系统性红斑狼疮.ppt

Systemic Lupus Erythematosus
Background:
Systemic lupus erythematosus (SLE)
1) A chronic, multifaceted inflammatory autoimmune disease
2) Affect every organ 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:
70-100 cases per 100,000 population in China.
50 cases per 100,000 population in the US,
while 200 cases in the African-American 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 14-64 years, because of female sex hormone production.
Males do not have an age-related peak in incidence.
Causes
Although the specific cause of SLE is unknown, multiple factors are associated with the development of the disease.
These include genetic, racial, hormonal, and environmental factors.
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.
Genetic factors
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