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Connective Tissue Disease Flashcards

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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?

24
Q

how is SLE generally managed?

Study These Flashcards
A

counselling
regular monitoring
avoid excessive sun-exposure
pregnancy issues

25
which drugs are used to treat SLE?
``` NSAIDs & simple analgesia anti-malarials - hydroxychloroquine steroids immunosuppressives biologics ```
26
which immunosuppressive drugs can be used in SLE?
``` azathioprine cyclophosphamide methotrexate mychophenolate mofetil ```
27
which biologics can be used in SLE
anti-CD20 - rituximab | anti-Blys - belimumab
28
what do SLE patients die of?
complications of SLE in the first few years infection cardiovascular disease
29
name the laboratory & clinical features of which 2 must be present to make a diagnosis of anti-phospholipid syndrome
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
which sex is anti-phospholipid syndrome more common in?
females
31
what are the features of anti-phospholipid syndrome?
superficial thrombophlebitis & livedo reticularis mild/moderate thrombocytopenia libman-sacks endocarditis catastrophic anti-phospholipid syndrome
32
what are the neurological features of anti-phospholipid syndrome?
migraine | transverse myelitis
33
how is anti-phospholipid syndrome treated?
thrombosis - lifelong anticoagulation pregnancy loss - aspirin & heparin during pregnancy attention to vascular risk factors
34
what is sjorgen's syndrome?
an autoimmune condition causing lymphocyte infiltration of exocrine glands causing xerostomia & keratoconjuctivitis sicca
35
what is the classification criteria for primary sjorgen's syndrome?
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
what is the peak age of primary sjorgen's syndrome?
40-60
37
in which sex is primary sjorgen's syndrome more common?
females
38
what can be done to treat primary sjorgen's syndrome?
``` eye drops punctal plugs saliva replacement piocarpine hydroxychloroquine steroids immunosuppression attention to CVS risk factors ```
39
what is systemic sclerosis?
an autoimmune disease with features of vasculopathy, inflammation & fibrosis
40
what are the common features of systemic sclerosis (CREST+)?
``` Calconosis Raynaud's Esophageal dysmotility Sclerodactyly Telangectasia + pulmonary hyperesnion ```
41
which antibodies is systemic sclerosis associated with?
anti-centromere antibodies | anti-scl-70 antibodies
42
which other 4 systems can be affected by systemic sclerosis?
GI, resp, renal & CVS
43
what are the GI features of systemic sclerosis?
Oesophageal hypomobility Small bowel hypomobility, bacterial overgrowth Pancreatic insufficiency Rectal hypomobility
44
what are the resp features of systemic sclerosis?
Interstitial lung disease Pulmonary hypertension Chest wall restriction
45
what are the renal features of systemic sclerosis?
Hypertensive renal crisis | Ischaemic
46
what are the CVS features of systemic sclerosis?
Raynaud’s with digital ulceration Atherosclerotic disease Hypertensive cardiomyopathy
47
what is the peak are of systemic sclerosis?
25-55
48
in which sex is systemic sclerosis more common?
females
49
what can be used to treat systemic sclerosis?
``` Calcium channel blockers Prostacyclin (Iloprost) ACE inhibitors Prednisolone Immunosuppression Bosentan, Sildenafil ```
50
what is the major criteria for mixed connective tissue disease?
``` Severe myositis. Pulmonary involvement. Raynaud's phenomenon. Swollen hands observed. Sclerodactyly. Anti-U1-RNP >1:10,000. ```
51
which autoantibody would you expect to see in SLE?
``` ANA anti-DNA binding anti-Sm anti-Ro anti-La ```
52
which autoantibody would you expect to see in sjogren's syndrome?
anti-Ro | anti-La
53
which autoantibody would you expect to see in systemic sclerosis?
anti-centromere | anti-scl-70
54
which autoantibody would you expect to see in mixed connective tissue disease?
anti-RNP
55
which autoantibody would you expect to see in polymyositis?
anti-Jo-1
56
which autoantibody would you expect to see in anti-phospholipid syndrome?
anti-cardiolipin antibodies | lupus anti-coagulant
57
what is undifferentiated connective tissue disorder?
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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