[Systemic lupus erythematosus and antiphospholipid syndrome].
Systemic lupus erythematosus (SLE) is a chronic autoimmune disorder characterized by the synthesis of antinuclear antibodies. Nucleosomes-the disc-like-structural unit of chromatin being composed of histones and DNA-are the primary antigenic structure that induces the formation of complement-activating antigen-antibody complexes in basement membranes. The autoreactivity in SLE has been elucidated in drug-induced SLE: Hypomethylation of DNA leads to overexpression of integrin CD 11a/CD 18, increased binding of T-cells to macrophages and B-cells, a higher rate of apoptosis of macrophages and elevated B-cell activity with consecutive production of autoantibodies. Disturbances of apoptosis (e.g. mutation of Fas gene) are relevant also in non drug-induced SLE. The morphologic diagnosis-by light microscopy, immunohistology and electron microscopy-of skin and renal biopsies can confirm the diagnosis and help in prognostic assessment as well as therapeutic decisions in SLE. The value of the morphologic diagnosis in SLE is enhanced by reporting semiquantitative scores of disease activity and chronicity. The antiphospholipid syndrome (APS) is characterized by thrombosis, thrombocytopenia, recurrent fetal loss and antiphospholipid antibodies. APS associated with SLE or other systemic autoimmune diseases has been termed secondary APS. Antibodies in APS are directed against phospholipids and phospholipid-protein complexes. One of these proteins is beta 2-glycoprotein 1. An important mechanism of the increased rate of thrombosis in APS is probably the inhibition of the protein C catalyzed inactivation of clotting factors V and VIII. Venous thrombosis with recurrent pulmonary emboli and arterial thrombi in brain, heart and kidney (thrombotic microangiopathy) can lead to divergent clinical manifestations in APS.
Gröne
H J
HJ
Medizinisches Zentrum für Pathologie, Philipps-Universität Marburg.
D000818:Animals; D017152:Antibodies, Antiphospholipid / Q000502:physiology; D015703:Antigens, CD / Q000276:immunology; D016736:Antiphospholipid Syndrome / Q000276:immunology* / Q000473:pathology*; D017209:Apoptosis; D001402:B-Lymphocytes / Q000276:immunology; D001779:Blood Coagulation Factors / Q000502:physiology; D006801:Humans; D008180:Lupus Erythematosus, Systemic / Q000276:immunology* / Q000473:pathology*; D008181:Lupus Nephritis / Q000473:pathology; D008264:Macrophages / Q000276:immunology; D013601:T-Lymphocytes / Q000276:immunology
D017152:Antibodies, Antiphospholipid; D015703:Antigens, CD; D001779:Blood Coagulation Factors
Systemischer Lupus erythematodes und Antiphospholipid-Syndrom.