Systemic Lupus Erythromatosis
SLE
- antinuclear antibodies
- 10:1 more in females
- although lupus has a very characteric presentation, the symptoms are also found in others so diagnosis is based on: some of the less common features, characteric distribution of symptoms and blood tests.
- In blood tests, 95% of cases test positive for the ANA although it can be found in healthy people too. A more specific te4st for lupus is to test for anti-double-stranded dna antibodies and anti-SM AB , the levels of which reflect the activity of the disease
- results in complement deficiency where some actually have a genetic deficiency in complement components whilst in others, there is an acquired defect of the system
- thought to be the result of its consumption by autoantibodies which initially react to the component C1q.
- the lack of efficient complement has a number of consequences
1. immune complexes cannot be cleared and as a result they are deposited in vascular basement membranes or access tissue macrophages
2. It also impairs resistance to infectiom
3. Is impairs selection deletion of autoreactive V cells resulting in the formation of a range of autoantibodies
- the complement system is not the only target of autoantibodies in SLE; some act directly on tissues such as the kidneys, red blood cells, platelets and others, including the brain.
- these autoantibodies are so effective because they are able to perpetuate their production through a varierty of bypass mechanisms including impaired clearances of nuclear debris and signalling to B cells.
-the clinical features of lupus therefore correspond to the antibody targets seen in the table below:
Tissue | Clinical feature | | | | | | | | |
Cerebritis
- change in mood or personality
- epilepsy
- infarct or haemorrhage presenting as stroke
- rise in ICP
- drowsiness
- a large proportion of morbidity and mortality in patients with SLE is caused by the neuropsychiatric complications,
- antinuclear antibodies
- 10:1 more in females
- although lupus has a very characteric presentation, the symptoms are also found in others so diagnosis is based on: some of the less common features, characteric distribution of symptoms and blood tests.
- In blood tests, 95% of cases test positive for the ANA although it can be found in healthy people too. A more specific te4st for lupus is to test for anti-double-stranded dna antibodies and anti-SM AB , the levels of which reflect the activity of the disease
- results in complement deficiency where some actually have a genetic deficiency in complement components whilst in others, there is an acquired defect of the system
- thought to be the result of its consumption by autoantibodies which initially react to the component C1q.
- the lack of efficient complement has a number of consequences
1. immune complexes cannot be cleared and as a result they are deposited in vascular basement membranes or access tissue macrophages
2. It also impairs resistance to infectiom
3. Is impairs selection deletion of autoreactive V cells resulting in the formation of a range of autoantibodies
- the complement system is not the only target of autoantibodies in SLE; some act directly on tissues such as the kidneys, red blood cells, platelets and others, including the brain.
- these autoantibodies are so effective because they are able to perpetuate their production through a varierty of bypass mechanisms including impaired clearances of nuclear debris and signalling to B cells.
-the clinical features of lupus therefore correspond to the antibody targets seen in the table below:
Tissue | Clinical feature | | | | | | | | |
Cerebritis
- change in mood or personality
- epilepsy
- infarct or haemorrhage presenting as stroke
- rise in ICP
- drowsiness
- a large proportion of morbidity and mortality in patients with SLE is caused by the neuropsychiatric complications,
Links: S.sh.18.1&link_from=S.sh.18|1&pdf_key=FPDDNCGCBDODNF00&pdf_index=/fs047/ovft/live/gv038/00002281/00002281-199609000-00004&link_set=S.sh.18|1|sl_10|resultSet|S.sh.18.19|0 - responsiveness to treatment varies on severity/number of neuropsychiatric manifestations. If multiple, less likely to respond to high-dose corticosteroids with or without cytotoxic additions such as cyclophosphamide. GOOD TO READ http://www.ncbi.nlm.nih.gov/pubmed/8941443 http://www.ncbi.nlm.nih.gov/pubmed/22467931 http://www.ncbi.nlm.nih.gov/pubmed/22379459 http://www.ncbi.nlm.nih.gov/pubmed/21909801 http://www.ncbi.nlm.nih.gov/pubmed/21909801