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MSK > Connective Tissue Diseases > Flashcards

Connective Tissue Diseases Flashcards

1
Q

These are diseases of connective tissue. True or false?

A

False

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2
Q

SLE is what type of disease?

A

Autoimmune

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3
Q

What type of hypersensitivity disease is SLE?

A

Type III- immune complex mediated

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4
Q

Normally cells break down and are cleared by the immune system. Is this process faster or slower in SLE?

A

Slower

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5
Q

What are antibodies formed against in SLE?

A

Contents of broken down cells which have not been cleared due to defective immune system

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6
Q

Which sex is SLE more common in?

A

Females

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7
Q

What race is SLE more common in?

A

Asians

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8
Q

What is the extent of SLE generally in males?

A

They are less likely to develop it, but if they do the extent is usually much worse

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9
Q

Is there a genetic component to SLE?

A

Yes

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10
Q

Which hormone is related to SLE?

A

Oestrogen

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11
Q

What are 3 examples of environmental factors which may influence SLE?

A

Viruses, silica dust and UV light

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12
Q

Is apoptosis increased or decreased in SLE?

A

Increased

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13
Q

What is the most important thing to look out for in patients with either diagnosed or potential SLE? Why?

A

Renal disease- it causes no clinical signs or symptoms

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14
Q

Where do immune complexes get deposited in the kidneys in SLE?

A

Mesangium

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15
Q

What is the mesangium?

A

A structure associated with capillaries in the glomerulus

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16
Q

Where do immune complexes form in SLE?

A

In the circulation

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17
Q

What is serositis?

A

Inflammation of the lining of something

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18
Q

What are some mucocutaneous features of SLE?

A
  • Photosensitivity
  • Malar rash
  • Discoid lupus
  • Mouth ulcers
  • Non- scarring alopecia
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19
Q

Where does the malar rash of SLE usually spare?

A

Naso-labial folds

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20
Q

A lupus rash only lasts for a short time. True or false?

A

False- it will last for a long time after it has started

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21
Q

In terms of the hands, what is the difference between SLE and RA patients?

A

RA will not be able to make a fist, SLE will

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22
Q

What is suggestive of renal involvement in SLE?

A

Proteinurea > 500mg in 24 hours, red cell casts

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23
Q

What test should always be performed when suspicious of SLE?

A

Urinalysis, if + then renal biopsy

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24
Q

What feature of SLE gives an increased bleeding risk?

A

Thrombocytopenia

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25
Q

People with SLE are very susceptible to infection. True or false?

A

True

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26
Q

What three groups of auto-antibody are associated with SLE?

A
  • Anti-Nuclear Antibody
  • Anti- Double Stranded DNA Antibody
  • Anti-Extractable Nuclear Antigens Antibody
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27
Q

What are 4 anti-ENA antibodies which may be associated with SLE?

A

Anti-Ro, Anti-La, Anti-Sm, Anti-RNP

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28
Q

Which two antibodies found in SLE are usually associated with cutaneous manifestations and secondary Sjogren’s features?

A

Anti Ro and Anti La

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29
Q

If Anti-Ro and Anti-La antibodies are present in a pregnant mother, what can this cause?

A

Premature lupus and foetal heart block

30
Q

Which Anti-ENA antibody is most specific for SLE?

A

Anti-Sm

31
Q

What is classed as a positive ANA test?

A

Positive in titre of 1:160 or higher

32
Q

Is ANA sensitive/specific/both for SLE?

A

Sensitive but not specific

33
Q

When should a positive ANA test be taken seriously?

A

When there are other autoantibodies present or when the patient presents with signs of CTD

34
Q

Is anti-double stranded DNA antibody specific for SLE?

A

Yes

35
Q

Concentrations of anti-double stranded DNA antibody vary with disease activity of SLE. True or false?

A

True

36
Q

What happens to complement levels during a flare of SLE?

A

They fall

37
Q

What drug will all patients with SLE be started on?

A

Hydroxychloroquine

38
Q

What are the main groups of drugs given for SLE?

