2: SLE Flashcards
(105 cards)
1
Q
What is SLE
A
Multi-system autoimmune disorder
2
Q
In which gender is SLE more common
A
Females (10:1)
3
Q
What is the peak incidence of SLE
A
20-40y
4
Q
What is the typical demographic for SLE
A
Middle age females
5
Q
What ethnicity is SLE more common in
A
Asians
African Americans
6
Q
What is the aetiology of SLE
A
Genetic predisposition with genes:
HLA DR1
HLA DR3
7
Q
What are two ‘groups’ of risk factors for SLE
A
High oestrogen
Enviromental
8
Q
What are 3 causes of high oestrogen that predispose to SLE
A
COCP
HRT
Endometriosis
9
Q
What are 4 environmental causes that predispose to SLE
A
Smoking
Epstein Barr Virus
Medications
UV
10
Q
What infection predisposes to SLE
A
Epstein Barr Virus
11
Q
What two medications predispose to SLE
A
- Procainamide
- Hydralazine
12
Q
How is the progression of SLE characterised
A
Remission and relapse
13
Q
What % if people with SLE present with skin symptoms
A
70%
14
Q
What are 5 cutaneous symptoms of SLE
A
- Malar rash
- Discoid rash
- Photosensitivity
- Oral ulcers
- Non-scarring alopecia
15
Q
What is malar rash
A
Erythematous rash with sparing of the naso-labial folds
16
Q
What is discoid rash
A
Erythematous patches with adherent keratotic scarring
17
Q
What % of people with SLE have joint symptoms
A
80
18
Q
What are 3 systemic symptoms of SLE
A
Lethargy
Weight loss
Low-grade fever
19
Q
What are the main symptoms of SLE
A
- Arthalgia (90%)
- Lethargy (80%)
- Cutaneous (70%)
20
Q
What are the 2 MSK symptoms of SLE
A
Myalgia
Lymphadenopathy
21
Q
What are 2 serositis symptoms of SLE
A
Pericarditis
Pleuritis
22
Q
What is the kidney symptom of SLE
A
Lupus nephritis
23
Q
What are 3 GI symptoms of SLE
A
Oeseophagitis
Pancreatitis
Hepatitis
24
Q
What are 3 vascular symptoms of SLE
A
Reynaud’s
Vasculitis
VTE
25
What are 5 neurological symptoms of SLE
```
Seizures
Psychosis
Personality change
Aseptic meningitis
Polyneuropathy
Myasthenia Gravis
```
26
What are 3 haematological symptoms of SLE
Haemolytic anaemia
Thrombocytopenia
Leucopenia
27
Describe the criteria for SLE diagnose
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
28
What are the 11 clinical criteria for lupus nephritis
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
29
What are the 6 lab criteria of lupus nephritis
1. ANA
2. anti-dsDNA
3. anti-smith
4. anti-phospholipid
5. low complement
6. positive coombs test
30
What is a good mnemonic to remember features of SLE
SOAP BRAIN MD
31
What are the symptoms/features of SLE
```
Serositis
Oral ulcers
Arthritis
Photosensitivity
Blood disorders
Renal involvement
Anti-nuclear antibodies
Immunological phenomenon
Neurological disorders
Malar rash
Discoid rash
```
32
What are 3 sub-types of lupus
1. Discoid lupus
2. Sub-acute cutaneous lupus
3. Drug-induced lupus
33
What is discoid lupus erythematosus
Triggered by UV - it is erythematous scaly plaques
34
What % of lupus is discoid lupus erythematosus
50-80%
35
What triggers discoid lupus
UV
36
What regions does discoid lupus affect
head, neck and face
37
How will discoid lupus present
erythematous scaly plaques that leave scar tissue
38
What is the risk of developing SLE with discoid lupus
10-15%
39
What % of lupus is subacute cutaneous lupus
10%
40
Which regions does subacute cutaneous lupus affect
Neck, shoulder and forearms but spares the face
41
Which region does subacute cutaneous lupus not affect
Face
42
How does subacute cutaneous lupus present
small erythematous rash
43
What is drug-induced lupus erythematosus
lupus like symptoms that resolve in days to months following cessation
44
What drugs trigger drug-induced lupus
Ms. Pip:
```
Methyldopa
Sulfa-containing drugs
Procainamide
Isoniazid
Phenytoin
```
45
Which 5 drugs may cause drug-induced lupus
- procainmide
- hydralazine
- isoniazid
- phenytoin
- methyldopa
46
Which age group is drug induced lupus more common
older individuals
47
How does drug induced lupus present
- fever
- serositis
- poly arthritis
- myalgia
- rash
48
What is present in drug-induced lupus on investigation
anti-histone antibodies
49
What type of hypersensitivity reaction is SLE
Type 3 hypersensitivity reaction
50
Explain the pathophysiology of SLE
- 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
What investigations are used for SLE
1. Anti-nuclear antibodies
2. Anti dsDNA antibodies
3. Anti smith antibodies
4. Rheumatoid factor
5. SS-A
6. SS-B
52
What is the most specific antibody for SLE
Anti-smith
53
What is the most sensitive antibody for SLE
Anti-dsDNA (70%)
54
What 3 tests are used to monitor disease activity in SLE
ESR
Complement
Anti dsDNA
55
How will CRP present in SLE and therefore what does a raised CRP indicate
CRP will be normal - therefore a raised CRP indicates underlying infection.
