systemic lupus erythematosus Flashcards
what is SLE in gen (summary)
• Systemic lupus erythematosus is a chronic autoimmune inflammatory disease that can affect any organ system, including msksltl system, characterized by production of wide array of Abs against nuclear and cytoplasmic cell components
how does gender r/t prevalence of SLE
• Females get it 10:1 vs males, this ratio is more like 30:1 in childbearing years
how does ethnicity r/t prevalence of SLE
- More common in African, Hispanic and Asian vs Caucasians
* In Canada its more severe in First Nations
prevalence of SLE
how many canadians have it
- It is a major rheumatic disease with prevalence of 1/ 2,000
- 15,000 Canadians have SLE
et of SLE
what kind of genetic implications are there
- Cause is unknown
- Genetic predisposition is evidenced by occurrence of familial cases of SLE esp among identical twins
- d/t inc in Aboriginals and blacks indicates genetic factors
- Cause is unknown
- Genetic predisposition is evidenced by occurrence of familial cases of SLE esp among identical twins
- d/t inc in Aboriginals and blacks indicates genetic factors
- up to 4 genes may be involved including: HLA-DR, HLA-DQ loci in the MHC II
- Imbalance of sex hormones may play role in dev of SLE.
- in summary: Genetic, hormonal, immunologic, and environmental factors
how do hormones r/t et of SLE
Androgens seem protective and E encourages dev. This may lead to heightened T helper and dec suppressor Tcells which miht lead to dev of autoAbs
not sure if nec but env triggers
• Env triggers:
o UVB light (specifically UVB assoc w exposure to sun or unshielded fluorescent bulbs may trigger exacerbations). 1/3 of SLE pts are photosensitive
o Chemicals eg drugs and hair dye
o Infectious agents
o Some drugs can promote a lupus like response (esp in elderly). Many drugs listed eg hydralazine and procainamide. The disease usually recedes when drug is d/c
patho of SLE
- Characterized by the formation of autoantibodies and immune complexes
- People with SLE have B cell hyperreactivity and inc production of autoAbs and Abs against nonself antigens.
- The B cells are polyclonal, each producing a diff type of Ab.
- The autoAbs can directly damage tissues or combine with corresponding antigens to form tissue damaging immune complexes. Antibodies have been identified against an array of nuclear and cytoplasmic cell components.
what kinds of Abs are involved in SLE
• autoAbs identified in SLE: antinuclear antibodies (including antideoxyribonucleic acid Abs) People with lupus (I assume you can just say Lupus judging by text instead of saying person with SLE??) also have self-antigens directed against blood (platelets, RBC, lymphocytes) and plasma proteins (clotting and complement factors)
maybe not important/in outcomes but what is the disease course?
acute or insidious?
pattern?
deadly?
- Onset may be acute or insidious
- Characterized by remissions and exacerbations
- Rare cases result in death in wks to months
which systems can SLE affect
• SLE has been called the great imitator because it can affect almost any organ system
with this in mind ( Instead of listing manifestations, identify the major organ systems affected and briefly describe the pathologic presentation in each)
and the fact I put more rather than less onto each card….
msksltl mnfts
what is most common?
what else is there
- Arthralgias (pain in a joint) and arthritis are most commonly occurring early sign (90% have arthralgia at some point during disease)
- Polyarthritis
- Osteoporosis
- myositis
mnfts r/t skin
- can vary greatly
- classified as acute, subacute or chronic
- acute skin lesions include the classic malar or “butterfly” rash on nose and cheeks (this is also seen in other conditions)
- hair loss
- sun sensitivity
mnfts r/t CNS
how common is CNS involvement?
- 30-75% have CNS involvement with pathologic basis unclear. May be d/t acute vasculitis altering blood flow; IR with antineuronal Abs; production of antiphospholipid Abs that damage blood vessels and->clots in brain
- Seizures (more frequent with renal failure)
- Psychotic symptoms: depression, euphoria, dec cog fx, confusion, alt LOC
mnfts r/t renal
- 50% of SLE pts have renal involvement
- Glomerulonephritis in several forms
- Nephrotic syndrome proteinuria edema
- May have renal failure