A

NSAIDs and simple analgesia

39
Q

What dose of steroids should be given for an SLE patient with skin rashes, arthritis and serositis?

A

< 15mg/day prednisolone

40
Q

What dose of steroids should be given for an SLE patient with haematological involvement?

A

0.5mg/kg/day prednisolone

41
Q

What dose of steroids should be given for an SLE patient with resistant changes or major organ involvement?

A

1mg/kg/day prednisolone

42
Q

What is the most used immunosuppressant in SLE?

A

Cyclophosphamide

43
Q

What two biologics are options for SLE?

A

Rituximab, belimumab

44
Q

What is the treatment plan for mild SLE?

A
  • Hydroxychloroquine
  • Topical Steroids
  • NSAIDs
45
Q

What is the treatment plan for moderate SLE?

A
  • Hydroxychloroquine
  • Oral steroids
  • Azathioprine/methotrexate
46
Q

What is the treatment plan for severe SLE?

A
  • Hydroxychloroquine
  • IV Steroids
  • Cyclophosphamide
  • Rituximab
47
Q

What are the two main features of APS?

A
  • Venous or arterial thrombosis

- Adverse pregnancy outcomes

48
Q

What is the rash of APS known as?

A

Livedo reticularis

49
Q

What other conditions is APS associated with?

A

Any other autoimmune condition but especially SLE

50
Q

What are the 3 anti-phospholipid antibodies?

A
  • Anti-Cardiolipin (IgM/IgG)
  • Lupus anticoagulant
  • Anti-Beta 2 Glycoprotein
51
Q

What is the rule with regards to autoantibodies to make a diagnosis of APS?

A

Must be positive on 2 occasions 12 weeks apart

52
Q

Do you have to be sensitive to all 3 antibodies for a diagnosis of APS?

A

No

53
Q

What lifelong therapy should APS patients be on?

A

Anticoagulation (aspirin/heparin) and hydroxychloroquine

54
Q

The diminished function of what causes the typical dry eyes and mouth of Sjogren’s syndrome?

A

Lacrimal and salivary glands

55
Q

Who is Sjogren’s syndrome most common in?

A

Women around 50s-60s

56
Q

Apart from the dry mouth/eyes etc, what are other clinical features of Sjogren’s?

A

Joint Pains
Fatigue
Dental caries

57
Q

Which antibodies are most associated with Sjogren’s?

A

Anti-Ro and anti-La

58
Q

Apart from the antibodies, what other tests can be done for Sjogren’s?

A

Salivary gland ultrasound

Labial gland biopsy

59
Q

What is the general treatment for Sjogren’s syndrome?

A

Artificial tears and saliva

Hydroxychloroquine

60
Q

Should immunosuppressants ever be given in Sjogren’s?

A

Yes if there is major organ involvement

61
Q

What is the difference between diffuse and limited systemic sclerosis?

A

Limited- only really involves the skin

Diffuse- involves skin and other organ systems

62
Q

What antibodies are associated with diffuse systemic sclerosis?

A
  • Anti-Topoisomerase
  • Anti-SCL-70
  • Anti-RNA- III polymerase
63
Q

Where is the skin involvement in diffuse systemic sclerosis?

A

Proximal to forearms and torso

64
Q

Where is the skin involvement in limited systemic sclerosis?

A

Distal to elbows

65
Q

What are the CREST signs of limited systemic sclerosis?

A
  • Calcinosis
  • Raynaud’s
  • Oesophageal dysmotility
  • Sclerodactyly
  • Telangectasia
66
Q

What type of hypertension is related to systemic sclerosis?

A

Pulmonary

67
Q

What antibody is associated with limited systemic sclerosis?

A

Anti-centromere antibody

68
Q

What can you use to treat both Raynaud’s and pulmonary hypertension?

A

Ca++ channel blockers

69
Q

What can you use to treat reflux?

A

PPIs, H2 antagonists

70
Q

What antibody is positive in mixed CTD?

A

Anti-RNP

MSK (6 decks)

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