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Rheumatology > 2: SLE > Flashcards

2: SLE Flashcards

1
Q

What is SLE

A

Multi-system autoimmune disorder

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2
Q

In which gender is SLE more common

A

Females (10:1)

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3
Q

What is the peak incidence of SLE

A

20-40y

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4
Q

What is the typical demographic for SLE

A

Middle age females

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5
Q

What ethnicity is SLE more common in

A

Asians

African Americans

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6
Q

What is the aetiology of SLE

A

Genetic predisposition with genes:
HLA DR1
HLA DR3

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7
Q

What are two ‘groups’ of risk factors for SLE

A

High oestrogen

Enviromental

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8
Q

What are 3 causes of high oestrogen that predispose to SLE

A

COCP
HRT
Endometriosis

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9
Q

What are 4 environmental causes that predispose to SLE

A

Smoking
Epstein Barr Virus
Medications
UV

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10
Q

What infection predisposes to SLE

A

Epstein Barr Virus

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11
Q

What two medications predispose to SLE

A
  • Procainamide

- Hydralazine

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12
Q

How is the progression of SLE characterised

A

Remission and relapse

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13
Q

What % if people with SLE present with skin symptoms

A

70%

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14
Q

What are 5 cutaneous symptoms of SLE

A
  1. Malar rash
  2. Discoid rash
  3. Photosensitivity
  4. Oral ulcers
  5. Non-scarring alopecia
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15
Q

What is malar rash

A

Erythematous rash with sparing of the naso-labial folds

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16
Q

What is discoid rash

A

Erythematous patches with adherent keratotic scarring

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17
Q

What % of people with SLE have joint symptoms

A

80

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18
Q

What are 3 systemic symptoms of SLE

A

Lethargy
Weight loss
Low-grade fever

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19
Q

What are the main symptoms of SLE

A
  • Arthalgia (90%)
  • Lethargy (80%)
  • Cutaneous (70%)
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20
Q

What are the 2 MSK symptoms of SLE

A

Myalgia

Lymphadenopathy

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21
Q

What are 2 serositis symptoms of SLE

A

Pericarditis

Pleuritis

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22
Q

What is the kidney symptom of SLE

A

Lupus nephritis

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23
Q

What are 3 GI symptoms of SLE

A

Oeseophagitis
Pancreatitis
Hepatitis

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24
Q

What are 3 vascular symptoms of SLE

A

Reynaud’s
Vasculitis
VTE

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25
Q

What are 5 neurological symptoms of SLE

A 
Seizures
Psychosis 
Personality change 
Aseptic meningitis 
Polyneuropathy 
Myasthenia Gravis
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26
Q

What are 3 haematological symptoms of SLE

A

Haemolytic anaemia
Thrombocytopenia
Leucopenia

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27
Q

Describe the criteria for SLE diagnose

A

Requires four or more of criteria list (at least one clinical and one lab), or biopsy proven lupus nephritis with positive ANA or anti-DNA

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28
Q

What are the 11 clinical criteria for lupus nephritis

A
  1. Acute cutaneous lupus
  2. Chronic cutaneous lupus
  3. Non-scarring alopecia
  4. Oral ulcers
  5. Synovitis
  6. Serositis
  7. Proteinuria or red cell casts
  8. Neurological features
  9. Haemolytic anaemia
  10. Leucopenia
  11. Thrombocytopenia
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29
Q

What are the 6 lab criteria of lupus nephritis

A
  1. ANA
  2. anti-dsDNA
  3. anti-smith
  4. anti-phospholipid
  5. low complement
  6. positive coombs test
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30
Q

What is a good mnemonic to remember features of SLE

A

SOAP BRAIN MD

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31
Q

What are the symptoms/features of SLE

A 
Serositis 
Oral ulcers 
Arthritis 
Photosensitivity 
Blood disorders 
Renal involvement 
Anti-nuclear antibodies 
Immunological phenomenon
Neurological disorders
Malar rash 
Discoid rash
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32
Q

What are 3 sub-types of lupus

A
  1. Discoid lupus
  2. Sub-acute cutaneous lupus
  3. Drug-induced lupus
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33
Q

