Rheumatic fever

Dr. Nashwah Al- Hariry

Lecturer of Pathology
Suez University
➢ Define Rheumatic Vulvitis
➢ Discuss the pathophysiology and pathogenesis of
rheumatic heart disease
➢ Discuss the pathological features of rheumatic heart
disease.
➢ Discribe the complications of rheumatic heart disease
Rheumatic Fever
Definition:
 An autoimmune collagen disease affecting the heart
and extracardiac sites
 Inflammation affects all parts of the heart
( preicardium, endocardium and myocardium).
 Valvular Inflammation → Scarring → Mitral Stenosis
Rheumatic Fever
Pathogenesis
 Antigenic similarity: Antibodies against group A
streptococcal molecules that cross-react with host
myocardial antigens.
 Altered antigenicity: Binding of the streptococcal
antigen to the human tissue renders it antigenic leading
to production of antibodies against the human tissue
(autoantibodies).
 Rheumatic Fever
Clinical Features
 Characteristic 2- to 3-week delay in symptom onset after
infection (time needed to generate an immune response)
 Streptococci are absent from the lesions.
 Genetic susceptibility is likely (only 3% of infected
patients develop rheumatic fever).
 Most often in children → carditis.
 20% of first attacks occur in adults (predominantly
arthritis).
 < 1% of patients die of acute RF.
Rheumatic Fever
Clinical Features
Phases of rheumatic fever:
I. Acute:
Heart, And extracardiac sites (joints, brain skin and others).
II. Chronic:
Heart only ⇒► Fibrosis ( Affecting mainly the cardiac valves
leading to stenosis)
Rheumatic Fever
Pathologic features
A- Rheumatic Heart Disease
1) Rheumatic Myocarditis:
a)Acute phase: Aschoff nodules
G/P: Multiple tiny greyish white nodules (1-2mm)
M/P:
▪Fibrinoid necrosis + Lymphocytes + Aschoff cells (mononuclear
or multinucleated cells of histiocytic origin).
▪Surrounding tissue► Oedema + inflammation
b)Chronic phase: Fibrosis
MORPHOLOGY
Aschoff body in acute rheumatic carditis; there is central
necrosis associated with a circumscribed collection of
mononuclear inflammatory cells, including some activated
macrophages (Anitschkow cells).
 Rheumatic heart disease
Pathologic features
A- Rheumatic Heart Disease
2) Rheumatic Pericarditis:
a)Acute phase: Serofibrinous inflammation + fibrin deposition
(between viseral and parietal layers) ►
(Bread and Butter appearance).
b)Chronic phase: Fibrosis
➢Whitish patches ► Milk spots
➢Adhesions:
Parietal and visceral pericardium
Pericardium and mediastinum
 Rheumatic Fever
Pathologic features
A- Rheumatic Heart Disease
3) Rheumatic Endocarditis:

Mural: Valvular
Acute: Acute:
➢Aschoff bodies (posterior ➢Valve cusps:
wall of left atrium). •Swollen, infalmmation ,edema
Chronic: fibrosis •Vegetations (Thrombi):
-Lines of contact
➢MaCcallam’s patches
-Multiple, small, grayish, firmly
adherent to the cusps
Chronic: fibrosis
Valve stenosis/incompetence
 MORPHOLOGY
Acute rheumatic mitral valvulitis on top of chronic rheumatic heart disease. Small
vegetations (verrucae) are visible along the line of closure of the mitral valve leaflet
(arrows). Previous episodes of rheumatic valvulitis have caused fibrous thickening
and fusion of the chordae tendineae.
MORPHOLOGY
Mitral stenosis with diffuse fibrous thickening and distortion
of the valve leaflets & commissural fusion “fishmouth” or
“buttonhole” stenosis
 MORPHOLOGY
Surgically removed specimen of rheumatic aortic stenosis, demonstrating
thickening and distortion of the cusps with commissural fusion.
Rheumatic Fever
Pathologic features
B- Extracardiac Disease
1. Migratory polyarthritis of large joints: affecting in
fleeting way.
►Joint is red, hot and swollen
2. Subcutaneous nodules
3. Erythematous annular rash (erythema marginatum)
4. Sydenham chorea, a neurologic disorder
characterized by involuntary purposeless, rapid
movements (also called St. Vitus dance).
5. Rheumatic arteritis (Hypersensitivity angiitis):
coronary, renal mesenteric
 Complications
 Valvular stenosis and regurgitation; stenosis predominate.
➢ Mitral valve alone is involved in 70% of cases.
➢ Combined mitral and aortic disease in 25%.
➢ Tricuspid valve is less frequently (and less severely) involved.
➢ Pulmonic valve almost always escapes injury.
➢ Mitral stenosis → left atrium dilates → atrial fibrillation→
thrombosis.
➢ Long-standing passive venous congestion → pulmonary vascular and
parenchymal injury →left-sided heart failure → right ventricular
hypertrophy and failure.

