Transcript 12 Systemic disease of connective tissues

Rheumatic
illnesses
As.-prof. V.Voloshyn
In Accordance with prof. Ya.Ya. Bodnar,
T.V.Datsko
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Rheumatic illnesses
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- are the group of diseases in basis of
development of which is the progressive
system disorganization of connecting
tissue and vessels, which is predefined
by immune violations .
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Classification of rheumatic
illnesses
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rheumatism;
rheumatoid arthritis (RA);
lupus erythematosus (LE);
systemic scleroderma (SS);
nodular periarteritis (NPA) and other systemic
vasculites;
dermatomyositis;
illness (syndrome) of Sjogren;
illness of Bechterew (spondylitis deformans). 3
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All rheumatic illnesses are
combined by the systemic
progressive damage of connecting
tissue, which is characterized by
stages and includes 4 types of
changes:
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Stages of disorganization of
connecting tissue
Mucoid swelling
Fibrous changes
Cellular reactions
Sclerosis
(granulomatic inflammation)
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Rheumatism
(Sokolsky-Bouillaud disease)
is the infectiously-allergic disease which
is characterized by system disorganization
of connecting tissue with the dominant
defeat of the heart-vessels system.
An exciter is the β-haemolitic streptococcus
of group A. 1-3 % of peoples which had
streptococci quinsies will be ill by rheumatism. This
mean that is the inclination coupled with Xchromosome.
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Etiology of rheumatism
The features of influencing of etiologic factor (βhaemolitic streptococcus of A group) are based in its
possibility to produces the row of toxins and enzymes which can
damage tissues of organism :
 hyaluronidasa – increases of vascular-tissue permeability and
penetration of streptococcus;
 S streptolysin – damages of connecting tissue of vessels;
 O streptolysin –damages connecting tissues of heart;
 С-polysaccharide – has the cross antigenicity in attitude toward
the connecting tissue of heart and reticuloepithelium of thymus;
 M-protein – damages the cardiomyocytes up to their necrosis.
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Clinico-morphologic forms of
rheumatism
cardiovascular;
 polyarthritic;
 nodular;
 cerebral.
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Cardiovascular form of rheumatism
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Endocarditis: valvular, chordal, atparietal; acute
diffuse, acute warty, fibroplastic, relapsing warty
Myocarditis: granulomatic (nodular productive),
diffuse interstitial exudative, focal interstitial
exudative.
Pericarditis: serous, serofibrinous, fibrinous.
Vasculitis: capillaritis, arteriolaritis, arteritis.
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Endocarditis
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The heart tends to dilate
Acute and chronic valvulitis
Mural thrombi
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Endocarditis
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Myocarditis
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Myocarditis
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Morphology of Aschoff body
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Is the typical lesion
of rheumatic
myocarditis,
developing several
weeks after the onset
of symptoms. This
structure initially
consists of rounded
by lymphocytes,
plasma cells, macrophages, and giant cells.
Eventually, the Aschoff body is replaced by a
nodule of scar tissue.
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Morphology of Anitschkow cells
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Anitschkow cells are unusual
cells within the Aschoff body,
whose nuclei contain a central
band of chromatin. In cross
section these nuclei have an
“owl eye” appearance, and
when cut longitudinally trey
resemble aq “caterpillar”.
Anitschkow cell may become
multinucleated, in which case
they are termed Aschoff
myocytes.
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Pericarditis
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Tenacious irregular deposits of fibrin are found on both the
visceral and parietal surfaces of the pericardium. These
deposits resemble the shaggy surfaces of two slices of buttered
bread that have been pulled apart (“bread and butter
pericarditis”). The pericarditis may be recognized clinically by a
friction rub, but it has little functional effect and ordinarily does
not lead to constrictive pericarditis.
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Pericarditis
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Complications of chronic rheumatic
heart disease
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1. Bacterial endocarditis follow of bacteremia, such as
hose that occur during dental procedures. The scarred
valves of rheumatic heart disease provide an
attractive environment for bacteria that wound
ordinarily bypass a normal valve
2.Mural thrombi from in the atrial or ventricular
chambers in 40% of patients with rheumatic valvular
disease. They give rise to thromboemboli, which
produce infarcts in various organs. Rarely, a large
thrombus in the left atrial appendage develops a
stalk and acts as a ball valve that obstructs the
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mitral valve orifice
Complications of chronic rheumatic
heart disease (continuation)
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3.Congestive heart failure is
associated with rheumatic disease
of both the mitral and aortic
valves.
4. Cor pulmonale may develop as
a result of secondary pulmonary
hypertension.
5. Adhesive pericarditis commonly
follows the fibrinous pericarditis of
the acute attack, but almost
never results in constrictive
pericarditis.