What is discoid lupus erythematosus

A

Triggered by UV - it is erythematous scaly plaques

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34
Q

What % of lupus is discoid lupus erythematosus

A

50-80%

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35
Q

What triggers discoid lupus

A

UV

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36
Q

What regions does discoid lupus affect

A

head, neck and face

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37
Q

How will discoid lupus present

A

erythematous scaly plaques that leave scar tissue

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38
Q

What is the risk of developing SLE with discoid lupus

A

10-15%

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39
Q

What % of lupus is subacute cutaneous lupus

A

10%

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40
Q

Which regions does subacute cutaneous lupus affect

A

Neck, shoulder and forearms but spares the face

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41
Q

Which region does subacute cutaneous lupus not affect

A

Face

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42
Q

How does subacute cutaneous lupus present

A

small erythematous rash

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43
Q

What is drug-induced lupus erythematosus

A

lupus like symptoms that resolve in days to months following cessation

44
Q

What drugs trigger drug-induced lupus

A

Ms. Pip:

Methyldopa
Sulfa-containing drugs 
Procainamide
Isoniazid
Phenytoin
45
Q

Which 5 drugs may cause drug-induced lupus

A
  • procainmide
  • hydralazine
  • isoniazid
  • phenytoin
  • methyldopa
46
Q

Which age group is drug induced lupus more common

A

older individuals

47
Q

How does drug induced lupus present

A
  • fever
  • serositis
  • poly arthritis
  • myalgia
  • rash
48
Q

What is present in drug-induced lupus on investigation

A

anti-histone antibodies

49
Q

What type of hypersensitivity reaction is SLE

A

Type 3 hypersensitivity reaction

50
Q

Explain the pathophysiology of SLE

A
  • Individuals exposed to UV causes DNA damage and apoptosis. This produces apoptotic bodies and expose remaining body to the nucleus
  • Susceptibility genes (HLADR2 + HLADR3) are more likely to recognise these antigens as foreign and generate an immune response
  • Also reduce clearance of apoptotic bodies increasing nuclear antigens in circulation
  • Anti-nuclear antibodies bind antigens to form complexes
  • Complexes enter different tissues and adhere to walls in vessels
  • When adhered they produce an inflammatory reaction that activates complement system causing damage
  • This disrupts osmotic channels in cell membranes causing rupture
51
Q

What investigations are used for SLE

A
  1. Anti-nuclear antibodies
  2. Anti dsDNA antibodies
  3. Anti smith antibodies
  4. Rheumatoid factor
  5. SS-A
  6. SS-B
52
Q

What is the most specific antibody for SLE

A

Anti-smith

53
Q

What is the most sensitive antibody for SLE

A

Anti-dsDNA (70%)

54
Q

What 3 tests are used to monitor disease activity in SLE

A

ESR
Complement
Anti dsDNA

55
Q

How will CRP present in SLE and therefore what does a raised CRP indicate

A

CRP will be normal - therefore a raised CRP indicates underlying infection.

56
Q

How will complement present in disease activity and why

A

Complement will be low - due to use in reaction

57
Q

What general treatment is used for SLE

A
  • Avoid sun exposure
  • High Factor Sun cream
  • Hydroxychloroquine
  • Topical steroids
58
Q

what has hydroxychloroquine be shown to do

A

Reduce disease activity and improve survival

59
Q

when are topical steroids recommended

A

Skin flares

60
Q

what is used as maintenance therapy for SLE

A

NSAIDs

Hydroxychloroquine

61
Q

if individuals have high antibody positive disease what else may be used

A

Belimumab

62
Q

what defines a mild flare

A

No organ damage

63
Q

what is used to treat a mild flare of SLE

A

Low dose oral steroids

Hydroxychloroquine

64
Q

what defines a moderate flare

A

Evidence of organ involvement

65
Q

what is used to manage a moderate flare of SLE

A

DMARDs

Mycophenolate

66
Q

what defines a severe flare of SLE

A

Organ threatening: haemolytic anaemia, nephritis, pericarditis, CNS disease

67
Q

how is a severe flare of SLE treated

A

IV steroids
Mycophenolate
Rituximab
Cyclophosphamide

68
Q

What is survival of SLE at 15y

A

80%

69
Q

What are two risks of SLE

A

increase risk of osteoporosis and CVD

70
Q

What are two complications of SLE

A

Lupus nephritis

Antiphospholipid syndrome

71
Q

What is lupus nephritis

A

Interstitial nephritis caused by deposition of complement in SLE

72
Q

What % of individuals with SLE develop lupus nephritis

A

50%

73
Q

How may lupus nephritis present

A

nephritic or nephrotic syndrome

74
Q

What is WHO class I lupus nephritis

A

Normal kidney

75
Q

What is WHO class II lupus nephritis

A

Mesangial Glomerulonephritis

76
Q

What is WHO class 3 lupus nephritis

A

Focal (segmental glomerulonephritis)