 Infective endocarditis
 Diagnosis Of Acute Rheumatic Fever
 Serologic evidence of previous streptococcal infection in
conjunction with two or more of the Jones criteria:
1. Carditis
2. Migratory polyarthritis of large joints
3. Subcutaneous nodules
4. Erythematous annular rash (Erythema Marginatum)
5. Sydenham chorea, a neurologic disorder characterized
by involuntary purposeless, rapid movements (also
called St. Vitus dance).
6. Minor criteria (fever, arthralgias, or elevated acute
phase reactants).
 Infective endocarditis
Definition
Microbial infection of the heart valves or the mural endocardium
→ vegetations of thrombotic debris and organisms →
destruction of the underlying cardiac tissues.
 The aorta, aneurysmal sacs, other blood vessels, and
prosthetic devices may become infected.
Etiology
 Vast majority of cases are caused by extracellular bacteria.
 Fungi, rickettsiae (agents of Q fever), and chlamydial species.
Types
 Acute & subacute forms.
 Clear delineation between acute and subacute endocarditis is
not always possible.
 Infective endocarditis
Acute Endocarditis Subacute Endocarditis
➢ Caused by organisms of low virulence
➢ Destructive infections ➢ affecting a previously abnormal heart,
especially scarred or deformed valves.
➢ Caused by highly
➢ Most patients recover after
virulent organism appropriate antibiotic therapy.
➢ Attacking a previously ➢ S. aureus (common to skin) can attack
normal valve. healthy as well as deformed valves
(10% to 20% of cases); major
➢ Streptococcus viridans offender in infections occurring in
cause 50% to 60% of intravenous drug abusers.
cases occurring on  Additional bacterial agents include
diseased valves enterococci e.g Haemophilus,
commensal in the oral cavity.
 More rarely, gram-negative bacilli and
fungi.
 Infective endocarditis
Acute Endocarditis
 Mitral and Aortic valves(the
most common).
 Tricuspid valve (I.V drug
abuser).
 +/- Mural endocardium.
➢ Pathology:
▪ Acute suppurative inflammation
+ valve perforation.
▪ Vegetations:
Gross: Multiple yellowish,friable,
detachable.
M/P: Platelets, fibrin, bacteria,
neutrophils and pus cells.
Subacute Endocarditis
 Mitral and Aortic valves(the
most common).
 Mural MacCallam’s patches.
➢ Pathology:
▪ The original pathology
▪ Vegetations:
Gross: Multiple grey, friable,
detachable.
M/P: Platelets, fibrin, bacteria
some inflammatory cells
mainly histiocytes.
 Infective endocarditis
Acute Endocarditis Subacute Endocarditis
 Complications

 Embolic lesions:  Embolic lesions:

Detachement of septic vegetations ►
systemic pyaemia
Microemboli are formed ► Petechia,
 Nail bed (splinter) hemorrhages
 Retinal hemorrhages (roth spots).
 Painless palm or sole erythematous
lesions (janeway lesions).
 Painful fingertip nodules (osler nodes)
 Infective endocarditis
Acute Endocarditis
➢ Toxic lesions:
▪Sever toxaemia and septicaemia.
▪Fever, chills, weakness
➢ Prognosis
▪Fatal due toxaemia
▪Cusp perforation and incompetence
Subacute Endocarditis
➢ Toxic lesions:
▪ Mild toxaemia, nonspecific fatigue, weight
loss, and a flulike syndrome, anaemia,
clubbing of fingers.
▪ Splenomegaly
▪ Glomerulonephritis (antigen-antibody
complexes), with hematuria, albuminuria, or
renal failure.
➢ Prognosis
 Infections with low-virulence organisms (e.g.,
Streptococcus viridans or Streptococcus bovis)
→ the cure rate is 98%
 For enterococci and Staphylococcus aureus
infections → cure rates 60% 90%
 Infections with aerobic gram negative bacilli or
fungi are associated with fatality rate of
approximately 50%.