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Musculoskeletal > Connective Tissue Disease > Flashcards

Connective Tissue Disease Flashcards

1
Q

what are connective tissue diseases?

A

characterised as a group by the presence of spontaneous over activity of the immune system

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2
Q

what is systemic lupus erythematous (SLE)?

A

a systemic autoimmune disease that can affect any part of the body

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3
Q

how does SLE cause damage to the body?

A

immune system attacks the body’s cells & tissue causing inflammation & tissue damage. Antibody-immune complexes precipitate & cause a further immune response.

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4
Q

which se does SLE affect more?

A

females

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5
Q

which ethnicities have a higher prevalence of SLE?

A

asians, afro-americans, afro-caribbeans & hispanic americans

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6
Q

an increase in which hormone is associated with a higher incidence of SLE?

A

oestrogen

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7
Q

what environmental factors is SLE associated with?

A
  • viruses
  • UV light
  • silica dust
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8
Q

what are the constitutional symptoms of SLE?

A 
fever
malaise 
poor appetite 
weight loss 
fatigue
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9
Q

what are the mucocutaenous features of SLE?

A

photosensitivity
malar rash
discoid lupus erythematosus
subacute cutaneous lupus

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10
Q

what are the MSK features of SLE?

A

non-deforming polyarthritis/polyarthralgia
deforming arthropathy (Jaccoud’s arthritis)
erosive arthritis
myopathy

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11
Q

myopathy

A

weakness
myalgia
myositis

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12
Q

what are the pulmonary features of SLE?

A 
pleurisy 
infections
diffuse lung infiltration & fibrosis 
pulmonary hypertension 
pulmonary infarct
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13
Q

what are the cardiac features of SLE?

A

pericarditis
cardiomyopathy
pulmonary hypertesnion
Libman-Sachs endocarditis

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14
Q

what is the presentation of SLE glomerulonephritis?

A 
proteinuria 
urine sediments 
urine RBS & casts 
hypertension
acute renal failure 
chronic renal failure
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15
Q

what are the neurological features of SLE?

A 
depression/psychosis 
migranous headache 
cerebral ischaemia 
cranial or peripheral neuropathy 
cerebellar ataxia
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16
Q

what are the haematological features of SLE?

A

lymphadenopathy
leucopenia
anaemia
thrombocytopenia

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17
Q

what are the intrinsic factors that make SLE patients more susceptible to infection?

A

low complements
impaired cell mediated immunity
defective phagocytosis
poor antibody response to certain antigens

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18
Q

what extrinsic factors would make SLE patients more susceptible to infection?

A

steroids
immunosuppressive drugs
nephrotic syndrome

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19
Q

what screening tests can be done for suspected SLE?

A 
FBC
renal function tests incl urine examination 
anti-nuclear antibody 
anti-double stranded DNA antibodies 
ENA
complement levels
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20
Q

when should a positive ANA test be taken seriously?

A

if other antinuclear antibodies are positive or if the patient presents with CTD features

  • anti-dsDNA
  • anti-Sm
  • anti-Ro
  • anti-RNP
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21
Q

which of the antibodies is highly specific for SLE?

A

anti-dsDNA

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22
Q

how does anti-dsDNA level correlate with SLE activity?

A

positively

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23
Q

how do C3/C4 levels correlate with SLE activity?

A

negatively

24
Q

how is SLE generally managed?

A

counselling
regular monitoring
avoid excessive sun-exposure
pregnancy issues

25
Q

which drugs are used to treat SLE?

A 
NSAIDs & simple analgesia
anti-malarials - hydroxychloroquine
steroids
immunosuppressives
biologics
26
Q

which immunosuppressive drugs can be used in SLE?

A 
azathioprine
cyclophosphamide 
methotrexate 
mychophenolate 
mofetil
27
Q

which biologics can be used in SLE

A

anti-CD20 - rituximab

anti-Blys - belimumab

28
Q

what do SLE patients die of?

A

complications of SLE in the first few years
infection
cardiovascular disease

29
Q

name the laboratory & clinical features of which 2 must be present to make a diagnosis of anti-phospholipid syndrome

A

positive anti-cardiolipin antibodies
lupus anticoagulant activity
anti-beta2-glycoprotein
arterial or venous thrombosis
pregnancy loss with no other explanation or 3 pregnancy losses with no other explanation
1 pre-term loss because of eclampsia or with signs of placental insufficiency

30
Q

which sex is anti-phospholipid syndrome more common in?

