Systemic Lupus Erythematosus XUE Yu Department of Rheumatology Huashan Hospital, Fudan University June 5th. 2013
Systemic Lupus Erythematosus XUE Yu Department of Rheumatology Huashan Hospital，Fudan University June 5th, 2013
Introduction Systemic lupus erythematosus sLE is a chronic autoimmune disease that can be fatal however with recent medical advances fatalities are becoming increasingly rare The immune system attacks the body,'s cells and tissue, resulting in inflammation and tissue damage SLE can affect any part of the body, but most often harms the heart, joints, skin, lungs, blood vessels, liver, kidneys, and nervous system Lupus can occur at any age, and is most common in women particularly 90% of patients are women of child-bearing age
Introduction • Systemic lupus erythematosus (SLE) is a chronic autoimmune disease that can be fatal; however, with recent medical advances, fatalities are becoming increasingly rare. • The immune system attacks the body’s cells and tissue, resulting in inflammation and tissue damage. • SLE can affect any part of the body, but most often harms the heart, joints, skin, lungs, blood vessels, liver, kidneys, and nervous system. • Lupus can occur at any age, and is most common in women, particularly 90% of patients are women of child-bearing age
Epidemiology Incidence: 1 in 1. 000-10.000 Female to male ratio: 9-1 More common in African-Americans but it affects all races Mean age of onset: 28 years Positive family history in 10-15% of patients Monozygotic twins exhibit a greater rate of concordance(24%)than dizygotic twins(1-3%) The risk of a child developing lupus born from a mother (or father) with lupus is calculated to be 3-4% at worst
Epidemiology • Incidence: 1 in 1,000 -10,000 • Female to male ratio: 9-1 • More common in African-Americans but it affects all races • Mean age of onset: 28 years • Positive family history in 10 -15% of patients • Monozygotic twins exhibit a greater rate of concordance (24%) than dizygotic twins (1-3%) • The risk of a child developing lupus born from a mother (or father) with lupus is calculated to be 3-4% at worst
Etiology The cause(s)of lupus is currently unknown, but there are environmental and genetic factors involved 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
Etiology • The cause(s) of lupus is currently unknown, but there are environmental and genetic factors involved. 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
Immunogenetics Increased Risk for slE in. HLA-DR2 (anti-DNA Abs HLA-DR3(anti-Ro Abs) Null alleles at c2 and C4 loci SLE may be transmitted in an autosomal dominant pattern(family studies)
Immunogenetics Increased Risk for SLE in: • HLA-DR2 (anti-DNA Abs) • HLA-DR3 (anti-Ro Abs) • Null alleles at C2 and C4 loci • SLE may be transmitted in an autosomal dominant pattern (family studies)