolysin O (ASO) Titers,
- Increased anti–DNase B titers (less reliable than ASO titers)

- Elevated acute phase reactants: ↑CRP, ↑ESR

- ± Neutrophilic Leukocytosis
- Jones criteria Prof. Magdy ElShamy 19
Lecture: 10

Jones Criteria of Rheumatic Fever

Major Minor
1- Migratory polyarthritis of large joints 1- Fever
2- Pancarditis 2- Arthralgias (pain without joint swelling)

3- Sydenham chorea 3- ↑ acute phase reactants as ↑CRP

4- Subcutaneous nodules
5- Skin rash (erythema marginatum)

Diagnosis requires:
- Laboratory evidence of a recent streptococcal infection (↑ ASO Titre)
in conjunction with Two major OR One major + Two minor Jones
criteria

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Complications Of
Chronic Rheumatic Heart Disease:

1- Vavular disease: in the form of stenosis or incompetence.

2- Arrhythmias: particularly atrial fibrillation in case of MS

→ ± mural thrombi in dilated left atrium

3- Increased risk of subacute bacterial endocarditis on

deformed valves.

4- Congestive heart failure: from valvular disease, arrythmias

(→ congestion in the lungs & in time → right ventricular hypertrophy & failure)

(N.B.: Surgical repair or replacement of diseased valves has greatly

improved the outlook for patients with RHD).

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Robbins Basic Pathology, 9th Edition:

Chapter 10: The Heart:
Valvular Heart Disease

Robbins & Cotran Pathologic Basis of Disease, 8th Edition:

Chapter 12: The Heart:
Valvular Heart Disease
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