Transcript Document

Rheumatic diseases
Assistant of professor
Nechiporenko G.V.
COLLAGEN
DISEASES
Rheumatic fever
 Systemic lupus erythematosus
 Rheumatoid arthritis
 Systemic sclerosis
 Polyarteritis nodosa
 Siogren’s syndrome

Rheumatic fever (RF)
 It
is a systemic, poststreptococcal, nonsuppurative inflammatory
disease, principally affecting
the heart, joints, central
nervous system, skin and
subcutaneous tissues.
Clinical-anatomical forms of
RHEUMATIC FEVER
1.Cardiovascular form (endocarditis,
myocarditis, pericarditis). Chronic stage of
RF involves usually all layers of the heart
(pancarditis) causing major consequence
referred to as rheumatic heart disease
(RHD). William Boyd many years ago
gave the dictum “rheumatic licks the joint,
but bites whole heart”.
2. Polyarthritic form (large joints).
3.Nodular form (nodules around vessels).
4.Cerebral form (chorea minor).
RHEUMATIC
ENDOCARDITIS
Rheumatic valvulitis and mural
endocarditis are chiefly responsible for the
major cardiac manifestations in chronic
rheumatic heart disease.
 Acute stage of rheumatic valvulitis shows
thickening and loss of translucency of the valve
cusps with following development of verrucae
(vegetations, warty) along the line of closure.
Histological changes are disorganization of
connective tissue with mucoid sweeling, fibrinoid
degeneration, cellular reactions with formation
of Aschoff bodies, thrombi.

The small verrucous vegetations are seen along the closure
line of mitral valve with acute rheumatic fever. These
warty vegetations average only a few millimeters

In time, chronic
rheumatic valvulitis may
develop by organization
of the acute endocardial
inflammation along with
fibrosis, as shown here
affecting the mitral valve.
Note the shortened and
thickened chordae
tendineae.
RHEUMATIC MYOCARDITIS
1.Nonspecific exudative interstitial
myocarditis.
2.Granulomatous myocarditis
 The giagnostic perivascular nodules (Aschoff
bodies) are most frequent in the interventricular
septum, left ventricle and left atrium.
 Evolution of fully-developed Aschoff bodies
involves 3 stages:
 Early (exudative or degenerative) stage- about
4th week of illness with progressive
disorganization of connective tissue
(hypersensitivity of immediate type with fibrinoid
degeneration).

Intermediate (proliferative or
granulomatous) stage-in 4th- to 13th
week of ilness. Formation of lymphocytes,
plasma cells, a few neutrophils and the
characteristic cardiac histiocytes
(Anitschkow cells) at the margin of lesion.
Some of these modified cardiac histiocytes
become multinucleate cells containing 1to
4 nuclei and are called Aschoff cells
(hypersensitivity of delayed type with
cellular infiltration).
 Late
(healing or fibrous) stage- about
12 to 16 weeks after the ilness. The
Anitschkow cells in the nodule
become spindle-shaped with
diminished cytoplasm and the solid
nuclear stain. These cells tend to be
arranged in a palisaded manner. It is
replaced by a small fibrocollagenous
scar with little cellularity
Acute rheumatic myocarditis with "Aschoff nodules“.
These are centered in interstitium around vessels. The
myocarditis may cause congestive heart failure.

Fibrinous pericarditis.

A window of adherent
pericardium has been
opened to reveal the
surface of the heart.
There are thin strands
of fibrinous exudate
that extend from the
epicardial surface to
the pericardial sac.
The pericardial surface shows strands of pink
fibrin extending outward. Fibrin can be organized
and cleared, though sometimes adhesions may
remain.
Valvular diseases and
deformities
Stenosis is the term used the failure of a valve to open
completely during diastole resulting in obstruction to the
forward flow of the blood.
 Mitral stenosis occurs in about 40% of all patients with
RHD. About 70% of the patients are women.
 The valve cusps are diffusely thickened by fibrous tissue
and/or calcific deposits. ”Purse-string puckering”,
”button-hole“ or “fish-mouth” mitral orifice.
 Effects: dilatation and hypertrophy of the left atrium;
pulmonary hypertension with following chronic venous
congestion of the lungs, hypertrophy and dilatation of
the right ventricle; right heart failure in time.

