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Rheumatic Rheumatic illnessesillnesses

In Accordance with prof. Ya.Ya. Bodnar, T.V.Datsko

As.-prof. V.Voloshyn

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Rheumatic illnessesRheumatic illnesses

-- are the group of diseases in basis are the group of diseases in basis of development of which is the of development of which is the progressive system disorganization progressive system disorganization of connecting tissue and vessels, of connecting tissue and vessels, which is predefined by immune which is predefined by immune violationsviolations ..

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Classification of rheumatic Classification of rheumatic illnessesillnesses

rheumatism;rheumatism; rheumatoid arthritis (RA);rheumatoid arthritis (RA); lupus erythematosus (LE);lupus erythematosus (LE); systemic scleroderma (SS);systemic scleroderma (SS); nodular periarteritis (NPA) and other systemic nodular periarteritis (NPA) and other systemic

vasculites;vasculites; dermatomyositis;dermatomyositis; illness (syndrome) of Sjogren;illness (syndrome) of Sjogren; illness of Bechterew (spondylitis deformans).illness of Bechterew (spondylitis deformans).

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All rheumatic illnesses are All rheumatic illnesses are combined by the systemic combined by the systemic progressive damage of progressive damage of connecting tissueconnecting tissue, , which is which is characterized by characterized by stagesstages and and includes 4 types of changes:includes 4 types of changes:

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Mucoid swelling

Stages of disorganization of Stages of disorganization of connecting tissueconnecting tissue

Fibrous changes

Cellular reactions(granulomatic inflammation) Sclerosis

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Rheumatism Rheumatism (Sokolsky-Bouillaud disease)(Sokolsky-Bouillaud disease)

is the infectiously-allergic disease which is the infectiously-allergic disease which is characterized by system disorganization is characterized by system disorganization of connecting tissue with the dominant of connecting tissue with the dominant defeat of the heart-vessels system.defeat of the heart-vessels system.

An exciterAn exciter is the is the ββ--haemolitic haemolitic streptococcus of group Astreptococcus of group A. 1-3 % of peoples . 1-3 % of peoples which had streptococci quinsies will be ill by which had streptococci quinsies will be ill by rheumatism. This mean that is the inclination rheumatism. This mean that is the inclination coupled with X-chromosomecoupled with X-chromosome..

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Etiology of rheumatismEtiology of rheumatism

The features of influencing of etiologic factor (βThe features of influencing of etiologic factor (β--haemolitic streptococcus of A group) haemolitic streptococcus of A group) are based in its are based in its possibility to produces the row of toxins and enzymes which can possibility to produces the row of toxins and enzymes which can damage tissues of organismdamage tissues of organism ::

hyaluronidasa – increases of vascular-tissue permeability and hyaluronidasa – increases of vascular-tissue permeability and penetration of streptococcus;penetration of streptococcus;

S streptolysin – damages of connecting tissue of vessels;S streptolysin – damages of connecting tissue of vessels;O streptolysin –damages connecting tissues of heart;O streptolysin –damages connecting tissues of heart;С-С-polysaccharide – has the cross antigenicity in attitude toward polysaccharide – has the cross antigenicity in attitude toward

the connecting tissue of heart and reticuloepithelium of thymus;the connecting tissue of heart and reticuloepithelium of thymus;M-protein – damages the cardiomyocytes up to their necrosis.M-protein – damages the cardiomyocytes up to their necrosis.

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Clinico-morphologic forms Clinico-morphologic forms of rheumatismof rheumatism

cardiovascular;cardiovascular; polyarthritic;polyarthritic; nodular;nodular; cerebral.cerebral.

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Cardiovascular form of Cardiovascular form of rheumatismrheumatism

EndocarditisEndocarditis:: valvular, chordal, atparietal; valvular, chordal, atparietal; acute diffuse, acute warty, fibroplastic, acute diffuse, acute warty, fibroplastic, relapsing warty relapsing warty

MyocarditisMyocarditis: granulomatic (nodular : granulomatic (nodular productive), diffuse interstitial exudative, focal productive), diffuse interstitial exudative, focal interstitial exudative.interstitial exudative.

PericarditisPericarditis: serous, serofibrinous, fibrinous.: serous, serofibrinous, fibrinous. VasculitisVasculitis: capillaritis, arteriolaritis, arteritis.: capillaritis, arteriolaritis, arteritis.

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EndocarditisEndocarditis

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The heart tends to dilate

Acute and chronic valvulitis

Mural thrombi

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EndocarditisEndocarditis

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MyocarditisMyocarditis

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MyocarditisMyocarditis

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Morphology of Aschoff body 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

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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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.

