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Connective tissue diseases Flashcards

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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
how is SLE diagnosed
need 4 positive findings which are a mixture of presentation and immunological criteria
26
SLE treatment (4)
hydroxychloroquine short term steroids - topical/PO/IV depending on severity immunosuppressants (azathioprine, methotrexate, cyclophosphamide) if moderate/severe rituximab (biologics) if severe
27
what happens to a patients SLE during pregnancy
gets worse so warn them
28
complications of SLE/treatment
infection (form steroids/immunosuppression) | anti phospholipid syndrome
29
are people likely to die if they get SLE
no, 90% 10 year survival
30
what diseases can sjogrens syndrome be secondary to (2)
rheumatoid arthritis | SLE
31
who is sjogrnes syndrome common in
elderly women
32
presentation of sjogrens syndrome (4)
sicca symptoms - dry eyes, dry mouth dental problems form lack of saliva vaginal dryness parotid gland enlargement
33
what type of condition is sjogrens syndrome
inflammatory
34
pathogenesis of sjogrens syndrome
decreased lacrimal and salivary gland function = decrease in mucosal moisture
35
investigations for sjogrens syndrome (5)
``` positive schirmers test (filter paper in eye to test tear production) ANA (anti nuclear antibody) positive anti-ro anti-la labial gland biopsy ```
36
what 2 blood tests are diagnostic of sjogrens syndrome if both present
anti-ro | anti-la
37
positive anti-ro and anti-la
sjogrens syndrome
38
cure for sjogrens syndrome
no cure
39
treatment for sjogrens syndrome
methotrexate (for immunosuppression) symptomatic; artificial tears/eye drops saliva supplements
40
common complication of sjogrens syndrome
poor dental hygiene (from lack of saliva)
41
what is another name for systemic sclerosis
scleroderma
42
what is characteristic of systemic sclerosis
thickening of skin
43
what are the 2 types of systemic sclerosis
diffuse cutaneous systemic sclerosis | limited systemic sclerosis
44
which type of systemic sclerosis has later organ involvement (and hence better prognosis)
limited systemic sclerosis (think diffuse = spreads faster)
45
which type of systemic sclerosis happens faster (and hence worse prognosis)
diffuse systemic sclerosis
46
what is the skin involvement like in diffuse cutaneous systemic sclerosis
proximal to forearms (think diffuse = goes everywhere basically), incl torso
47
what is the skin involvement like in limited systemic sclerosis
distal to forearms (think limited = doesnt really spread far), not torso
48
what organ involvement occurs in diffuse cutaneous systemic sclerosis (the conditions not just the organs)
interstitial lung disease | renal failure
49
which anti-body is diagnostic of diffuse cutaneous systemic sclerosis
anti scl-70 positive
50
which anti-body is diagnostic of limited systemic sclerosis
anti-centromere positive
51
presentation of limited systemic sclerosis (acronym 5)
CREST; ``` calcinosis raynaulds esophageal dysmotility sclerodactyly (thickness/tightness of skin) telangiectasia (dilated blood vessels) ```
52
anti scl-70 positive
diffuse cutaneous systemic sclerosis
53
anti-centromere positive
limited systemic sclerosis
54
general presentation of systemic sclerosis (both diffuse cutaneous and limited) (4)
sclerodactyly (thickening/tightness of skin) raynaulds beaking of nose calcinosis (eg joint pain)
55
what antibody is positive for both limited and diffuse cutaneous systemic sclerosis
ANA (antinuclear antibody)
56
what investigation would you want to do looking for complications of systemic sclerosis
echocardiogram for pulmonary hypertension
57
treatment of systemic sclerosis (4)
``` symptomatic; if raynaulds - vasodilators (eg CCB) if renal involvement - ACE inhibitors If GI involvement - PPIS if lung involvement - immunosuppression ```
58
complications of systemic sclerosis (3)
renal lung (eg pulmonary fibrosis) pulmonary hypertension
59
what is mixed connective tissue disease
when there is an overlap of symptoms of other connective tissue diseases
60
which antibody is specific to mixed connective tissue disease
anti-RNP
61
treatment of mixed connective tissue disease
symptomatic
62
screening after diagnosis of mixed connective tissue disease (for complications)
pulmonary function tests | echocardiogram
63
anti-RNP
mixed connective tissue disease
64
common complication of mixed connective tissue disease
pulmonary hypertension
65
what is anti-phospholipid syndrome
unexplained recurrent arterial or venous thrombosis
66
what condition can anti-phospholipid syndrome be secondary to
SLE
67
which age group of people are most likely to get ant-phospholipid syndrome
young people
68
presentation of anti phospholipid syndrome
migraine miscarriage (<34 weeks) levido-reticularis (lace like pattern on skin)
69
investigations for anti phospholipid syndrome
anti-cardiolipin antibody lupus anticoagulant anti-beta2 glycoprotein
70
how many times do you need to repeat anti-phospholipid investigations to confirm diagnosis and how far apart are the repetitions why
twice 12 weeks apart can randomly be present = false positives
71
treatment of anti-phospholipid syndrome
warfarin for life (blood thinner) LMW heparin if pregnant to prevent pregnancy complications hydroxychloroquine
72
what type of drug is hydroxychloroquine
anti-malarial
73
what type of connective tissue disease presents with levido reticularis (lace like purple pattern on skin)
anti phospholipid syndrome
74
complications of anti-phospholipid syndrome + mortality
catastrophic APS (multiorgan infarction) 50% mortality if CAPS
75
which renal problems are associated with lupus (2)
proteinuria | nephrotic syndrome
76
% of people with lupus with renal involvement
50%
77
lupus with proteinuria on urinalysis what is the next line investigation why
renal biopsy classification of lupus nephritis determines treatment

J MSK (30 decks)

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