56
How will complement present in disease activity and why
Complement will be low - due to use in reaction
57
What general treatment is used for SLE
- Avoid sun exposure
- High Factor Sun cream
- Hydroxychloroquine
- Topical steroids
58
what has hydroxychloroquine be shown to do
Reduce disease activity and improve survival
59
when are topical steroids recommended
Skin flares
60
what is used as maintenance therapy for SLE
NSAIDs
| Hydroxychloroquine
61
if individuals have high antibody positive disease what else may be used
Belimumab
62
what defines a mild flare
No organ damage
63
what is used to treat a mild flare of SLE
Low dose oral steroids
| Hydroxychloroquine
64
what defines a moderate flare
Evidence of organ involvement
65
what is used to manage a moderate flare of SLE
DMARDs
| Mycophenolate
66
what defines a severe flare of SLE
Organ threatening: haemolytic anaemia, nephritis, pericarditis, CNS disease
67
how is a severe flare of SLE treated
IV steroids
Mycophenolate
Rituximab
Cyclophosphamide
68
What is survival of SLE at 15y
80%
69
What are two risks of SLE
increase risk of osteoporosis and CVD
70
What are two complications of SLE
Lupus nephritis
| Antiphospholipid syndrome
71
What is lupus nephritis
Interstitial nephritis caused by deposition of complement in SLE
72
What % of individuals with SLE develop lupus nephritis
50%
73
How may lupus nephritis present
nephritic or nephrotic syndrome
74
What is WHO class I lupus nephritis
Normal kidney
75
What is WHO class II lupus nephritis
Mesangial Glomerulonephritis
76
What is WHO class 3 lupus nephritis
Focal (segmental glomerulonephritis)
77
What is WHO class 4 lupus nephritis
Diffuse proliferative glomerulonephritis
78
What is WHO class 5 lupus nephritis
Diffuse membraneous glomerulonephritis
79
What is WHO class 6 lupus nephritis
Sclerosing glomerulonephritis
80
What is the most common and severe form of lupus nephritis
Who Class 4: diffuse proliferative glomerulonephritis
81
What investigations may be done in lupus nephritis
1. Urinalysis
2. A:Cr ratio
3. Kidney biopsy
82
When is a kidney biopsy considered
A:Cr >30
P:Cr >50
83
How will diffuse proliferative glomerulonephritis appear on kidney biopsy
- Endothelial and mesangial proliferation (wire-loop appearance)
- Thickened capillary wall
- Subendothelial immune deposits
84
What is the management of lupus nephritis
- Treat HTN
- Corticosteroids
- Immunosuppressants (azathioprine, cyclophosphamide)
85
How is lupus nephritis prevented
Patients with SLE should have regular urinalysis at check-ups to look for proteinuria
86
What is antiphospholipid syndrome
Autoimmune disease that increases risk of thrombosis due to pro-coagulators antibodies
87
What causes primary antiphospholipid syndrome
Associated with HLA DR7
88
What are 5 causes of secondary antiphospholipid syndrome
- SLE
- RA
- HIV
- Hep A,B and C
- Infection (TB, syphillis, lymes disease)
89
What % of individuals with SLE have antiphospholipid syndrome
20-30%
90
What is a mnemonic to remember symptoms of anti-phospholipid syndrome. What are the symptoms of antiphospholipid syndrome
CLOTS
```
Coagulation defect (arterial and venous)
Livedo reticularis
Obstetric complications
Thrombocytopenia
Splinter haemorrhages
```
91
What are 4 arterial thrombotic complications of antiphospholipid syndrome
TIA
Stroke
End-organ ischaemia (MI)
Occlusion of distal extremity
92
What are 4 venous thrombotic complications of antiphospholipid syndrome
PE
DVT
Livedo reticularis
Chronic venous ulcers
93
What are two obstetric complications of antiphospholipid syndrome
Recurrent miscarriage
| Premature birth
94
Why do obstetric complications of antiphospholipid syndrome occur
Thrombosis of the placenta vessels causing infarction
95
Explain the pathophysiology of antiphospholipid syndrome
- 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
What prompts investigation for antiphospholipid syndrome
- history of recurrent miscarriages
| - history of arterial/venous thrombosis
97
What investigation is performed in antiphospholipid syndrome
antiphospholipid antibody panel
98
What are the antiphospholipid antibodies
- lupus anticoagulant
| - anticardiolipin antibody
99
How will lupus anticoagulant appear on coagulation screen
raised aPTT (partial thromboplastin time)
100
if patients have anti-cardiolipin antibodies what will they test falsely positive for and why
syphillis. As the test for syphillis uses cardiolipin
101
What is the acute management of antiphospholipid syndrome
LMWH
| IV Unfractioned heparin
102
If severe, what is offered for antiphospholipid syndrome
High-dose corticosteroids or plasmapheresis
103
what prophylaxis is offered for low risk antiphospholipid syndrome
Aspirin
104
what prophylaxis is offered for high risk antiphospholipid syndrome with no desire to be pregnant
Warfarin
105
what prophylaxis is offered for high risk antiphospholipid syndrome with desire to be pregnant
LMWH and aspirin
