内科学教学课件：Systemic Lupus Erythematosus.pptx

Systemic Lupus Erythematosus Systemic Lupus erythematosus ( SLE ) is a syndrome of unknown aetiology most commonly affecting young women. Virtually any organ of the body may be involved . Typically the course of the disease is a series of remissions and exacerbations. With good management, the ten years survival may be over 90%. INTRODUCTION Etiology and Pathogenesis of SLE 1. Genetic factorMany studies have described familial aggregation of SLE. 5-13% of lupus have at least one first or second degree relative with lupusIt was found a 24-58% concordance in monozygotic twins. 2-5% concordance in dizygotic twins or siblings.. The risk of a child developing lupus born from a mother (or father) with lupus is calculated to be 3-4% at worst. What are the reasons of Genetic susceptibility?It seems likely that most of the genes predisposing to SLE are normal. An individual inherits an unlucky combination of normal genetic polymorphisms, each of which permit a little immune overreponse, or presentation of high quantities of target antigens in certain tissues. The combination of which is just enough to permit SLE to evolve after some environmental stimulus.C2, C4, C1q deficiencies, DR2, DR3, 1q41-42 region, Fc-r RIIA, IL10 and Bcl polymorphisms. 2. Environmental factorsUV light, especially UVB, flares SLE in most patients. It is unclear whether exposure to UV light can initiate the lupus, but onset after a sunburn is not unusual. There is good evidence that exposure of skin to UV light alters the location and chemistry of DNA as well as the availability of Ro and RNP antigens. Drug-induced lupus. Drugs ( hydralazine, procainamide, beta-blokers, isoniazid, penicillamine) can induce lupus. Drug-induce lupus may resemble SLE both clinically and serologically. Usually the disease is mild, and renal and neurological complications are rare. Generally, lupus that is caused by a drug exposure goes away once the drug is stopped. Allergy. Does it induce