PericarditPericarditisis

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PericarditPericarditisis

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Complications of chronic rheumatic heart disease

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 mitral valve orifice

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Complications of chronic rheumatic heart disease (continuation)

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 Polyarthritic form of rheumatismrheumatism

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Nodular form of Nodular form of rheumatismrheumatism

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Cerebral form of Cerebral form of rheumatismrheumatism

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System System lupus lupus erythematosuserythematosus

Lupus erythematosusLupus erythematosus (LE) (LE) is chronic polysyndrome is chronic polysyndrome disease mostly of young women and girls, which disease mostly of young women and girls, which develops on a background the genetic predefined develops on a background the genetic predefined imperfection of immunoregulatory processes. It results to imperfection of immunoregulatory processes. It results to the uncontrolled productions of antibodies to own tissues the uncontrolled productions of antibodies to own tissues and their components with development of autoimmune and their components with development of autoimmune and immunocomplex chronic inflammation.and immunocomplex chronic inflammation.

Morbidity by LE makes 48-50 cases per 100 thousands Morbidity by LE makes 48-50 cases per 100 thousands of population, death rate – 5,8 per 1000 000 (at this 4,7 of population, death rate – 5,8 per 1000 000 (at this 4,7 are women).are women).

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PathomorphologyPathomorphology

Arteriolitis, capillaritis, vasculitis, interstitial Arteriolitis, capillaritis, vasculitis, interstitial inflammation of all internal organs with inflammation of all internal organs with passing to the sclerosis, periarterial “bulbous” passing to the sclerosis, periarterial “bulbous” sclerosis of spleen, hyperproductions of sclerosis of spleen, hyperproductions of immunoglobulins, losing of DNA, presence of immunoglobulins, losing of DNA, presence of lupusal cells, erythema of skin (contour of lupusal cells, erythema of skin (contour of butterfly), endocarditis of butterfly), endocarditis of ЛібманаЛібмана and Saxon, and Saxon, гломерулонефритгломерулонефрит, , поліартритполіартрит without without deformation of jointsdeformation of joints

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Symptom of “Butterfly”Symptom of “Butterfly”

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Endocarditis of Libman-Endocarditis of Libman-SacksSacks

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Periarterial “bulbous” Periarterial “bulbous” sclerosissclerosis

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GlomerulonephritisGlomerulonephritis

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Rheumatoid arthritisRheumatoid arthritisRheumatoid ArthritisRheumatoid Arthritis (RA) (RA)

(polyarthritis)(polyarthritis) is the chronic system is the chronic system diseaseof connective tissue with the diseaseof connective tissue with the progressive defeat of peripheral (synovial) progressive defeat of peripheral (synovial) joints mostly as the type of erosive-joints mostly as the type of erosive-destructive polyarthritis.destructive polyarthritis.

RRАА is registered in the all countries of the is registered in the all countries of the world and all world and all climate-geographical climate-geographical areas with areas with frequency from 0.6 to 1.3 %. Everywhere frequency from 0.6 to 1.3 %. Everywhere women are unwell more frequent (3-4:1).women are unwell more frequent (3-4:1).

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Etiology and pathogenyEtiology and pathogenyThe defeat of connective tissue (mostly joints) is a result of The defeat of connective tissue (mostly joints) is a result of

immunopathological processes (autoaggressions).immunopathological processes (autoaggressions).It is consider that the violation of immune answer regulation It is consider that the violation of immune answer regulation

as a result of disbalance of function of Т- and В-lymphocytes at as a result of disbalance of function of Т- and В-lymphocytes at RA is the reason of immunocomplex defeat. Because the deficit RA is the reason of immunocomplex defeat. Because the deficit of the system of T- lymphocells leads to activating of В- of the system of T- lymphocells leads to activating of В- lymphocells and uncontrolled synthesis of antigenes (IgG) by the lymphocells and uncontrolled synthesis of antigenes (IgG) by the plasmatic cells. Antigenes IgG at RA are changed possess plasmatic cells. Antigenes IgG at RA are changed possess [pe'zes] (володіти) autoreactivity. That is why antibodies of [pe'zes] (володіти) autoreactivity. That is why antibodies of ІІgG, gG, ІІggММ, , ІІgA (rheumatoid factors) act against abnormal antigenes. At gA (rheumatoid factors) act against abnormal antigenes. At interaction (co-operation) of rheumatoid factors with IgG immune interaction (co-operation) of rheumatoid factors with IgG immune complexes formed and initiate the row of chain reaction.complexes formed and initiate the row of chain reaction.

