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LCRS Musculoskeletal > Systemic Lupus Erythematosus > Flashcards

Systemic Lupus Erythematosus Flashcards

1
Q

what is SLE?

A

rare disease and is part of a family of overlapping chronic autoimmune diseases

shows type 3 hypersensitivity (immune complexes) and type 2 (antibodies against RBC, WBC, platelets leading to pancytopenia)

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2
Q

examples of autoimmune diseases in the family that SLE is part of?

A

RA
Sjogren’s syndrome
dermatomyositis, polymyositis
systemic sclerosis.

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3
Q

who does SLE affect mostly?

A

female (9:1)
15-40 years
Afro-carribean, Far East Asian, Asian

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4
Q

what does SLE primarily affect?

A

joints and skin and kidenys

– also affects lungs and has unique haematology

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5
Q

what are the genetic associations of SLE?

A
  • Multiple genes: Fc receptors, IRF5, CTLA4, MHC class 2 HLA genes (DR3)
  • Complement deficiency: C1q and C3.
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6
Q

what is the (general) presentation of SLE?

A 
Malaise
fatigue
fever
weight loss
lymphadenopathy (lymphomas excluded).
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7
Q

what are specific features of SLE?

A

Butterfly rash
alopecia
arthralgia
Raynaud’s phenomenon.

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8
Q

what are some severe presentations of SLE?

A

o Inflammation of the kidney, CNS, heart & lungs.
o Accelerated atherosclerosis.
o Vasculitis.

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9
Q

what is the cause of autoantigens production in SLE?

A

there is impaired clearance associated with inflammation of apoptotic bodies. They linger in the body and expose nuclear antigens which then generate autoantigens

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10
Q

what is the main driver of SLE?

A

immune complexes

these bind to endothelial cell to cause local inflammation and activate enzymes after complement activation to cause cell apoptosis

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11
Q

what causes the formation of immune complexes?

A
  • dendritic cells take up autoantigens
  • overactive B cells exposed to auto antigens (and activated)
  • plasma cells form IgG autoantibodies that form immune complexes (ab-ag)
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12
Q

what is the effect of immune complexes?

A

deposit in tissues (kidneys/skin mainly) and then activates complement in the tissues

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13
Q

summary of auto-antibody formation summary

A
  1. Abnormal clearance of apoptotic bodies.
  2. Dendrites take up autoantigens –>b-cell activation.
  3. B-cell Ig-class switch and affinity maturation.
  4. IgG-autoantibodies.
  5. Immune complexes.
  6. Complement activation via classical pathway.
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14
Q

how can SLE be tested for in the lab?

A

check for

  • antinuclear antibodies
  • anti dsDNA antibodies

if ANAs present, they will bind to the cell’s nuclear antigens
fluorescently labelled AB is added that binds to ANAs and you observe the pattern of attachment

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15
Q

what other tests can used?

A 
anti-dsDNA and Sm (more specific) 
anti-Ro and/or La
increased complement consumption 
normocytic anaemia 
haematology and renal
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16
Q

why might complement consumption be measured to check activity of SLE?

A

patients with active lupus will have more complement bound to immune complexes and so the blood complement will be LOW in active lupus

17
Q

why may SLE patients present with autoimmune haemolytic anaemia?

A

ABs bind to RBC surfaces and they are extravascularly removed in the spleen.

18
Q

how is the severity of SLE assessed?

A
  1. Identify pattern of organ involvement.
  2. Monitor function of affected organs:
    a. Renal
    – BP, U&E, urine sediment GFR.
    b. Lungs/CVS
    – lung function tests, echocardiography.
  3. Identify the patterns of autoantibodies expressed.
    a. Anti-dsDNA, anti-Sm
    – high levels of these ABs can predict renal disease.
    b. Anti-cardiolipin antibodies.
19
Q

what will happen to the erythrocyte sedimentation rate in disease that is become severe?

A

increase

20
Q

what is importance of picking up symptoms early?

A

so less damage to organs systems occur

21
Q

in what stage of disease severity is there organ involvement?

A

moderate to severe

mild has no organ involvement

22
Q

what is the treatment for mild SLE?

A

 Paracetamol ± NSAIDs.
 Hydroxychloroquine (for the rash)
 Topical corticosteroids.

23
Q

what are the severities of SLE and what is involved in each?

A

mild–> joint +/- skin

moderate–> inflammation of organs, pleuritis, pericarditis

severe–> severe inflammation of vital organs

24
Q

what is the treatment for moderate SLE?

A

 Hydroxychloroquine and NSAIDs will stop working in moderate disease.

Switch from topic to oral glucocorticoids
High dose and titrate down

25
Q

why is a high dose given for moderate SLE? what can be done instead?

A

o High dose needed to supress disease activity but also is very toxic so can give the corticosteroids with steroid-sparing agents to lower the dose required.

26
Q

what are the 2 drugs given in severe SLE?

A

 Azathioprine – a steroid-sparing drug
Cyclophosphamide – only used in SEVERE organ involvement

  • mycophenolate mofetil
  • rituximab
27
Q

what is the side effect of Azathioprine?

A

risk of neutropenia and bone marrow suppression so requires blood monitoring.

28
Q

what is the side effect of Cyclophosphamide?

A

infertility

29
Q

why is azathioprine a favourable option?

A

safe during pregnancy as it is steroid sparing

30
Q

what is the prognosis of SLE?

A

15 year survival
o No nephritis = 85%.
o Nephritis = 60%.

31
Q

when is prognosis worse?

A

black, male, low socio-economic background/status.

32
Q

what are the two peaks in SLE mortality?

A

o Early death – renal failure, CNS disease, infection.

o Late death – MI, stroke.

33
Q

what can be seen on a blood film in SLE?

A

 Shistocytes.
 Spherocytes.
 Tear drop cells.
 Few – leukocytes and platelets

34
Q

what can be seen in renal biopsy in SLE?

A

 Hyper-cellular – more cells than normal.
 Mesangial proliferation.
 Crescent development – inflammatory cells that have migrated into the glomerulus.
 Rapidly proliferating glomerulonephritis.

35
Q

which antibody in SLE is specific to the disease?

A

anti dsDNA antibodies

36
Q

which antibody is less specific to the disease?

A

anti-nuclear antibodies

37
Q

which HLA defect leads to SLE?

A

HLA DR3

LCRS Musculoskeletal (11 decks)

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