2. •Systemic lupus erythematosus (SLE) is the
chronic polysyndromic disease developing on a
background of genetically caused imperfection
of immune regulator processes, leading to
uncontrollable production of antibodies to own
cells and their components, with development
of autoimmune and immune complex chronic
inflammation
3. •Now becomes more and more obvious, that SLE
does not concern any more to rare diseases:
disease meets with frequency 2-4 on 10.000
populations in all climatic geographic zones and
on all continents. In children's age girls of
pubertal age is more often falling ill (ratio to
boys 9:1).
4. • Etiology of SLE. Concrete etiologic factor at SLE is not
established (possibly, plays a role multifactor influence).
Among available hypotheses of disease appearance the
greatest attention the following deserve:
Factors of an environment. There is an opinion, that viruses,
toxins and medical products can cause development of SLE,
but convincing data are not present. About the virus nature
clinical manifestations (erythema, enanthema,
pancytopenia), similarity of immune disorders at SLE and
AIDS (lymphocytopenia, decrease of T-helpers), revealing of
markers of chronic virus infection (lymphocytotoxic
antibodies) testify.
5. • Genetic factors. Research of family cases of disease and disease
of twins reveal genetic predisposition to SLE development.
Numerous researches prove connection of the certain
combinations of antigens of HLA-system (system of the genes
regulating height of the immune response to a concrete antigen,
localized in 6-th chromosome) – А11, В7, В35, DR2, DR7, and
different combinations of antigens are certain at various types of
SLE course, and also at a prevalence of this or that damage
(nephritis or damage of CNS).
Autoimmunity. Loss of tolerance to autoantigens (to components
of connective tissue) with formation of autoantibodies
(antinuclear AA against DNA of connective tissue cells) is
consequence of genetically determined disorder of immune
regulator functions which consequence is superfluous activity of
B-lymphocytes.
6. • Estrogenic stimulation. A line of clinical researches
confirms stimulating influence of estrogenic
hormones on development of SLE (not without
reason, in pediatrics it is defined as disease of the
pubertal period girls). Moreover, decrease of
testosterone and relative increase of estradiol in boys
with SLE is described.
7. • Pathogenesis of SLE. The starting factor of autoimmunity failure
with production of antinuclear AA against nucleuses (DNA) of
connective tissue cells (fibroblasts) can be viral infection, insolation,
exposing to the wind, overcooling, influence of chemical factors
(including medicines). Pathogenetic value of consists in their ability
to form circulating immune complexes (CIC): as AA here antinuclear
AA (Ig G and M) acts, as AG – native DNA of fibroblasts nucleoses (n-
DNA). CIC are fixed in blood vessels and renal glomerulas, leading to
their 2 damage: under influence of complexes and during their
removal a line of mediators is formed, which basic role consists in
maintenance of the conditions promoting to fagocytosis of CIC and
their digestion (complement, lysosomal enzymes, cinins, hystamine,
serotonin, super-oxide anion-radical), however their surplus causes
formation of the focal of autoimmune inflammation.
8. • One of ways, by means of which CIC have the damaging
effect, is activation of serum complement with decrease in
its early components and disappearance of C3-component
(simultaneously last in glomerulas reveals). There is a direct
communication between level of С3-component of
complement and activity of lupus-nephritis (last that higher,
than low level of С3-component in blood). In places of
damage of connective tissue pathomorphologically the
amorphous masses of nuclear substance painted in blue-
purple color by hematoxylin are defined. These products of
nuclear disintegration (karyorrhexis) carry the name of
hematoxylinic bodies.
9. • Phagocytosis of nuclear disintegration products
(hematoxylinic bodies) by neutrophils leads to formation of
LE-cells (from Lupus Erythematodes) – neutrophils which
cytoplasm is filled by absorb nucleus of connective tissue
cell (hematoxylinic body), and nucleus of neutrophil is
shifted to periphery (it is split above hematoxylinic body).
Diagnostic value has also phenomenon of rosette-formation
– surroundings of hematoxylinic body by neutrophils. Thus,
in SLE pathogenesis the great value has both autoimmune
mechanism (formation of antinuclear AA at external
influences on a background of genetically caused
imperfection of immune regulation), and dynamically
connected with it immune complex mechanism with
activation of system of complement and others mediators
of inflammation in places of immune complexes fixing.
