Systemic lupus erythematosus Flashcards
How common is systemic lupus erythematosus?
100000 GP’s -about 3 cases
rare, and people dont think about it
Chronic AID from the same family of RA, systemic sclerosis
Where does SLE sit in a sprectrum of AID of ocnnective issues?
Sits around the middle, with RA being the start and end being multiple sclerosis
What groups are more commonly affected by SLE? And which organs? genetic or not?
Females 9x more likely 15-40y/o Afro carribean, asian, chinese varies bewteen 4 and 280/100000 Principally affects joints and skins But can appear in lungs, kidneys, bone marrow, brain
has genetic association-mutltiple genes -not going into it
some are certain=C1q and C3
Other are just overrepresented in lupus
What the clinical features of SLE?
Presentation-malaise, fatigue, fever, weight loss,
Lymphadenpathy
->can be a lot of things like glandular fever
-> feel miserable overall
Characteritic featurs-Butterfly rash on face but not nose, alopecia, arthralgia
Reynauds presentation
=> Not not always there-just need to be at back of mind
life threatning-inflammation kidney, CNS, heart, lung
Accelerated artherosclerosis
vasculitis
specrtum of the disease is massive-so many organs can be involved
What are some clinical feature of SLE rash?
Rash can be very subtle on the cheeks, chin, neck forearms
avoids the nose/nasal labia folds
Redness-erythrema
No pus on the rash-not acne
If goes deeper in dermis-depigmentation, permanent scarring. also can lead to scarring alpoecia
What is the pathway leading to development of SLE?
Lot of things going wrong
Genetic predisposition->environement triggers (virus?)-> innate immune system activation, then adaptive
this probably happens a lot in patients but does go further-at some point adaptive comes in and that will end up causing lupus
This leads to immune complexes-main cause of the disease
-> complement activated AID
What is the relation bewteen SLE and apoptosis?
Seems like defect of the clearence of apoptotic cells in SLE-
OVer active immune system but at the same time and cant clear
AG on these cells hang around too long and allows development of auto-antinuclear AB against abnormally cleared apoptotic cells
defective clearance + lack of clearence
What is the pathway of pathophysiolgy of SLE?
Abnormal clearance-> APC uptake and activation of B cell -> class switch to auto IgG -> immune complexes to skin, organs, etc-> complement activation, inflammation
What are the laboratory tests that can identify SLE?
Serum for antinuclear AB-presence of ab that binds nuclei of cells (then find that with Anti AB AB)
THe pattern of the binding can help understand which one of different AID it is–SLE usually homogenous patterns
But not diagnostic but indicator
Refine with Anti-dsDNA and Sm-AB against dsDNA (present in 90% of patient. 20% have ANTI-SM-specific to lupus)
Anti-Ro and La -can be in sjorgens but also is LPS
What are non AB based tests for SLE?
Increased complement consumption-lower C3, C4 in blood and higher in skin
Might have pro-coagulat AB
FBC-lymphopaenia, normochronic aneamia
Leukopenia, thrombocytopenia
Renal-proteinuria, heamaturia
Active urinary sediment
=> need to assess which organs involved and monitor how severe it is->
What is the job of the doctor after an SLE diagnosis?
Cure and PRE-EMPT severe attacks
look at blood often to see any indication of early flares
Try and look at all the smaller symptoms-aploecia
Use laboratory markers to see if active-complement (if low-maybe active), ESR-goes up, CRP -normal
Anti-dsDNA
other ABS and CRP are poor indicators
How is SLE divided into 3 categories?
need to classify at each visit
mild-jont/skin involvment
Moderate-other organs-pleuritis, pericarditis, nephritis
Severe-Vital organs-severe nepthratis, CNS, pulmonary, Cardiac, lots of BM involvments
What are the 3 treatments used for MILD SLE?
for mild-paracetamol +/- NSAIDs –monitor renal function
Hydroxycloroquine-for mild activity
if rash not on face-topical corticosteroids
What are the 3 treatments used for Moderate/severe SLE?
If failure of NSAIDs or organs going bad
use a lot of corticosteroids-high dose steroids to stop the disease then low dose to keep it down
Azathioprine-effective steroid sparing agent-pregnancy safe–but need FBC regulation
Cyclophosmaide-more for severe-supress BM, infertility-IV intake –but cant be scared because needed
What are some novel treatments of severe SLE?
Mycophenolate mofetil-inhbitory of purine synthesis-widely used now for severe case as lymphocytes depend of purine
Rituximab-antiCD2- depletes B cells
Belimumab-targets Blys-decrease activity of B cells
=> try and switch off immune complex
