系统性红斑狼疮教材课程.pptVIP

Systemic Lupus Erythematosus;Background:;Epidemiology and Etiology Pathophysiology Clinical features Treatment ;Epidemiology and Etiology;Frequency;Causes;At least 10 different gene loci are known to increase the risk for SLE. A genetic predisposition is supported by the 10-fold increase in concordance among monozygotic twins versus dizygotic twins. Studies of the human leukocyte antigens (HLA) reveal that HLA-DR2 and HLA-DR3 occur more often in people with SLE than in the general population.;The presence of the null complement alleles and congenital deficiencies of complement (especially C4, C2, and other early components) are also associated with an increased risk of SLE.;Environmental factors;Pathophysiology; In SLE, many genetic-susceptibility factors, environmental triggers, antigen-antibody responses, B-cell and T-cell interactions, and immune clearance processes interact to generate and perpetuate autoimmunity. ;One proposed mechanism for the development of autoantibodies involves: A defect in apoptosis that causes increased cell death and a disturbance in immune tolerance. The redistribution of cellular antigens during apoptosis leads to a display of plasma and nuclear antigens on the cell surface. Thus, dysregulated (intolerant) lymphocytes begin targeting normally protected intracellular antigens. Immune complexes form in the microvasculature, leading to complement activation and inflammation. Antibody-antigen complexes deposit on the basement membranes of skin and kidneys. ;?CLINICAL;;Constitutional;Musculoskeletal;Dermatologic ;;;;photosensitive rash may be elicited from patients if they have any unusual rash or symptom exacerbation after sun exposure. ; Photosensitive SLE rashes are often macular and typically occur on the face , arms, or extremities, which are sun-exposed regions.;Discoid lesions often develop in sun-exposed areas but are plaquelike lesions with follicular plugging and scarring. They may be part of systemic lupus or may