A

females

31
Q

what are the features of anti-phospholipid syndrome?

A

superficial thrombophlebitis & livedo reticularis
mild/moderate thrombocytopenia
libman-sacks endocarditis
catastrophic anti-phospholipid syndrome

32
Q

what are the neurological features of anti-phospholipid syndrome?

A

migraine

transverse myelitis

33
Q

how is anti-phospholipid syndrome treated?

A

thrombosis - lifelong anticoagulation
pregnancy loss - aspirin & heparin during pregnancy
attention to vascular risk factors

34
Q

what is sjorgen’s syndrome?

A

an autoimmune condition causing lymphocyte infiltration of exocrine glands causing xerostomia & keratoconjuctivitis sicca

35
Q

what is the classification criteria for primary sjorgen’s syndrome?

A

need 4/6 including immunology or biopsy evidence:
subjective ocular/oral symptoms daily for > 3 months
objective evidence of ocular dryness or salivary gland involvement
immunology
biopsy evidence of lymphocytic infiltrate

36
Q

what is the peak age of primary sjorgen’s syndrome?

A

40-60

37
Q

in which sex is primary sjorgen’s syndrome more common?

A

females

38
Q

what can be done to treat primary sjorgen’s syndrome?

A 
eye drops
punctal plugs 
saliva replacement 
piocarpine 
hydroxychloroquine 
steroids 
immunosuppression 
attention to CVS risk factors
39
Q

what is systemic sclerosis?

A

an autoimmune disease with features of vasculopathy, inflammation & fibrosis

40
Q

what are the common features of systemic sclerosis (CREST+)?

A 
Calconosis 
Raynaud's 
Esophageal dysmotility 
Sclerodactyly
Telangectasia 
\+ pulmonary hyperesnion
41
Q

which antibodies is systemic sclerosis associated with?

A

anti-centromere antibodies

anti-scl-70 antibodies

42
Q

which other 4 systems can be affected by systemic sclerosis?

A

GI, resp, renal & CVS

43
Q

what are the GI features of systemic sclerosis?

A

Oesophageal hypomobility
Small bowel hypomobility, bacterial overgrowth
Pancreatic insufficiency
Rectal hypomobility

44
Q

what are the resp features of systemic sclerosis?

A

Interstitial lung disease
Pulmonary hypertension
Chest wall restriction

45
Q

what are the renal features of systemic sclerosis?

A

Hypertensive renal crisis

Ischaemic

46
Q

what are the CVS features of systemic sclerosis?

A

Raynaud’s with digital ulceration
Atherosclerotic disease
Hypertensive cardiomyopathy

47
Q

what is the peak are of systemic sclerosis?

A

25-55

48
Q

in which sex is systemic sclerosis more common?

A

females

49
Q

what can be used to treat systemic sclerosis?

A 
Calcium channel blockers
Prostacyclin (Iloprost)
ACE inhibitors
Prednisolone
Immunosuppression
Bosentan, Sildenafil
50
Q

what is the major criteria for mixed connective tissue disease?

A 
Severe myositis.
Pulmonary involvement.
Raynaud's phenomenon.
Swollen hands observed.
Sclerodactyly.
Anti-U1-RNP >1:10,000.
51
Q

which autoantibody would you expect to see in SLE?

A 
ANA
anti-DNA binding 
anti-Sm
anti-Ro
anti-La
52
Q

which autoantibody would you expect to see in sjogren’s syndrome?

A

anti-Ro

anti-La

53
Q

which autoantibody would you expect to see in systemic sclerosis?

A

anti-centromere

anti-scl-70

54
Q

which autoantibody would you expect to see in mixed connective tissue disease?

A

anti-RNP

55
Q

which autoantibody would you expect to see in polymyositis?

A

anti-Jo-1

56
Q

which autoantibody would you expect to see in anti-phospholipid syndrome?

A

anti-cardiolipin antibodies

lupus anti-coagulant

57
Q

what is undifferentiated connective tissue disorder?

A

connective tissuish but usually doesn’t fit a particular syndrome, usually has a better prognosis & would expect some antibody positivity

Musculoskeletal (22 decks)

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