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PathomorphologyPathomorphology

Arteriitis, arteriolitis, progressive Arteriitis, arteriolitis, progressive destructive polyarthritis, fibrotic-destructive polyarthritis, fibrotic-bone ankilosis , osteoporosis, bone ankilosis , osteoporosis, polyserositis (multiplemeaning), polyserositis (multiplemeaning), glomerulonephritis, pyelonephritis, glomerulonephritis, pyelonephritis, amyloidosis of kidneys, amyloidosis of kidneys, cardiosclerosiscardiosclerosis

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Rhematoid synoviitisRhematoid synoviitis

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Complication of Complication of Rheumatoid ArthritisRheumatoid Arthritis

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Rheumatoid ArthritisRheumatoid Arthritis

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Systemic scleroderma Systemic scleroderma (scleriasis) (Progressive (scleriasis) (Progressive systemic sclerosis)systemic sclerosis)

Systemic scleroderma is Systemic scleroderma is the system the system disease of connecting tissue and small vessels, disease of connecting tissue and small vessels, which is characterized by the widespread fibrotic-which is characterized by the widespread fibrotic-sclerotic changes of skin, stromae of internalss sclerotic changes of skin, stromae of internalss and symptomes of obliterated endocarditic in the and symptomes of obliterated endocarditic in the form of syndrome of Reino.form of syndrome of Reino.

Primary morbidity in the USA is known only – Primary morbidity in the USA is known only – 12 accidents on the 1 million populations in a 12 accidents on the 1 million populations in a year. The women are unwell in 3-7 times more year. The women are unwell in 3-7 times more frequent. Age-dependent peak – 30-60 years.frequent. Age-dependent peak – 30-60 years.

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Etiology and pathogenyEtiology and pathogeny

Etiology of Etiology of Systemic scleroderma Systemic scleroderma is is unknown. The violation of microcirculation as a unknown. The violation of microcirculation as a result of the defeat of vascular wall and of plasma result of the defeat of vascular wall and of plasma and cellular properties of blood is the important and cellular properties of blood is the important factor of pathogeny of factor of pathogeny of Systemic sclerodermaSystemic scleroderma. . This leads to surplus synthesis of soluble forms of This leads to surplus synthesis of soluble forms of collogen, damage of endothelium and its collogen, damage of endothelium and its substitution by smooth muscle collagen-producing substitution by smooth muscle collagen-producing , increasing of possibility to the spasm and , increasing of possibility to the spasm and hyperplasia of internal membrane of vesselshyperplasia of internal membrane of vessels . .

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PathomorphologyPathomorphology

Arteriitis, arteriolitis, sclerosis, Arteriitis, arteriolitis, sclerosis, hyalinosis, atrophy of skin hyalinosis, atrophy of skin (parchment skin), sclerodermic heart (parchment skin), sclerodermic heart (large nidusal cardiosclerosis), (large nidusal cardiosclerosis), sclerodermic (cortical necrosis), sclerodermic (cortical necrosis), basal pneumofibrosis.basal pneumofibrosis.

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Systemic sclerodermaSystemic scleroderma

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Systemic sclerodermaSystemic scleroderma

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Nodular periarteritisNodular periarteritis Nodular periarteritisNodular periarteritis is system necrotic is system necrotic

vasculitis as the type segmental defeat of vasculitis as the type segmental defeat of small and middle caliber arteries with small and middle caliber arteries with formation of the aneurysm protrudes. The men formation of the aneurysm protrudes. The men of young age are ill mostly, morbidity makes of young age are ill mostly, morbidity makes 2-3 on the 1 million populations in the year.2-3 on the 1 million populations in the year.

EtiologyEtiology. Nodular periarteritis. Nodular periarteritis develops develops after the acute respirators infections including after the acute respirators infections including the streptococcus illness,the streptococcus illness,

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PathomorphologyPathomorphology The defeat of arteries of muscular type of The defeat of arteries of muscular type of

small and middle calibers in the area of their fork small and middle calibers in the area of their fork is the most characteristic morphological sign of is the most characteristic morphological sign of nodular periarteritis. The feature of nodular nodular periarteritis. The feature of nodular periarteritis is the simultaneous defeat of periarteritis is the simultaneous defeat of endothelia of vessels (deposit of immune endothelia of vessels (deposit of immune complexes), internal elastic membrane complexes), internal elastic membrane (polymorphic-cellular inflammation contains the (polymorphic-cellular inflammation contains the lymphoid epithelioid neutrophiles, fibroblasts) lymphoid epithelioid neutrophiles, fibroblasts) and perivascular tissue (cellular infiltration and and perivascular tissue (cellular infiltration and scarring).scarring).

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Nodular periarteritisNodular periarteritis

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I thank you for I thank you for attentionattention !!