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J MSK > Connective tissue diseases > Flashcards

Connective tissue diseases Flashcards

1
Q

what inflammatory cell is associated with connective tissue diseases

A

autoantibodies

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2
Q

are connective tissue disease more common in males or females

A

females

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3
Q

what are the connective tissue diseases (6)

A 
systemic lupus erythematous (SLE) 
sjorgens syndrome 
systemic sclerosis 
mixed connective tissue disease 
polymyositis (a muscle disease)
anti-phospholipid syndrome
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4
Q

what is the general treatment for mild connective tissue disease (3)

A

steroids (short term)
NSAIDs
hydroxychloroquine

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5
Q

what is the general treatment for joint inflammation associated with connective tissue disease (2)

A

DMARDs (eg methotrexate)

steroids (short term)

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6
Q

what is the general treatment for severe connective tissue disease (2)

A

high dose steroids (short term)

immunosuppression

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7
Q

what is the female:male ratio in SLE

A

9:1

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8
Q

what group of people are most likely to have SLE (sex and age)

A

women of child bearing age aged 20-30, (to do with hormones)

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9
Q

is SLE rare or common

A

rare but well known

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10
Q

what process increases at the start of SLE pathogenesis

A

apoptosis

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11
Q

increased apoptosis results in … in SLE

A

increased autoantibody production (to try and clear up dead cells that are floating about)

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12
Q

what is the consequence of increased autoantibody production in SLE

A

increased immune response

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13
Q

what are the 3 stages of SLE pathogenesis

A

increased apoptosis
autoantibody production
unnecessary immune response

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14
Q

what happens when all these immune complexes are gathered for no reason in SLE

A

they collect in blood vessels, skin, kidneys etc = cause inflammation

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15
Q

what type of hypersensitivity reaction is SLE

A

type III

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16
Q

how does SLE present (11)

A 
LOTS OF THINGS SO HARD TO DIAGNOSE, red flags;
butterfly malar rash 
fever
malaise 
weight loss
painless mouth/nasal ulcers 
inflammatory arthritis 
blood problems 
neuro problems 
anti phospholipid syndrome
renal problems - lupus nephritis 
photosensitivity
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17
Q

SLE investigations and findings for diagnosis (3)

A

ANA (anti nuclear antibody) positive
anti-dsDNA (anti double stranded DNA) positive
anti-sm positive

+more

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18
Q

what is the normal ratio of ANA

what is the ration of ANA in SLE

A

low - 1:160 or 1:80

> 1:80

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19
Q

is ANA specific to SLE

does ANA vary with disease progression in SLE

A

no present in other autoantibody conditions

no, either positive or negative

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20
Q

is anti-dsDNA specific to SLE

does anti-dsDNA vary with disease progression in SLE

A

yes

yes - increases during flares, but always positive

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21
Q

which antibody test is best for SLE

A

anti-dsDNA (though ANA and anti-sm are also pretty good)

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22
Q

what investigation can diagnosis a SLE flare up

what happens to the levels during a flare up

A

complement 4 (C4)

decrease during flare up

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23
Q

which disease do you ALWAYS want to screen for after diagnosis of SLE

why

A

kidney disease

it is asymptomatic

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24
Q

how do you screen for kidney disease in SLE after diagnosis

A

urinalysis

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25
Q

how is SLE diagnosed

A

need 4 positive findings which are a mixture of presentation and immunological criteria

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26
Q

SLE treatment (4)

A

hydroxychloroquine
short term steroids - topical/PO/IV depending on severity
immunosuppressants (azathioprine, methotrexate, cyclophosphamide) if moderate/severe
rituximab (biologics) if severe

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27
Q

what happens to a patients SLE during pregnancy

A

gets worse so warn them

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28
Q

complications of SLE/treatment

A

infection (form steroids/immunosuppression)

anti phospholipid syndrome

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29
Q

are people likely to die if they get SLE

A

no, 90% 10 year survival

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30
Q

what diseases can sjogrens syndrome be secondary to (2)

A

rheumatoid arthritis

SLE

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31
Q

who is sjogrnes syndrome common in

A

elderly women

32
Q

presentation of sjogrens syndrome (4)

A

sicca symptoms - dry eyes, dry mouth
dental problems form lack of saliva
vaginal dryness
parotid gland enlargement

33
Q

what type of condition is sjogrens syndrome

A

inflammatory

34
Q

pathogenesis of sjogrens syndrome

A

decreased lacrimal and salivary gland function = decrease in mucosal moisture

35
Q

investigations for sjogrens syndrome (5)

A 
positive schirmers test (filter paper in eye to test tear production) 
ANA (anti nuclear antibody) positive 
anti-ro
anti-la
labial gland biopsy
36
Q

what 2 blood tests are diagnostic of sjogrens syndrome if both present

A

anti-ro

anti-la

37
Q

positive anti-ro and anti-la

A

sjogrens syndrome

38
Q

cure for sjogrens syndrome

A

no cure

39
Q

treatment for sjogrens syndrome

A

methotrexate (for immunosuppression)

symptomatic;

artificial tears/eye drops
saliva supplements

40
Q

common complication of sjogrens syndrome

A

poor dental hygiene (from lack of saliva)