77
Q

What is WHO class 4 lupus nephritis

A

Diffuse proliferative glomerulonephritis

78
Q

What is WHO class 5 lupus nephritis

A

Diffuse membraneous glomerulonephritis

79
Q

What is WHO class 6 lupus nephritis

A

Sclerosing glomerulonephritis

80
Q

What is the most common and severe form of lupus nephritis

A

Who Class 4: diffuse proliferative glomerulonephritis

81
Q

What investigations may be done in lupus nephritis

A
  1. Urinalysis
  2. A:Cr ratio
  3. Kidney biopsy
82
Q

When is a kidney biopsy considered

A

A:Cr >30
P:Cr >50

83
Q

How will diffuse proliferative glomerulonephritis appear on kidney biopsy

A
  • Endothelial and mesangial proliferation (wire-loop appearance)
  • Thickened capillary wall
  • Subendothelial immune deposits
84
Q

What is the management of lupus nephritis

A
  • Treat HTN
  • Corticosteroids
  • Immunosuppressants (azathioprine, cyclophosphamide)
85
Q

How is lupus nephritis prevented

A

Patients with SLE should have regular urinalysis at check-ups to look for proteinuria

86
Q

What is antiphospholipid syndrome

A

Autoimmune disease that increases risk of thrombosis due to pro-coagulators antibodies

87
Q

What causes primary antiphospholipid syndrome

A

Associated with HLA DR7

88
Q

What are 5 causes of secondary antiphospholipid syndrome

A
  • SLE
  • RA
  • HIV
  • Hep A,B and C
  • Infection (TB, syphillis, lymes disease)
89
Q

What % of individuals with SLE have antiphospholipid syndrome

A

20-30%

90
Q

What is a mnemonic to remember symptoms of anti-phospholipid syndrome. What are the symptoms of antiphospholipid syndrome

A

CLOTS

Coagulation defect (arterial and venous) 
Livedo reticularis
Obstetric complications 
Thrombocytopenia 
Splinter haemorrhages
91
Q

What are 4 arterial thrombotic complications of antiphospholipid syndrome

A

TIA
Stroke
End-organ ischaemia (MI)
Occlusion of distal extremity

92
Q

What are 4 venous thrombotic complications of antiphospholipid syndrome

A

PE
DVT
Livedo reticularis
Chronic venous ulcers

93
Q

What are two obstetric complications of antiphospholipid syndrome

A

Recurrent miscarriage

Premature birth

94
Q

Why do obstetric complications of antiphospholipid syndrome occur

A

Thrombosis of the placenta vessels causing infarction

95
Q

Explain the pathophysiology of antiphospholipid syndrome

A
  • Antiphospholipid antibodies form complexes with anti-coagulatory proteins (protein C, S, anti-thrombin 3)
  • Antibodies activate platelets and vascular endothelium
  • This induces a hyper-coagulable state
96
Q

What prompts investigation for antiphospholipid syndrome

A
  • history of recurrent miscarriages

- history of arterial/venous thrombosis

97
Q

What investigation is performed in antiphospholipid syndrome

A

antiphospholipid antibody panel

98
Q

What are the antiphospholipid antibodies

A
  • lupus anticoagulant

- anticardiolipin antibody

99
Q

How will lupus anticoagulant appear on coagulation screen

A

raised aPTT (partial thromboplastin time)

100
Q

if patients have anti-cardiolipin antibodies what will they test falsely positive for and why

A

syphillis. As the test for syphillis uses cardiolipin

101
Q

What is the acute management of antiphospholipid syndrome

A

LMWH

IV Unfractioned heparin

102
Q

If severe, what is offered for antiphospholipid syndrome

A

High-dose corticosteroids or plasmapheresis

103
Q

what prophylaxis is offered for low risk antiphospholipid syndrome

A

Aspirin

104
Q

what prophylaxis is offered for high risk antiphospholipid syndrome with no desire to be pregnant

A

Warfarin

105
Q

what prophylaxis is offered for high risk antiphospholipid syndrome with desire to be pregnant

A

LMWH and aspirin

Rheumatology (10 decks)

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