SLE

Question 1

Which six diseases contribute to the spectrum of autoimmune connective diseases?

Answer 1

Rheumatoid arthritis 
SLE
Dermatomyositis 
Polymyositis
System sclerosis 
Sjogren's syndrome

Question 2

What is Sjogren’s syndrome?

Answer 2

Autoimmune attack against moisture production aka dry mouth and dry eyes

Question 3

Describe the typical presentation of SLE

Answer 3

Malaise
Fatigue
Fever
Weight Loss
Lymphadenopathy

Question 4

What are the specific features of SLE presentation?

Answer 4

Butterfly rash (malar rash)
Alopecia
Arthralgia
Raynaud’s phenomenon

Question 5

What other organs may be affected in SLE?

Answer 5

Kidney
CNS
Heart
Lungs

Question 6

Describe the cardiovascular complications of SLE

Answer 6

Accelerated atherosclerosis
Vasculitis
MI

Question 7

How many of the 11 criteria does someone have to meet to have SLE?

Answer 7

4 out of 11

Question 8

What are the criteria for an SLE diagnosis?

Answer 8

Malar rash
Discoid rash 
Photosensitivity
Oral ulcers
Arthritis 
Serositis
Renal disorder 
Neurological disorder
Haematological disorder 
Immunologic disorder  e.g. anti-dsDNA Abs
Antinuclear antibody in raised titre

Question 9

What renal disorders may be associated with SLE?

Answer 9

Proteinuria

Question 10

What neurological disorders may be associated with SLE?

Answer 10

Seizures

Psychosis

Question 11

What are the two types of rash associated with SLE?

Answer 11

Discoid rash

Malar rash

Question 12

What is serositis?

Answer 12

Inflammation of a serous membrane

Question 13

Give two examples of serositis associated with SLE

Answer 13

Pleuritis

Pericarditis

Question 14

Briefly describe the process of autoantibody production in SLE

Answer 14

Abnormal clearance of apoptotic cell material
Presentation of self antigen by dendritic cells to B cells
Activation of B cells, Ig class switching and affinity mutation
IgG autoantibody production
Immune complexes produced

Question 15

Which cell and antibody is mainly implicit in SLE?

Answer 15

B cells, IgG

Question 16

What is the effect of autoantibody production?

Answer 16

Complement activation
Cytokine generation
End organ damage

Question 17

Describe 3 common laboratory tests done for SLE

Answer 17

ANA
Anti-dsDNA and Anti-Sm
Anti-Rho and/or anti-La

Question 18

What are the four patterns seen in positive ANA?

Answer 18

Homogenous
Speckled
Nucleolar
Centromere

Question 19

Which ANA pattern correlates to antibodies against DNA?

Answer 19

Homogenous ANA

Question 20

Which does a speckled ANA pattern show?

Answer 20

Antibodies against:
Ro
La
Sm
RNP

Question 21

What does a nucleolar ANA pattern show?

Answer 21

Antibodies against topoisomerase

Question 22

What does a nucleolar ANA pattern correlate with clinically?

Answer 22

Scleroderma

Question 23

What does a centromere ANA pattern correlate with?

Answer 23

Some cutaneous scleroderma involvement

Question 24

Compare the specificities of ANA and anti-dsDNA

Answer 24

ANA - non specific

Anti-dsDNA - more specific, less sensitive

Question 25

What is specificity?

Answer 25

Ability for a test to correctly pick up when someone doesn't have the disease (true negative)

Question 26

What is sensitivity?

Answer 26

Ability for a test to correctly pick up a disease (true positive)

Question 27

What other markers are picked up in lab tests for SLE?

Answer 27

Increased complement consumption Anti-cardiolipin antibodies Lupus anticoagulant Beta-1 glycoprotein

Question 28

What haematological features are seen in SLE?

Answer 28

``` Lymphopenia Normochromic anaemia Leukopenia AIHA Thrombocytopenia ```

Question 29

What renal markers are seen in SLE?

Answer 29

Proteinuria Haematuria Active urinary sediment

Question 30

How do you assess the severity of SLE?

Answer 30

Identify the pattern of organ involvement Monitor function of these affected organs e.g. lung function tests, echocardiography Identify pattern of autoantibodies expressed

Question 31

Watching out for which clinical features will help to pre-empt severe attacks?

Answer 31

``` Weight loss Fatigue Malaise Hair loss Rash ```

Question 32

Watching out for which laboratory features will help to pre-empt severe attacks?

Answer 32

ESR Increased complement consumption Increased anti-dsDNA

Question 33

Which lab markers are poor indicators of an SLE attack?

Answer 33

ANA | CRP

Question 34

How do you classify the severity of SLE?

Answer 34

Mild - join/skin involvement Moderate - inflammation of organs Severe - severe inflammation in vital organs incl lungs, kidney, CNS and heart

Question 35

What is the first line of treatment for MILD SLE?

Answer 35

Paracetamol/NSAIDs Hydroxychloroquine Topical corticosteroids

Question 36

What must you do whilst prescribing paracetamol or NSAIDs?

Answer 36

Monitor renal function

Question 37

When would you give hydroxychloroquine in mild SLE?

Answer 37

If there's any arthropathy or cutaneous manifestations

Question 38

What is the indication for prescribing corticosteroids in moderate disease?

Answer 38

If NSAIDs and hydroxychloroquine didn't work | Organ involvement/life threatening disease

Question 39

Which drugs would you give for severe SLE?

Answer 39

Azathioprine Cyclophosphamide Mycophenolate mofetil RItuximab

Question 40

What is the bimodal mortality pattern of SLE?

Answer 40

Early active lupus - renal failure, CNS disease, infection | Late - MI