Connective Tissue Disease - Systemic Lupus Erthematous Flashcards

1
Q

What are connective tissue diseases?

A

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

Not diseases of connective tissue

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2
Q

Which part of the body does SLE affect?

A

Any part of the body

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3
Q

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

A

Type III

(Immune complex mediated)

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4
Q

Which types of people are more susceptible to SLE?

A
  • Females
  • Hispanic americans
  • Asians
  • Afro-Carribeans
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5
Q

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

A

Oestrogen

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6
Q

Which environmental factors may trigger SLE?

A
  • Smoking
  • Silica dust or cement
  • UV light
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7
Q

Which virus is known to induce SLE?

A

Epstein-Barr virus

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8
Q

What is the pathophysiology behind SLE?

A
  • 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
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9
Q

Why is renal disease a major worry in SLE?

A

It is symptomless

Eventuallu will lead to necrosis and scarring

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10
Q

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

A
  • Immune complexes are deposited in the mesangium
  • Complement is activated, leukocytes and other inflammatory cells are attracted
  • Necrosis and scarring results
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11
Q

Where may ulcers be found in a patient with SLE?

A

Mouth (or nasal) ulcers

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12
Q

How is SLE diagnosed?

A

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)

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13
Q

SLE can be associated with thrombocytopenia, what is this?

A

A low platelet count

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14
Q

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

A

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

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15
Q

How can the hair be affected in SLE?

A

Alopecia can occur

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16
Q

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

A
  • Butterfly malar rash
  • Space naso-labial folds
  • Can be associated with UV light exposure
17
Q

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

A

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
Q

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

A

SLE can cause serositis

19
Q

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

A
  • 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
Q

What are some common neurological features of SLE?

A
  • Headache/migraines (most common)
  • Depression
  • Seizures
  • Foot drop
  • Mononeuritis multiplex
  • Cranial or peripheral neuropathy
21
Q

What are some haematological symptoms in SLE?

A
  • Lymphadenopathy
  • Leukopenia
  • Lymphopenia
  • Thrombocytopenia
  • Haemolytic anaemia
22
Q

What does anti-phospholipid syndrome involve?

A

Thrombosis of arteries and/or veins

23
Q

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

A

Livedo reticularis

24
Q

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

A

ANA

(anti-nuclear antibody)

25
Q

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

A

Symptoms are present

Other anti-nuclear antibodies are high

26
Q

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

A

Anti-dsDNA

(anti-double stranded DNA antibody)

It can be associated with Lupus Nephritis

27
Q

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

A

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
Q

Anti-Ro is associated with which other antibody?

A

Anti-La

29
Q

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

A

Anti-Sm

(anti-Smith)

30
Q

What are the three anti-phospholipid antibodies?

A
  1. Anti-cardiolipin antibody
  2. Lupus anticoagulant
  3. Anti-beta 2 glycoprotein
31
Q

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

A

They must be positive on 2 occasions 12 weeks apart

32
Q

How is CRP affected in SLE?

A

It remains unchanged

(ESR/PV will increase with active disease however)

33
Q

How are C4 levels impacted in SLE?

A

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

34
Q

Everyone with SLE is given which anti-malarial drug?

A

Hydroxychloroquine

35
Q

Which treatments (pharmacological) can be given for SLE?

A
  • NSAIDs/analgesia
  • Hydroxychloroquine
  • Steroid (not used long term)
  • Immunosuppressives
36
Q

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

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

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

37
Q

What impact do immunosuppressive drugs have on the bone?

A

They cause bone marrow suppression

38
Q

Which biological agents may be used in SLE?

A
  • Rituximab
  • Belimumab