医学免疫学课件：Complement and system lupus erythematosus.pptxVIP

Complement and system lupus erythematosus;Introduction ?Complement system ?Systemic lupus erythematosus(SLE) C1q deficiency SLE C2C4 deficiency SLE Mechanisms Hypothesis ;The activation pathways and regulation of complement system;The functions of complement system ?Opsonization ?Clearance of ICs ?Connection of innate immunity and adaptive immunity ?Complement dependent cytotoxicity;SLE Manifestations Features: ?Autoantibodies: anti-nuclear antibodies, anti-phospholipid antibodies, etc. ?Injury to the skin, joints, kidney, and serosal membranes is prominent. ?Every other organ in the body may also be affexted. ?Fairly common, 1 in 2500 in certain populations. ?Predominantly affects women, 1 in 700 among women of childbearing age. ;SLE Pathogenesis: ?Genetic factors: Higher rate of concordance(20%)in monozygotic twins when compared with dizygotic twins(1% to 3%). Inherited deficiencies of early complement components, such as C2, C4, or C1q. ;SLE Pathogenesis: ?Immunologic factors: Failure of self-tolerance in B cells CD4+ helper T cells specific for nucleosomal antigens also escape tolerance and contribute to the production of high-affinity pathogenic autoantibodies. ;SLE Pathogenesis: ?Environmental factors: Exposure to ultraviolet(UV) light Sex hormones(gender bias) Drugs, such as hydralazine, procainamide, and D-penicillamine can include and SLE-like response in humans. ;Complement Deficiencies in Systemic Lupus Erythematosus. Angela R. Bryan Eveline Y. Wu Springer Science+Business Media New York 10 May 2014 ;Clinical presentation of human C1q deficiency: How much of a lupus? Mihaela Stegert a, Merete Bock a, Marten Trendelenburg Molecular Immunology 67 (2015) 3–11;Schejbel, L., Skattum, L., Hagelberg, S., Ahlin, A., Schiller, B., Berg, S., Genel, F., Trueds- son, L., Garred, P., 2011. Molecular basis of hereditary C1q deficiency – revisited: identification of several novel disease-causing mutations. Genes Immun. 12, 626–634. ;Lewis MJ, Bot