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Polyarthritic form of
rheumatism
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Nodular form of
rheumatism
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Cerebral form of
rheumatism
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System lupus
erythematosus
Lupus erythematosus (LE) is chronic polysyndrome
disease mostly of young women and girls, which
develops on a background the genetic predefined
imperfection of immunoregulatory processes. It results
to the uncontrolled productions of antibodies to own
tissues and their components with development of
autoimmune and immunocomplex chronic inflammation.
 Morbidity by LE makes 48-50 cases per 100 thousands
of population, death rate – 5,8 per 1000 000 (at this 4,7
are women).
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Pathomorphology
Arteriolitis, capillaritis, vasculitis, interstitial
inflammation of all internal organs with passing
to the sclerosis, periarterial “bulbous” sclerosis
of spleen, hyperproductions of immunoglobulins,
losing of DNA, presence of lupusal cells,
erythema of skin (contour of butterfly),
endocarditis
of
Лібмана
and
Saxon,
гломерулонефрит,
поліартрит
without
deformation of joints
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Symptom of “Butterfly”
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Endocarditis of Libman-Sacks
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Periarterial “bulbous” sclerosis
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Glomerulonephritis
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Rheumatoid arthritis
Rheumatoid Arthritis (RA)
(polyarthritis) is the chronic system diseaseof
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connective tissue with the progressive defeat of
peripheral (synovial) joints mostly as the type
of erosive-destructive polyarthritis.
 RА is registered in the all countries of the
world and all climate-geographical areas with
frequency from 0.6 to 1.3 %. Everywhere
women are unwell more frequent (3-4:1).
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Etiology and pathogeny
The defeat of connective tissue (mostly joints) is a result of
immunopathological processes (autoaggressions).
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It is consider that the violation of immune answer
regulation as a result of disbalance of function of Т- and Вlymphocytes at RA is the reason of immunocomplex defeat.
Because the deficit of the system of T- lymphocells leads to
activating of В- lymphocells and uncontrolled synthesis of
antigenes (IgG) by the plasmatic cells. Antigenes IgG at RA
are changed possess [pe'zes] (володіти) autoreactivity. That
is why antibodies of ІgG, ІgМ, ІgA (rheumatoid factors) act
against abnormal antigenes. At interaction (co-operation) of
rheumatoid factors with IgG immune complexes formed and
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initiate the row of chain reaction.
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Pathomorphology
Arteriitis,
arteriolitis,
progressive
destructive polyarthritis, fibrotic-bone
ankilosis , osteoporosis, polyserositis
(multiplemeaning),
glomerulonephritis,
pyelonephritis, amyloidosis of kidneys,
cardiosclerosis
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Rhematoid synoviitis
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Complication of Rheumatoid
Arthritis
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Rheumatoid Arthritis
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Systemic scleroderma (scleriasis)
(Progressive systemic sclerosis)
Systemic scleroderma is the system
disease of connecting tissue and small vessels,
which is characterized by the widespread
fibrotic-sclerotic changes of skin, stromae of
internalss and symptomes of obliterated
endocarditic in the form of syndrome of Reino.
 Primary morbidity in the USA is known only –
12 accidents on the 1 million populations in a
year. The women are unwell in 3-7 times more
frequent. Age-dependent peak – 30-60 years.
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Etiology and pathogeny
Etiology of Systemic scleroderma is unknown.
The violation of microcirculation as a result of the
defeat of vascular wall and of plasma and cellular
properties of blood is the important factor of
pathogeny of Systemic scleroderma. This leads to
surplus synthesis of soluble forms of collogen,
damage of endothelium and its substitution by
smooth muscle collagen-producing , increasing of
possibility to the spasm and hyperplasia of internal
membrane of vessels .
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Pathomorphology
Arteriitis,
arteriolitis,
sclerosis,
hyalinosis, atrophy of skin (parchment
skin), sclerodermic heart (large nidusal
cardiosclerosis), sclerodermic
(cortical
necrosis), basal pneumofibrosis.
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Systemic scleroderma
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Systemic scleroderma
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Nodular periarteritis
Nodular periarteritis is system necrotic
vasculitis as the type segmental defeat of small
and middle caliber arteries with formation of
the aneurysm protrudes. The men of young
age are ill mostly, morbidity makes 2-3 on the
1 million populations in the year.
 Etiology. Nodular periarteritis develops after
the acute respirators infections including the
streptococcus illness,
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Pathomorphology
The defeat of arteries of muscular type of
small and middle calibers in the area of their
fork is the most characteristic morphological
sign of nodular periarteritis. The feature of
nodular periarteritis is the simultaneous defeat
of endothelia of vessels (deposit of immune
complexes),
internal
elastic
membrane
(polymorphic-cellular inflammation contains the
lymphoid epithelioid neutrophiles, fibroblasts)
and perivascular tissue (cellular infiltration and
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scarring).
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Nodular periarteritis
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I thank you for
attention !
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