【正文】 pericardial effusion) 7. Renal : a. Persistent proteinuria ( g/d or 3+) disorder OR b. Cellular casts of any type 8. Neurologic: a. Seizures (in the absence of other causes) disorder OR b. Psychosis (in the absence of other causes) 9. Hematologic: a. Hemolytic anemia disorder OR b. Leukopenia (4,000/mL on two or more occasions) OR c. Lymphopenia (l,500/mL on two or more occasions) OR d. Thrombocytopenia (100,000/mL in the absence of offending drugs) 10. Immunologic disorder: a. Antidoublestranded DNA OR b. AntiSm OR c. Positive finding of antiphospholipid antibodies based on (1) an abnormal serum level of IgG or IgM anticardiolipin antibodies, or (2) a positive test result for lupus anticoagulant using a standard method, or (3) a falsepositive serologic test for syphilis known to be positive for at least 6 months and confirmed by Treponema palliclum immobilization or fluorescent treponemal antibody absorption test 11. Antinuclear antibody: An abnormal titer of antinuclear antibody (ANA) by immunofluorescence or an equivalent assay at any time and in the absence of drugs known to be associated with druginduced lupus syndrome. For identifying patients in clinical studies, a person shall be said to have SLE if any four or more of the 11 criteria are present, either serially or simultaneously, during any interval or observation. This criteria holds a sensitivity of 85% and a specificity of 95%. TREATMENT Medical Care: Care of patients with systemic lupus erythematosus (SLE) depends on individual patient39。 associated with neonatal lupus Antiribosomal P Unmon antibodies that may correlate with lupus cerebritis AntiRNP indicate mixed connective tissue disease with overlap SLE, scleroderma, and myositis Anticardiolipin used to screen for antiphospholipid antibody syndrome ? Lupus anticoagulant Multiple tests (eg, Direct Russell Viper Venom test) to screen for inhibitors in the clotting cascade in antiphospholipid antibody syndrome ? Coombs test Coombs test–positive anemia to denote antibodies on RBCs ? Antihistone Druginduced lupus (DIL) ANA antibodies often this type (eg, with procainamide or hydralazine。 sensitivity only 70%。Systemic Lupus Erythematosus Dr. Ye yujin Rheumatology department The 1st Affiliated Hospital of Sun YatSen University 20221230 Background: Systemic lupus erythematosus (SLE) 1) A chronic, multifaceted inflammatory autoimmune disease 2) Affect every an system of the body 3) SLE is protean in its manifestations and follows a relapsing and remitting course Epidemiology and Etiology Pathophysiology Clinical features Treatment Epidemiology and Etiology Frequency Worldwide , the prevalence of SLE varies with ethnicity and geographical locations: 70100 cases per 100,000 population in China. 50 cases per 100,000 population in the US, while 200 cases in the AfricanAmerican women. 12 cases per 100,000 population in Britain 39 cases per 100,000 population in Sweden Sex Hormonal factors are important in the pathogenesis of the disease: ? In women of reproductive age , incidence of SLE is 6 to 10 fold higher than men. ? Additionally, pregnancy and administration of exogenous estrogen often precipitate exacerbations of SLE. ? Men at all ages have a risk of disease similar to that of women who are prepubertal or postmenopausal. Age ? Disease incidence is highest among women aged 1464 years, because of female sex hormone production. ? Males do not have an agerelated peak in incidence. Causes ? Although the specific cause of SLE is unknown, multiple factors are associated with the development of the disease. ? These include geic, racial, hormonal, and environmental factors. ? At least 10 different gene loci are known to increase the risk for SLE. – A geic predisposition is supported by the 10fold increase in concordance among monozygotic twins versus dizygotic twins. – Studies of the human leukocyte antigens (HLA) reveal that HLADR2 and HLADR3 occur more often in people with SLE than in the general population. Geic factors – The presence of the null plement alleles and congenital deficiencies of plement (especially C4, C2, and other early ponents) are also associated with an increased risk of SLE. Environmental factors ? UV exposure ? Microbial response ? Drugs Pathophysiology ? SLE is an autoimmune disorder that involves multisystem microvascular inflammation with the generation of autoantibodies. ? Many immune disturbances, both innate and acquired, occur in SLE, as noted in image 1 In SLE, many geicsusceptibility factors, environmental triggers, antigenantibody responses, Bcell and Tcell 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 plexes form in the microvasculature, leading to plement activation and inflammation. ? Antibodyant