Lupus Flashcards

1
Q

Systemic lupus erythematosus?

A

Autoimmune disease involving innate and adaptive immune system disturbance
Has autoantibodies - antibodies to nuclear components
Antibody-antigen (immune complexes) -> chronic tissue inflammation (particularly joints, skin and kidney)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
2
Q

SLE rash example?

A

Malar rash

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
3
Q

Autoimmune connective tissue disorder? General points

A
  1. Arthralgia and arthritis typically non-erosive (unlike RA)
  2. Serum autoantibodies characteristic
  3. Raynaud’s phenomenon common
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
4
Q

Raynaud’s phenomenon?

A

Intermittent vasospasm of digits, usually triggered by cold exposure
Triphasic colour changes

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
5
Q

Typical Raynaud’s colours?

A

White - vasospasm leading to blanching of digit
Blue - cyanosis as static venous blood deoxygenates
Red - reactive hyperaemia

Severe Raynaud’s - tissue ischaemia, ulcers and necrosis

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
6
Q

SLE epidemiology?

A

15-45
F:M - 9:1
Africa > Asian > white European

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
7
Q

Clinical features? Skin

A

Malar rash - erythema that spares nasolabial fold
Photosensitive rash
Mouth ulcers
Hair loss

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
8
Q

Clinical features? Vascular

A

Raynaud’s

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
9
Q

Clinical features? MSK

A

Arthralgia and arthritis (non-erosive)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
10
Q

Clinical features? Internal organs

A

Serositis (pericarditis, pleuritis, rarely peritonitis)
Renal disease - glomerulonephritis
Cerebral disease
Myocarditis

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
11
Q

Clinical features? Haematological

A

Autoimmune thrombocytopenia
Haemolytic anaemia
Lymphopenia

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
12
Q

Clinical features? Other

A

Lymphadenopathy
Fever in absence of infection

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
13
Q

Anti-nuclear antibodies?

A

Hallmark of SLE, in all patients
Examples of other autoantibodies:
Anti-ds-DNA antibody
Anti-Ro
Anti-La
Anti-Smith
Anti-RNP

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
14
Q

Anti-phospholipid antibodies?

A

Directed to phospholipids on cell membrane
Associated with increased risk of thrombosis and pregnancy loss

persistent APL + clinical event = anti-phospholipid antibody syndrome

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
15
Q

Main autoantibodies in SLE?

A

Antinuclear antibodies - in all SLE, non-specific

Anti-double stranded DNA antibodies - specific for SLE, serum level DO correlate with disease activity

Anti-phospholipid antibodies - can occur in absence of SLE (primary anti-phospholipid antibody syndrome)

Anti-Sm antibodies - specific for SLE, serum levels DON’T correlate with disease activity

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
16
Q

SLE immunopathogenesis?

A

Overactivity of type 1 interferon pathway
Complement pathway abnormalities
Overactive B and T cells

17
Q

Waste disposal hypothesis

A
  1. Apoptosis -> translocation of nuclear antigens to membrane surface
  2. Impaired clearance of apoptotic cells = enhanced presentation of nuclear antigens to immune cells
  3. B cell autoimmunity
  4. Tissue damage by antibody effector mechanisms
18
Q

SLE investigations? Inflammation

A

High ESR, usually normal CRP

19
Q

SLE investigations? Renal

A

Measure urine protein, creatinine, albumin
Kidney biopsy if persistent proteinuria

20
Q

SLE investigations? Immunological

A

ANA
Anti-ds-DNA antibodies
Complement consumption (low C3 + C4)
Antiphospholipid antibodies

21
Q

SLE complement and antibodies in unwell patient?

A

Low C3 + C4
High anti-dsDNA antibodies

22
Q

SLE management aims?

A
  1. Treat SLE and prevent flares
  2. Prevent ionotropic harm of steroids (infection, osteoporosis, avascular necrosis)
  3. Specific treatment
23
Q

SLE management? Pharmacology

A

Hydroxychloroquine recommended for all lupus patients
steroids for flare
Serious disease - immunomodulatory (mycophenolate, methotrexate, Azathioprine)

24
Q

SLE renal disease treatment?

A

Mycophenolate +/- rituximab

25
Q

Severe or life threatening SLE treatment?

A

IV steroids + IV cyclophosphamide

26
Q

SLE + antiphospholipid antibody syndrome treatment?

A

Anticoag (warfarin)

27
Q

SLE and pregnancy considerations?

A
  1. Antiphospholipid antibodies associated with miscarriage (give aspirin or heparin)
  2. Pregnancy increased Haemodynamic demands so will worsen renal dysfunction
  3. Ro antibodies - can cause fetal heartblock
  4. Drugs
28
Q

Drug considerations? SLE and pregnancy, unsafe and safe

A

Unsafe - MMF, cyclophosphamide, methotrexate and warfarin are teratogenic

Safe - Hydroxychloroquine, Azathioprine and low weight molecular heparin

29
Q

Ionotopic steroid side effects?

A

Infection, osteoporosis, avascular necrosis