41
Q

what is another name for systemic sclerosis

A

scleroderma

42
Q

what is characteristic of systemic sclerosis

A

thickening of skin

43
Q

what are the 2 types of systemic sclerosis

A

diffuse cutaneous systemic sclerosis

limited systemic sclerosis

44
Q

which type of systemic sclerosis has later organ involvement (and hence better prognosis)

A

limited systemic sclerosis (think diffuse = spreads faster)

45
Q

which type of systemic sclerosis happens faster (and hence worse prognosis)

A

diffuse systemic sclerosis

46
Q

what is the skin involvement like in diffuse cutaneous systemic sclerosis

A

proximal to forearms (think diffuse = goes everywhere basically), incl torso

47
Q

what is the skin involvement like in limited systemic sclerosis

A

distal to forearms (think limited = doesnt really spread far), not torso

48
Q

what organ involvement occurs in diffuse cutaneous systemic sclerosis (the conditions not just the organs)

A

interstitial lung disease

renal failure

49
Q

which anti-body is diagnostic of diffuse cutaneous systemic sclerosis

A

anti scl-70 positive

50
Q

which anti-body is diagnostic of limited systemic sclerosis

A

anti-centromere positive

51
Q

presentation of limited systemic sclerosis (acronym 5)

A

CREST;

calcinosis 
raynaulds
esophageal dysmotility 
sclerodactyly (thickness/tightness of skin)
telangiectasia (dilated blood vessels)
52
Q

anti scl-70 positive

A

diffuse cutaneous systemic sclerosis

53
Q

anti-centromere positive

A

limited systemic sclerosis

54
Q

general presentation of systemic sclerosis (both diffuse cutaneous and limited) (4)

A

sclerodactyly (thickening/tightness of skin)
raynaulds
beaking of nose
calcinosis (eg joint pain)

55
Q

what antibody is positive for both limited and diffuse cutaneous systemic sclerosis

A

ANA (antinuclear antibody)

56
Q

what investigation would you want to do looking for complications of systemic sclerosis

A

echocardiogram for pulmonary hypertension

57
Q

treatment of systemic sclerosis (4)

A 
symptomatic;
if raynaulds - vasodilators (eg CCB)
if renal involvement - ACE inhibitors 
If GI involvement - PPIS
if lung involvement - immunosuppression
58
Q

complications of systemic sclerosis (3)

A

renal
lung (eg pulmonary fibrosis)
pulmonary hypertension

59
Q

what is mixed connective tissue disease

A

when there is an overlap of symptoms of other connective tissue diseases

60
Q

which antibody is specific to mixed connective tissue disease

A

anti-RNP

61
Q

treatment of mixed connective tissue disease

A

symptomatic

62
Q

screening after diagnosis of mixed connective tissue disease (for complications)

A

pulmonary function tests

echocardiogram

63
Q

anti-RNP

A

mixed connective tissue disease

64
Q

common complication of mixed connective tissue disease

A

pulmonary hypertension

65
Q

what is anti-phospholipid syndrome

A

unexplained recurrent arterial or venous thrombosis

66
Q

what condition can anti-phospholipid syndrome be secondary to

A

SLE

67
Q

which age group of people are most likely to get ant-phospholipid syndrome

A

young people

68
Q

presentation of anti phospholipid syndrome

A

migraine
miscarriage (<34 weeks)
levido-reticularis (lace like pattern on skin)

69
Q

investigations for anti phospholipid syndrome

A

anti-cardiolipin antibody
lupus anticoagulant
anti-beta2 glycoprotein

70
Q

how many times do you need to repeat anti-phospholipid investigations to confirm diagnosis

and how far apart are the repetitions

why

A

twice 12 weeks apart

can randomly be present = false positives

71
Q

treatment of anti-phospholipid syndrome

A

warfarin for life (blood thinner)
LMW heparin if pregnant to prevent pregnancy complications
hydroxychloroquine

72
Q

what type of drug is hydroxychloroquine

A

anti-malarial

73
Q

what type of connective tissue disease presents with levido reticularis (lace like purple pattern on skin)

A

anti phospholipid syndrome

74
Q

complications of anti-phospholipid syndrome + mortality

A

catastrophic APS (multiorgan infarction)

50% mortality if CAPS

75
Q

which renal problems are associated with lupus (2)

A

proteinuria

nephrotic syndrome

76
Q

% of people with lupus with renal involvement

A

50%

77
Q

lupus with proteinuria on urinalysis

what is the next line investigation

why

A

renal biopsy

classification of lupus nephritis determines treatment

J MSK (30 decks)

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