The mitral valve demonstrates the typical
"fish mouth" shape with chronic rheumatic
scarring.
Heart, rheumatic mitral stenosis, atrial changes - Gross, atrial
endocardial surface
The mitral valve has been reduced to a narrow orifice. The left atrium
is markedly dilated. Thrombi have formed on the atrial wall.
Aortic stenosis comprises about 25% of all
patients with chronic valvular heart disease.
About 80% patients are males. There are noncalcific and calcific type, the latter being more
common. The aortic cusps show characteristic
fibrous thickening and calcific nodularity of the
closing edges.
 Effects: obstruction to the outflow resulting in
concentric hypertrophy of the left ventricle,
there is dilatation as well as hypertrophy of the
left ventricle (eccentric hypertrophy).
 Three cardinal symptoms of aortic stenosis are:
exertional dyspnea, angina pectoris, syncope.

Rheumatic aortic stenosis
Insufficiency or incompetence or regurgitation
is the failure of valve to close completely during
systole resulting in back flow or regurgitation of
blood.
 Mitral insufficiency occurs in about 50% patients
with RHD more often in men (75%).
 Effects: dilatation and hypertrophy of the left
ventricle; dilatation of the left atrium;
pulmonary hypertension with following chronic
venous congestion of the lungs, hypertrophy and
dilatation of the right ventricle; right heart
failure in time.

Aortic insufficiency occurs predominantly in
males (75%) with RHD.
 The aortic valve cusps are thickened, deformed
and shortened and fail to close. There is
generally distension and distortion of the ring.
 Effects: hypertrophy and dilatation of the left
ventricle producing massive cardiac enlargement
so that the heart may weigh as much as 1000
gm.
 The characteristic physical findings of aortic
insufficiency are awareness of the beatings of
the heart, poundings in the head with each
heart beat, low diastolic and high pulse
pressure, rapidly rising and collapsing water
hammer pulse, booming ”pistol shot” sound over
the femoral artery.

This is an excised porcine bioprosthesis; the
undersurface is at the left and the outflow side is at
the right. Note there are three cusps sewn into a
synthetic ring.
Systemic lupus erythematosus
Systemic lupus erythematosus (SLE) is a chronic
disease with many manifestations. SLE is an
autoimmune disease in which the body's own
immune system is directed against the body's
own tissues. The etiology of SLE is not known. It
can occur at all ages, but is more common in
young women.
 The production of autoantibodies leads to
immune complex formation. The immune
complex deposition in many tissues leads to the
manifestations of the disease.
 Immune complexes can be deposited in
glomeruli, skin, lungs, synovium, mesothelium,
and other places. Many SLE patients develop
renal complications.

 The
young
woman has a
malar rash in
DLE
("butterfly"
rash because
of the shape
across the
cheeks).
Face, malar (butterfly) rash of
systemic lupus erythematosus -SLE
The skin in SLE may demonstrate a
vasculitis and dermal chronic
inflammatory infiltrates
Kidney, chronic lupus nephritis
Note the diffuse granularity of the cortex
Here is a glomerulus with thickened pink
capillary loops, the so-called "wire loops“.
Crescentic lupus nephritis
Seen here within the glomeruli are crescents
composed of proliferating epithelial cells.
Immunofluorescence with antibody to IgG.
A granular pattern of immunofluorescence is seen,
indicative of deposition of immune complexes in the
basement membranes of the glomerular capillary loops.
The thickened basement membrane (arrow) that results
from immune complex deposition in the glomerular
capillary loop is prominent in this electron micrograph. The
dark immune deposits are located mainly in a
subendothelial position.
The periarteriolar fibrosis ("onion
skinning") is seen in the spleen.
RHEUMATOID ARTHRITIS

Rheumatoid arthritis (RA) is a chronic
multisystemic inflammatory disease of
unknown origin involving peripheral joints
with symmetrical distribution. Its systemic
manifestations include hematologic,
pulmonary, neurological and cardiovascular abnormalities.
 RA is an autoimmune disease.
The characteristic feature is diffuse proliferative
synovitis with formation of pannus.
 Lesions affect small joints of hands and feet
mainly.
 The synovial membrane becomes thick,
edematous, hyperplastic and covered by villous
projections.
 Chronic inflammatory cellular infiltrate in the
synovium with predominance of lymphocytes,
plasma cells and some macrophages, at places
forming lymphoid follicles.
 Organized fibrin deposit over the synovial surface.
 Pannus creeps over the articular cartilage.
 Erosion of the articular cartilage and subchondral
bone.
 Fibrous and osseous ankylosis.

