connective tissue disease Flashcards

1
Q

what type of hypersensitivity reaction is systemic lupus erythematous?

A

type 3 HS

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2
Q

how many symptoms do you need for a diagnosis of SLE?

A

at least 4

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3
Q

what are the constitutional symptoms of SLE?

A
fatigue
weight loss
malaise 
fever
poor appetite
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4
Q

what are the skin symptoms of SLE?

A
malar rasht hat spares the nasolabial folds 
(butterfly rash) 
photosenstivity 
alopecia
scaly scarring discoid rash
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5
Q

what symptom of the mouth is common is SLE?

A

mouth ulcers

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6
Q

what sort of joint pain is common in SLE?

A

non erosive small joint arthritis similar to RA

Jaccouds arthritis - non erosive arthritis associated with ulnar deviation

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7
Q

what chest symptoms are in SLE?

A

pericarditis

pleural effusion/pleuritic chest pain/SOB

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8
Q

what affect can SLE have on your kindeys?

A

glomerulonephritis?

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9
Q

what haematological conditions can be caused by SLE?

A

lymphadenopathy
leukopenia
anaemia
thrombocytopaenia

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10
Q

what is Libman Sack in SLE?

A

a type of non bacterial sterile endocarditis

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11
Q

what is a symptom of the hands associated with SLE?

A

raynauds

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12
Q

what is the 1st screening test done for SLE? what should follow if positive?

A

urine analysis

renal biopsy

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13
Q

what immunology is associated with SLE?

A

anti - ANA (non specific)

anti - DsDNA

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14
Q

what is anti-DsDNA associated with?

A

it increases with disease activity

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15
Q

what would you expect C3/4 to do with active disease?

A

decrease

complement used up

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16
Q

is CRP used in SLE?

A

no

does not increase with flare ups (only raised during infection/co-exciting disease e.g. RA)

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17
Q

what is mild SLE?

A

skin disease and arthralgia

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18
Q

what is moderate SLE?

A

inflammatory arthritis and organ involvement

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19
Q

what is severe SLE?

A

organ disease

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20
Q

what is the treatment for mild SLE?

A

hydroxycloroquine
NSAIDs
topical steroids

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21
Q

what is the treatment for moderate SLE?

A

methotrexate
azathioprine
oral steroids

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22
Q

what is the treatment for severe SLE?

A

IV steroids
cyclophosphamide
rituximab

23
Q

what else should be done to manage SLE?

A

avoid sun exposure (photosensitivity)

24
Q

how does drug induced SLE present?

A

feature of lupus without renal or neurological involvement

- arthralgia, myalgia, malaria skin rash, ANA +ve

25
Q

what drug commonly causes SLE?

A

hydralazine

stops on stopping the drug

26
Q

what can cause anti-phospholipid syndrome?

A

primary

secondary to lupus

27
Q

what symptoms are associated with anti-phospholipid syndrome?

A

venous/arterial thrombosis (DVT)
recurrent miscarriage
thrombocytopenia

28
Q

what skin symptom is seen in APS?

A

livedo reticularis

mottled skin covered with red/purple patched in a web pattern

29
Q

how is a diagnosis of APS made?

A

1 lab and 1 clinical finding

30
Q

what are the clinical findings needed to make a diagnosis of APS?

A

aterial/venous thrombosis
pregnancy loss with no other explanation from 10 weeks onwards
3 pregnancy losses before 10 weeks
pregnancy loss due to pre-eclampsia/placental insufficiency

31
Q

what are the lab findings needed to make a diagnosis of APS?

A
2 positive findings, 12 weeks apart 
anti-cardiolipin antibody 
lupus anti-coagulation test 
anti-b2 glycoprotein 
causes low platelets and prolonged APTT
32
Q

what is the treatment for APS after a primary thrombosis?

A

low dose aspirin

33
Q

what is the treatment for APS after a secondary thrombosis?

A

after first VTW, life long warfarin
INR 2-3
if it happens on warfarin then low dose aspirin too

34
Q

what is the treatment for APS if pregnant?

A

aspirin between pregnancies

aspirin and heparin during pregnancy (warfarin teratogenic)

35
Q

what does sjogrens increase your risk of?

A

lymphoma

increased B cell activity

36
Q

what are the symptoms of Sjogrens syndrome?

A
DRYNESS
kertoconjunctivitis sicca (dry eyes) 
wake up at night needing water, difficulty swallowing bread
xerostomia (dry mouth) 
parotid swelling 
vaginal dryness
fatigue, raynauds 
tooth decay
37
Q

what test can be done for Sjogrens?

A

Schrimers test
filter paper on eye, access how wet it is
<5mm in 5 mins = +ve dry eyes

38
Q

what immunology is positive of Sjogrens

A

anti-ro
anti-la
ANA +ve (non specific)

39
Q

what is the treatment for Sjogrens?

A

1st line hydrocholoriquine (for fatigue and arthralgia)

pilocarpine - stimulate salivary glands

40
Q

who should not use pilocarpine?

A

asthmatics

41
Q

what should be monitored for in Sjogrens?

A

non Hodgkins B cell lymphoma

42
Q

what is scleroderma?

A

term meaning skin thickening

43
Q

what does 90% of sclerosis patients present with?

A

raynauds

44
Q

what is localised scleroderma?

A

dermal fibrosis and inflammation without any organ involvement
like to be seen with raynauds

45
Q

what is systemic sclerosis (CREST)?

A

skin fibrosis associated with sclerosis of viscera and proceeded by raynauds

46
Q

what it the presentation of sclerosis?

A
lung fibrosis 
pulmonary hypertension 
hypertension 
dysphagia
renal hypertensive crisis
47
Q

what is the classification for slerosis?

A

diffuse (30%)

limited (70%)

48
Q

what immunology and onset is seen in diffuse sclerosis?

A

anti-SCL 70

onset of wisespread skin slecrosis affecting trunk and entire limbs 1 year after raynauds

49
Q

what immunology and onset is seen in limited sclerosis?

A

anti-centromere

onset of skin fibrosis limited to distal limbs year after raynauds

50
Q

what are the classic features of limited sclerosis?

A
CREST + P
calcinosis - calcium deposits in fingers 
raynauds 
esophageal dysfunction (acid reflux)
sclerodactyly (thinking of skin)
talagiectasia
pulmonary hypertension
51
Q

how is sclerosis managed?

A

annual ECHO and PFT (due to pulmonary hypertension)
control BP - ACE inhibitor
immunosuppression - cyclophosphamide
(steroids may cause hypertensive crisis)

52
Q

what is mixed connective tissue disease?

A

features of SLE, systemic sclerosis and polymyositis

53
Q

what immunology is seen with mixed connective tissue disease?

A

anti-RNP

54
Q

what is 1st line drug treatment for raynauds?

A

calcium channel antagonist - nifedipine