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MSK- Rheumatology > Connective Tissue Diseases- SLE > Flashcards

Connective Tissue Diseases- SLE Flashcards

1
Q

What body systems do connective tissue diseases affect?

A

Can affect any of them

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2
Q

Give 6 examples of connective tissue diseases?

A

SLE, APS, Sjogren’s, Systemic Sclerosis, Dermatomyositis, Polymyositis

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3
Q

Are connective tissue diseases diseases of connective tissue?

A

No

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4
Q

Connective tissue diseases are characterised by what?

A

Spontaneous over activity of the immune system

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5
Q

How quickly do connective tissue diseases come on?

A

Often evolve over a number of years

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6
Q

What type of disease is SLE?

A

Chronic autoimmune disease

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7
Q

What are some commonly affected body parts/systems in SLE?

A

Skin, joints, kidneys, blood cells and nervous system

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8
Q

What type of hypersensitivity reaction is SLE?

A

Type III- immune complex mediated

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9
Q

Are broken down cells cleared quicker or slower in SLE?

A

Slower

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10
Q

What is the result of broken down cells being cleared slower in SLE?

A

The broken down cell contents are seen as foreign and so auto-antibodies are formed against them

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11
Q

Which sex is SLE more common in?

A

Females

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12
Q

What tends to happen if men get SLE?

A

This is much less common, but if men do get SLE they will often have much more severe disease

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13
Q

What races is SLE more common in?

A

Asians, Afro-Americans, Afro-Caribbeans, Hispanic Americans

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14
Q

What are the 4 main factors involved in the aetiology of SLE?

A

Environmental, genetic, hormonal, immunological

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15
Q

What is the concordance of SLE in monozygotic twins?

A

40%

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16
Q

If a mother has SLE, what are the chances of her son or daughter getting it?

A

Son- 1/250 Daughter- 1/40

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17
Q

SLE is associated with what hormone?

A

Oestrogen

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18
Q

When does SLE usually develop in a woman?

A

After puberty, at child bearing age (most commonly 20-30)

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19
Q

What is the current 10 year survival rate for SLE?

A

> 90%

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20
Q

What is mortality in SLE more common to be caused by now?

A

Immunosuppression from drug side effects CV events

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21
Q

What are some environmental factors which may contribute to SLE?

A

Viruses (e.g. Epstein Barr), UV light, silica dust

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22
Q

What happens to apoptosis in SLE?

A

It is increased and defective

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23
Q

What is the auto-antigen in SLE?

A

Contents of necrotic broken down cells

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24
Q

When someone presents with SLE, what is the most important thing to do and why?

A

Screen for renal disease because this causes no clinical signs

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25
Q

Where are antibody-antigen complexes deposited?

A

Basement membranes of skin and kidneys (mesangium)

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26
Q

What do the immune complexes in SLE consist of?

A

Nuclear antigens and anti-nuclear antibodies

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27
Q

Where do immune complexes form?

A

Small blood vessels

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28
Q

What happens once immune complexes are in the kidneys?

A
  • Activate complement - Attracts leukocytes - Release cytokines - Inflammation - Necrosis and scarring
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29
Q

What is the SLICC classification for SLE?

A

4 or more criteria (at least one immunologic and one clinical) OR Biopsy proven lupus nephritis with positive ANA or Anti-DNA

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30
Q

What are the 10 clinical criteria for SLE?

A
  • Acute/chronic cutaneous lupus - Oral/nasal ulcers - Non-scarring alopecia - Arthritis - Serositis - Renal involvement - Neurological involvement - Haemolytic anaemia - Leukopenia - Thrombocytopenia
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31
Q

What is thrombocytopenia?

A

Low platelets

32
Q

What is serositis?

A

Inflammation of the lining of something

33
Q

What are the 6 immunological criteria for SLE?

A
  • Anti-double stranded DNA - ANA - Anti-Sm - Anti-phospholipid antibody - Low complement - Direct Coomb’s test
34
Q

When should you not count a direct Coombs test?

A

In haemolytic anaemia

35
Q

What are some constitutional features of SLE?

A

Fever, malaise, poor appetite, weight loss, fatigue

36
Q

What are the mucocutaneous features of SLE?

A
  • Photosensitivity - Malar rash - Discoid lupus - Non-scarring alopecia - Mouth/nasal ulcers (painless) - Raynaud’s
37
Q

Where does the SLE malaria rash usually spare?

