Connective Tissue Disease - Systemic Lupus Erthematous Flashcards Preview

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Flashcards in Connective Tissue Disease - Systemic Lupus Erthematous Deck (38):
1

What are connective tissue diseases?

Diseases characterized by the presence of spontaneous over activity of the immune system.

Not diseases of connective tissue

2

Which part of the body does SLE affect?

Any part of the body

3

Systemic lupus erythematous is an example of which type of hypersensitivity reaction?

Type III

(Immune complex mediated)

4

Which types of people are more susceptible to SLE?

  • Females
  • Hispanic americans
  • Asians
  • Afro-Carribeans

5

What hormone is thought to be associated with increased chances of developing SLE?

Oestrogen

6

Which environmental factors may trigger SLE?

  • Smoking
  • Silica dust or cement
  • UV light

7

Which virus is known to induce SLE?

Epstein-Barr virus

8

What is the pathophysiology behind SLE?

  • There is an increase in self cell death
  • Defective apoptosis occurs
  • Necrotic release of nuclear material occurs
  • There is delayed clearance of nuclear material and autoantibodies are made against this material
  • Immune complex formation occurs

9

Why is renal disease a major worry in SLE?

It is symptomless

Eventuallu will lead to necrosis and scarring

10

Why does SLE lead to necrosis and scarring in the kidneys?

  • Immune complexes are deposited in the mesangium
  • Complement is activated, leukocytes and other inflammatory cells are attracted
  • Necrosis and scarring results

11

Where may ulcers be found in a patient with SLE?

Mouth (or nasal) ulcers

12

How is SLE diagnosed?

SLICC classification

(>= 4 criteria with at least 1 clinical and 1 labratory investigation required for diagnosis OR biopsy proven lupus nephritis and the presence of ANA or Anti dsDNA)

13

SLE can be associated with thrombocytopenia, what is this?

A low platelet count

14

How does discoid lupus erythematous differ in terms of facial rash, with SLE?

There is a scaly surface to the rash, which is also generally more well demarkated

15

How can the hair be affected in SLE?

Alopecia can occur

16

Describe the facial rash of SLE and the area(s) it avoids

  • Butterfly malar rash
  • Space naso-labial folds
  • Can be associated with UV light exposure

17

Jaccoud's arthritis is associated with SLE, what is it?

Inflammation of the joint capsule

  • MCP joint subluxation (of 2nd to 5th digits) and hyperextension at the related PIP joints
  • This is deforming, yet reversible

18

In SLE it is not uncommon for patients to present with percarditis or pleural effusion. Why is this?

SLE can cause serositis

19

If SLE impacts the kidneys, causing lupus nephritis, what characteristic features will be shown on urinalysis?

  • Proteinuria of >500mg in 24 hours
  • Red cell casts (blood in urine)

If either of these features is present then renal biopsy is required

20

What are some common neurological features of SLE?

  • Headache/migraines (most common)
  • Depression
  • Seizures
  • Foot drop
  • Mononeuritis multiplex
  • Cranial or peripheral neuropathy

21

What are some haematological symptoms in SLE?

  • Lymphadenopathy
  • Leukopenia
  • Lymphopenia
  • Thrombocytopenia
  • Haemolytic anaemia

22

What does anti-phospholipid syndrome involve?

Thrombosis of arteries and/or veins

23

What is the term given to the mottled, net like skin appearance seen in patients with anti-phospholipid syndrome?

Livedo reticularis

 

24

Which antibody is present in almost all SLE patients, but has a relatively low level of specificity?

ANA

(anti-nuclear antibody)

25

When would a raised ANA (>= 1 : 160) be suggestive of SLE?

Symptoms are present

Other anti-nuclear antibodies are high

26

Which antibody occurs in around 60% of patients with SLE, but is highly specific?

Anti-dsDNA

(anti-double stranded DNA antibody)

It can be associated with Lupus Nephritis

27

Which antibody associated with SLE is also associated with the skin manifestations of the disease?

Anti-Ro

Pregant women can pass on this antibody to their unborn child which can result in symptoms such as heart block or neonatal LE

28

Anti-Ro is associated with which other antibody?

Anti-La

29

Which antibody is very specific for SLE and has probable association with neurological involvement?

Anti-Sm

(anti-Smith)

30

What are the three anti-phospholipid antibodies?

  1. Anti-cardiolipin antibody
  2. Lupus anticoagulant
  3. Anti-beta 2 glycoprotein

31

What is the criteria involving anti-phospholipid syndrome antibodies in order for a diagnosis to be made?

They must be positive on 2 occasions 12 weeks apart

32

How is CRP affected in SLE?

It remains unchanged

(ESR/PV will increase with active disease however)

33

How are C4 levels impacted in SLE?

They are reduced - C4 is used up to form immune complexes

34

Everyone with SLE is given which anti-malarial drug?

Hydroxychloroquine

35

Which treatments (pharmacological) can be given for SLE?

  • NSAIDs/analgesia
  • Hydroxychloroquine
  • Steroid (not used long term)
  • Immunosuppressives

36

Which immunosupressives can be given for SLE, which is normally used and when may the other three not be used?

  • Cyclophosphamide (usually used)
  • Methotrexate
  • Azathioprine
  • Mycophenolate mofetil

Careful use of immunosuppressives is essential since they can be teratogenic in pregnancy

37

What impact do immunosuppressive drugs have on the bone?

They cause bone marrow suppression

38

Which biological agents may be used in SLE?

  • Rituximab
  • Belimumab

Decks in The Musculoskeletal System Class (58):