10. • Classification of SLE is based on definition of activity
of disease, features of its development and the
subsequent course. On activity active (three degrees
of activity) and inactive (remission) phases of SLE are
distinguished. On course acute, subacute and chronic
(primarily-chronic) are distinguished. The last in the
diagnosis takes out damage of various organs and
systems
11. • Clinical picture of SLE is defined by type of course and activity of
disease. At acute course patients can specify day when fever, acute
polyarthritis, serosits (pleurisy, pericarditis) has begun, "butterfly"
on the face has appeared. The nearest 3 -6 months from the
beginning of disease development of lupus-nephritis or CNS
damage, expressed multitude of syndromes is marked. Duration of
disease without treatment no more than 1 -2 years, but at early
diagnostics and active overwhelming (and subsequently and
supporting) treatment prognosis improves; cases of full clinical-
laboratory remission are described. Now such variant of SLE course
mainly in children, teenagers and young men is revealed (but much
less often, rather than 20-30 years ago).
12. • Subacute course meets most often: disease begins gradually, with the
general symptoms, arthralgias, relapsing arthritises, various
nonspecific damages of skin. Waviness of course is especially distinct,
and at every aggravation other organs and systems are involved in
process: within 2-3 years characteristic multitude of syndromes
develops, with frequent development of lupus-nephritis and
encephalitis. On M.S.Ignatova, at subacute SLE renal syndrome arises
less often (in 1/2 of patients) and later, on 3-4 year of disease. At this
variant extremely important timely diagnostics of disease, early active
treatment by big dozes of steroids and cytostatics and practically
constant supporting therapy, however quite often CRF develops.
13. • Damages of organs and systems at SLE are presented by following
changes: * Skin – erythematous dermatitis of cheeks, cheekbones and
bridge of nose as «butterfly»; discoid lupus erythematous (the focals
remind under the form coins with hyperemated edges, atrophy and
depigmentation in the center); nodular lupus damage of skin; the
damage caused by vasculitis (capillaritis, nettle rash, microinfarcts,
reticular livedo – treelike figure on skin of the lower extremities due
to phlebitis); photosensitization (skin rash at ultraviolet irradiation);
alopecia (focal and generalized); * Mucous – cheulitis, erosions; *
Joints – arthralgias; symmetric non erosive polyarthritis without
deformations; arthropathies with stable deformations (Jaccu
syndrome) due to involving in process of ligaments and sinews
(erosion are not present); * Muscles – myalgias, myositises,
glucocorticoid myopathy;
14. • Serous membranes – polyserositis, pleurisy and pericarditis (exudative, dry,
adhesive), perihepatitis, perisplenitis;
• * Heart – myocarditis, endocarditis, mitral regurgitation;
• Lungs – pneumonitis (bilateral flying infiltrations, frequently with hemoptysis),
pneumosclerosis, pulmonary hypertension at the isolated damage of
pulmonary artery;
• * Kidneys – lupus-nephritis, (focal, membranous, mesangioproliferative,
fibroplastic);
• * GIT – weakening of peristalsis of esophagus; erosion of mucous of stomach
and duodenum; hepatomegaly (without essential disorder of function),
interstitial vasculitis infarcts of intestines, perforations and bleedings;
• * Lymphatic system – splenomegaly, lymphoadenopathy; 4
• * CNS – generalized manifestations (depression, psychoses, disorders of
cognitive abilities, spasms); local (focal) disorders (hemiparesises, dysfunctions
of craniocereberal nerves, myelitises, and disorders of movement); cranial and
peripheral neuropathy; chorea;
15. • * Sjegren's syndrome - damage of secretory epithelial glands with
development of dry keratoconjunctivitis, parotitis, dryness of mucous of
oral cavities, pharynx, throat, trachea, genitals, skin, GIT;
• * Raynaud's syndrome – intermittent (periodic, alternating) attacks of
excessive pallor, and then with cyanosis of the fingers, provoked by a cold
or negative emotions. Warming causes vasodilation and intensive
reddening of skin with sensation of pulse and paresthesia («pricking»);
• * Antiphospholipid syndrome – arises on a background of hyperproduction
of antibodies to phospholipides of cellular membranes with thromboses of
various localizations.