Hand, acute rheumatoid arthritis
Synovium, rheumatoid arthritis
Primary lesion of rheumatoid arthritis is synovitis.
You can see here in the synovium collections of
dark blue lymphocytes.
Synovium, rheumatoid arthritis, pannus
The pannus isolates the articular cartilage from the synovial
fluid, resulting in degeneration of articular cartilage
Rheumatoid nodules consist of a central zone of fibrinoid
necrosis surrounded by a prominent rim of epithelioid
histiocytes and numerous mononuclear cells
Polyarteritis nodosa (PAN)
It is necrotising-granulomatous vasculitis
involving small and medium-sized muscular
arteries of multiple organs and tissues (kidneys,
heart, liver, GIT, muscles, pancreas, testes,
nervous system, skin).
 The condition is believed to result from
deposition of immune complexes and tumorrelated antigens. The disease occurs more
commonly in adult males.

Acute stage. There is fibrinoid necrosis in
the centre of nodules located in the media
of vessels. An acute inflammatory
response develops around it. The
inflammatory infiltrate is present in the
entire circumference of the affected vessel
(periarteritis) and consists chiefly of
eosinophils, mononuclear neutrophils.
 Healing stage. This is characterised by
marked fibroblastic proliferation producing
firm nodularity.
 Heales stage. The affected arterial wall is
markedly thickened due to dense fibrosis.

Kidney, polyarteritis nodosa
Two small renal infarcts of tissue supplied by small to medium-sized
arteries are evidenced by pale necrotic areas at the top of this
specimen. This is an immune-complex disease frequently associated
with hepatitis B virus (HBV) and cytomegalovirus (CMV) antigens.
Here is a vasculitis of a renal arterial branch.
Lymphocytes are scattered in and around
the vessel.
Systemic sclerosis or
Scleroderma
1.Localized form - morphea
It consists of lesions limited to the skin and subcutaneous
tissue
2.Generalized form- progressive systemic sclerosis
It consists of extensive involvement of the skin,
subcutaneous tissue and has visceral lesions
a) CREST-syndrome
C= calcinosis
R= Raunad’s phenomenon (functional vaso-spastic disorder
affecting small vessels of fingers and hands)
E= esophageal dismotility
S=sclerodactylia
T=telangiectasia
,
Hand, scleroderma sclerodactyly
Dense cutaneous fibrosis (note smooth shiny skin) has caused
immobility of the fingers, causing the claw-like appearance seen here.
In advanced cases, impairment of blood supply may lead to ulceration
or autoamputation.
Skin, scleroderma
The dermis is thickened due to growth of connective tissue
Lung, lower lobe, systemic sclerosis
Cardiomyopathies
Type of CMP
Findings
Dilated
(Congestive)
All four chambers are dilated, and there is also
hypertrophy. The most common cause is chronic
alcoholism, though some may be the end-stage of
remote viral myocarditis.
Hypertrophic
The most common form, idiopathic hypertrophic
subaortic stenosis (IHSS) results from asymmetric
interventricular septal hypertrophy, resulting in left
ventricular outflow obstruction.
Restrictive
The myocardium is infiltrated with a material that
results in impaired ventricular filling. The most
common causes are amyloidosis and
hemochromatosis.
The heart in the middle is relatively normal. The one on the
left shows concentric hypertrophy. The one on the right
shows ventricular dilation in dilated cardiomyopathy
This very large heart has a globoid shape because all of the
chambers are dilated. It felt very flabby, and the
myocardium was poorly contractile.
Here is a large, dilated left ventricle typical
of a dilated or congestive cardiomyopathy.

Hypertrophic
cardiomyopathy.

There is marked
left ventricular
hypertrophy, with
asymmetric bulging
of a very large
interventricular
septum into the
left ventricular
chamber.
Heart, hypertrophic
cardiomyopathy
The heart in cardiomyopathy demonstrates
hypertrophy of myocardial fibers (which also have
prominent dark nuclei) along with interstitial
fibrosis.
Amorphous deposits of pale pink material
(amyloid) are between myocardial fibers.
Amyloidosis is a cause of "infiltrative" or
"restrictive" cardiomyopathy.