狼疮讲课稿1概要1.ppt

Systemic Lupus Erythematosus(SLE)系统性红斑狼疮 Gao Guanmin(高冠民) 郑州大学一附院风湿科 Definition Systemic attack many organs of the body LUPUS ( Latin word ) A malignant ulcer consuming the nether parts is very hungry like unto a wolf Erythematosus Definition Prototypic AID* Chronic, multisystem Multiple autoantibodies which may contribute to tissue damage Auto-Immune DiseasesAID “The conquerors make war upon themselves Blood against blood, self against self” From King Richard III William Shakespeare (1592) Brief History 1845 : butterfly facial rash described 1895 -1903 : systemic form 1935:wire loop by postmortem exam 1948 : discovered the LE cell 1950s : LE be part of an antinuclear antibody reaction 1949: Steroid,antibiotic drugs Etiology and Pathogenesis Sex homone Female to male ratio -- 9:1 Genetic Predisposition Centered on abnormalities in T- cell-B-cell tolerance HLA-DR2,3 HLA-DQ TNFαgene TCR β gene Etiology and Pathogenesis PATHOGENESIS OF SLE AUTOANTIBODIES ANTIGEN IMMUNE COMPLEX(IC) INVOLVE ALL CONNECTIVE TISSUES Fibrinoid degeneration in vessel wall MULTIORGANS INJURED Fluorescent Band Clinical Features Heterogeneity A great imitator Varied subtype Varied syndrome: No diagnostic criteria, Only classification criteria  Internal Medicine Is a Branch of Lupus Depend on What Organs Involved, What Symptoms Have in Clinic  Morris Ziff Dallas 1990 Constitutional Symptoms Fever Extreme fatigue Weight loss Skin and Mucous Sensitivity to the sun Butterfly rashes Discoid lesions Oral ulcer Unusual loss of hair Hand Cutaneous