A

Naso-labial folds

38
Q

How long will a lupus rash last for?

A

A long time once it is established

39
Q

How would you describe the type of arthritis associated with SLE?

A

Non-deforming

40
Q

How can you tell apart rheumatoid arthritis and a lupus arthritis?

A

RA patients will not be able to make a fist but lupus patients can

41
Q

What is another MSK feature of SLE, not arthritis?

A

Increased risk of avascular necrosis of the femoral head (most likely due to steroid use)

42
Q

What are renal features of SLE?

A

Proteinuria of > 500mg in 24 hours, red cell casts

43
Q

What test should always be performed to assess renal function in SLE? If this is positive, what is the next test?

A
  • Urinalysis - Renal biopsy
44
Q

What are neurological features of SLE?

A
  • Depression - Psychosis - Migraines - Seizures - Neuropathy
45
Q

What does thrombocytopenia give an increased risk of?

A

Bleeding

46
Q

What is important to note about the immunity of SLE patients?

A

They are very susceptible to infection

47
Q

What auto-antibody is present in titres of 1:160 in almost all SLE patients?

A

ANA

48
Q

Is ANA sensitive, specific or both for SLE?

A
  • Sensitive
49
Q

What are some other conditions that may have positive ANA?

A

RA, Hep C, HIV

50
Q

When should a positive ANA be taken seriously?

A

If other auto-antibodies are present or the patient has clinical features of a connective tissue disease

51
Q

What auto-antibody is found in around 60% of SLE patients?

A

Anti-double stranded DNA

52
Q

Which auto-antibody is most specific for SLE?

A

Anti-double stranded DNA

53
Q

Are titres of anti-dsDNA constant in SLE?

A

No, they vary with disease activity

54
Q

The majority of ENA antibodies in SLE are what?

A

Anti-Ro

55
Q

What are ENA antibodies usually associated with?

A

Cutaneous manifestations and secondary Sjogren’s features

56
Q

What can happen if Anti-Ro/Anti-La are present in a pregnant mother?

A
  • Premature lupus - Foetal heart block
57
Q

What is the specificity/sensitivity of anti-Sm in SLE?

A

Low sensitivity Very specific

58
Q

Anti-Sm antibody in SLE has an association with what?

A

Neurological involvement

59
Q

What antibody is found in around 30% of SLE cases and overlaps with features such as skin lesions, Raynaud’s and low grade myositis?

A

Anti-RNP

60
Q

A fall in complement levels indicates what?

A

A flare in disease activity

61
Q

What are some screening tests that can be done to look for other organ involvement?

A
  • CXR/pulmonary function tests/CT chest - ECHO - Nerve conduction studies - Brain MRI - FBCs
62
Q

What two things should be used for monitoring of SLE as they vary with disease activity?

A
  • C3/C4 (decrease) - Anti-dsDNA (increase)
63
Q

What two things should be monitored for CV evaluation?

A

BP and cholesterol

64
Q

What drug will all patients with SLE be started on?

A

Hydroxychloroquine

65
Q

What is the appropriate dose of steroid for skin rashes, arthritis and serositis?

A

< 15mg prednisolone/day

66
Q

What is the appropriate dose of steroid for haematological abnormalities?

A

0.5mg/kg/day prednisolone

67
Q

What is the appropriate dose of steroid for severe, resistant changes and major organ involvement?

A

1mg/kg/day prednisolone

68
Q

What is the most used immunosuppressant drug in SLE?

A

Cyclophosphamide

69
Q

What two biologics may be used in SLE?

A
  • Rituximab - Belimumab
70
Q

In general, what is the treatment for mild SLE?

A

Hydroxychloroquine, topical steroids and NSAIDs

71
Q

In general, what is the treatment for moderate SLE?

A

Hydroxychloroquine, oral steroids, azathioprine/methotrexate

72
Q

In general, what is the treatment for severe SLE?

A

Hydroxychloroquine, IV steroids, cyclophosphamide

73
Q

What drug is used in cases of SLE which are unresponsive to normal treatment?

A

Rituximab

74
Q

What is this clinical sign known as?

A

Malar (butterfly) rash

75
Q

What is this clinical sign known as?

A

Alopecia (non-scarring)

76
Q

What is this clinical sign known as?

A

Raynaud’s Phenomenon

MSK- Rheumatology (12 decks)

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