LUPUS ERYTHEMATOSUS

Lupus erythematosus(Libman-Sacks disease) is either acute or chronic disease with involvement of connective tissue with marked autoimmunization and damage of skin, vessels, kidneys. Its incidence is the highest in young women (aged 18 — 23), the disease is less frequent in elderly women and in men.

The etiology of the disease is not clear, it may be caused by RNA virus.

Pathology. Lupus erythematosus is characterized by different cellular and tissue changes which can be divided into 5 groups:

1. Acute necrotic and degenerative changes of the connective tissue (all stages of deorganization). Fibrinoid is characterized by abundant nuclear protein and chromatin granules.

2. Subacute interstitial inflammation of all organs including nervous system with involvement of microcirculation (capillaritis, arteriolitis, vasculitis).

3. Changes of sclerotic character caused by the above changes. This group is characterized by onion-like sclerosis in the spleen.

4. Changes of the immune system. Focal accumulations of leukocytes with marked plasma-tization are present in the central and peripheral organs. Macrophage activity is increased.

5. Nuclear pathology in the cells of all organs and tissues, particularly in the lymphatic nodes. The shape of the nuclei does not change but they gradually lose DNA and look pale after staining. After the death of the cell, the nucleus disintegrates into granules, i.e. hematoxylin bodies. This phenomenon characterizes lupus erythematosus. Neutrophils and macrophages phagocytize hematoxylin bodies and form lupus cells. Their presence in the blood is a significant sign of lupus erythematosus. Except for the blood, they can be found in the bone marrow, spleen, lymphatic glands, the walls of the vessels.

Visceral manifestations of lupus erythematosus: lupus endo-, myo- and pericarditis, abacterial warty endocarditis called Libman-Sacks endocarditis. Kidney: two types of glomerulonephritis are known: 1) lupus nephritis, 2) common glomerulonephritis.

Lupus nephritis. The kidney is large, motley-colored due to hemorrhages. Microscopic examination reveals hematoxylin bodies, thickening of capillary membrane in the glomeruli with «wire loop» appearance, with hyaline thromboses and foci of

fibrinoid necrosis with immune complex fixation. The outcome is granular kidney and uremia.

In rare cases joints may be involved without their deformation.

Skin. On the lateral surfaces of the face there are symmetrical red desquamative areas which are called «butterfly formations)). In acute stages, rash can be present on the other regions of the body. Sclerosis, hyperkeratosis, atrophy of sweat and sebaceous gland develop, which may cause alopecia. Complications: granular kidney followed by uremia, endocarditis, purulent and septic processes due to steroid therapy.

Death is caused by uremia and sepsis.






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