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BRS YR2 > Lupus > Flashcards

Lupus Flashcards

1
Q

What are the autoimmune connective tissue disorders?

A
  • systemic lupus erythmatosus
  • autoimmue inflammatory muscle disease
  • systemic sclerosis (scleroderma)
  • Sjorgen’s syndrome
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2
Q

When are overlap syndromes of autoimmune connective tissue disorders most likely to occur?

A

In childhood

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3
Q

What is SLE?

A
  • autoimmune disorder affecting both innate and adaptive immune systems
  • involves autoantibodies to nuclear components of cells
  • immune complexes and other mechanisms cause chronic tissue inflammation
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4
Q

Where is inflammation most likely to manifest in SLE?

A

Joints, skin and kidney

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5
Q

How is arthirits and artharlagia different in SLE than in RA?

A

Non-erosive

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6
Q

What is a common feature of autoimmune connective tissue disorders?

A

Raynaud’s phenomenon

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7
Q

What is Raynaud’s phenomenon?

A
  • intermittent vasospasm of digits
  • usually triggered by cold exposure
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8
Q

What is the triphasic colour change which happens in Raynaud’s phenomenon?

A
  1. White - vasospasm causes digits to blanch
  2. Blue - cyanosis due to static venous blood becoming deoxygenated
  3. Red - reactive hyperaemia
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9
Q

What are the features of severe Raynaud’s?

A

Tissue ischaemia, ulcers and necrosis

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10
Q

What are the common clinical features of SLE?

A
  • malar (butterfly) rash
  • photosensitive rash
  • mouth ulcers
  • hair loss
  • Raynaud’s
  • arthralgia
  • serositis
  • renal disease
  • cerebral disease
  • myocarditis
  • autoimmune thrombocytopenia
  • haemolytic anaemia
  • lymphopenia
  • lymhodenopathy
  • fever with no infection
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11
Q

What is the hallmark feature of SLE?

A
  • Anti-nuclear antibodies (ANA)
  • found in all SLE patients
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12
Q

Why can ANA be used to rule out lupus but not diagnose it?

A

ANA is not specific for lupus and can be seen in other diseases, infections and even in healthy people

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13
Q

What will the clinical laboratory report if ANA is positive?

A
  • strength (maxiumum dilution at which antibody can still be detected)
  • pattern of staining (suggests which autoantigen the antibodies are reacting to)
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14
Q

Which further tests indicate that a patient is positive for SLE?

A
  • anti-ds-DNA antibodies
  • anti-Ro
  • anti-La
  • anti-Smith
  • anti-RNP
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15
Q

Which other antibodies do some patients with SLE present with?

A

Antiphospholipid antibodies (APL)

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16
Q

What is the presence of APL associated with?

A
  • arterial thrombosis (stroke)
  • venous thrombosis (DVT)
  • pregnancy loss
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17
Q

What is anti-phospholipid antibody syndrome?

A
  • presistent presence of APL + clinical event
  • cann occur in absence of SLE (primary APL syndrome)
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18
Q

Which antibodies are specific for SLE?

A
  • anti-double stranded DNA antibodies
  • anti-Smith antibodies
19
Q

Which antibody specific to SLE has serum levels which indicate the severity of the diesease?

A

Anti-ds DNA antibodies

20
Q

What is the immunopathogenesis of SLE?

A
  1. Interference with innate immunity
    - overactivity of type 1 interferon pathway
    - complement pathway abnormalities
  2. Interference with adaptive immunity
    - autoreactive B and T cells
21
Q

How does the immune system generate a repsone to nuclear antigens?

A

Waste disposal hypothesis

22
Q

What is the waste disposal hypothesis?

A
  • apoptosis translocates nuclear antigens to the membrane surface
  • impaired clearance of apoptotic cells means more nuclear antibodies are presented to immune cells
  • causes B cell autoimmunity
  • leads to tissue damage by antibodies activating complement and Fc receptor engagement
23
Q

Why are the manifestations of SLE so varied?

A

Autoimmunity is systemic so almost any organ can be involved

24
Q

Which investigations are done for SLE?

A
  • ESR (high)
  • CRP (usually normal)
  • FBC (anaemia, lymphopenia, thrombocytopenia)
  • urinalysis to measure urine protein
  • creatinine
  • U&E
  • albumin
  • kidney biopsy is persistent proteinuria
  • ANA
  • anti-ds DNA
  • compelement (low C3 and C4)
  • ALP
25
Q

Delete

A
26
Q

What should be measured in patients who are on medication for SLE and why?

A
  • FBC and LFT
  • may have adverse reactions to the medication
27
Q

How do you measure disease activity in SLE?

A
  • clinical symptoms and signs
  • low C3 and C4 levels
  • high anti-ds DNA antibodies
28
Q

What are the general principals of managing SLE?

A
  • aim for remission or low disease activity and preventing flare ups
  • balance controlling disease with avoiding iatrogenic harm from steroids
  • choose treatment depending on the disease severity and organ manifestations
29
Q

What are the steroid side effects seen in SLE?

A
  • infection
  • osteoporosis
  • avascular necrosis (necrosis of bone, often affecting hips) especially in patients with ALP
30
Q

Which medications are recommended for all patients with SLE?

A

Hydroxychloroquine

31
Q

When can steroids be used for SLE?

A

In acute flare ups

32
Q

What medications are used in more serious SLE?

A

Immunomodulatory agents (mycophenolate, methotrexate, azithioprine)

33
Q

Which medications are used for kindey disease in SLE?

A

Mycophenolate +/- rituximab

34
Q

Which medications are used in persistently active SLE?

A
  • B cell targeted therapies
  • rituximab (depeltes B cells, anti-CD20)
  • belimumab (anti-BAFF, cytokine needed for B cell survival)
35
Q

How do you treat life-threatening disease e.g myocarditis?

A
  • IV steroids
  • IV cyclophosphamide
  • +/- rituximab
36
Q

What should patients with SLE and antiphospholipid antibody syndrome be given?

A

Warfarin for anticoagulation

37
Q

What are the emerging therapies for SLE?

A

Interferon receptor blockade e.g anifrolumab

38
Q

Who does SLE usually affect?

A

Women during their reproductive years

39
Q

What needs to be taken into consideration when treating patients with SLE who are of child bearing age?

A
  • risk of disease and drugs to both mother and fetus
  • better outcomes with pre-pregnancy planning and getting SLE into remission first
40
Q

What is the effect of ALP on pregnancy?

A
  • associated with miscarriage
  • can reduce risk with aspirin or heparin
41
Q

How does pregnancy affect renal function?

A

Increases haemodynamic demands which worsens renal function

42
Q

What can Ro antibodies cause in pregnancy?

A

Fetal heartblock

43
Q

Which medications for SLE are teratogenic?

A
  • MMF
  • cyclophosphamide
  • methotrexate
  • warfarin
44
Q

Which medication for SLE are safe for pregnancy?

A
  • hydroxychloroquine
  • azathioprine
  • low molecular weight heparin

BRS YR2 (67 decks)

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