MedEd AI and vasculitides Flashcards

1
Q

what is sarcoidosis?

A

chronic multisystem granulamatous disorder of unknown cause

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2
Q

who is more likely to get sarcoidosis?

A

afro caribbeans

20-40 y/o females

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3
Q

what cells are present in sarcoidosis and what do they form?

A

T lymphocytes and macrophages which form non caseating granulomas

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4
Q

what granulmoas do you get in sarcoidosis? why

A

non caseating

there is no necrosis

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5
Q

how do granulomas differ between tb and sarcoidosis and explain why

A
tb= caseating because theres necrosis
sarcoidosis= caseating because there is no necrosis
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6
Q

how does acute sarcoidosis present?

A
fever
erythema nodosum
polyarthralgia
bilat hilar lymphadenopathy
dry cough with prgressive dyspnoea
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7
Q

what type of cough do you get in sarcoidosis?

A

dry cough with progressive dyspnoea

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8
Q

what are extrapulmonary features of sarcoidosis?

A
organomegaly 
uveitis
lupus pernio
subcut nodules 
arrhythmia 
symptoms of hypercalcaemia
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9
Q

what colour is lupus pernio and where is it found?

A

violet macular rash found on the face

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10
Q

in what resp condition is lupus pernio seen?

A

sarcoidosis

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11
Q

out of lupus pernio and lupus vulgaris which is present in tb and which in sarcoidosis?

A

lupus pernio= sarcoidosis

lupus vulgaris= tb

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12
Q

what ix are done for sarcoidosis and what will you see? which ix is diagnostic

A

bloods- high esr, lymphopenia, hypercalcaemia, raised ACE
cxr- bilateral hilar lymohadenopathy
diagnostic- bronchoscopy with biopsy

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13
Q

what are the cxr stages of sarcoidosis?

A

stage 1= BHL
stage 2= BHL with infiltrates
stage 3= only infiltrates
stage 4= extensive fibrosis

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14
Q

how is sarcoidosis managed?

A

conservation- most patients with stage 1 will spontaneously resolve
may give steroids- indications inc parenchymal lung disease, uveitis, hypercalcaemia, neurologic or cardiac involvement

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15
Q

what are some indication for giving steroids in sarcoidosis?

A

parenchymal lung involvement
uveitis
hypercalcaemia
neurologic or cradiac involvement

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16
Q

what tb abx induces lupus?

A

isoniazid

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17
Q

what is sle? describe what happens

A

a multisystemic relapsing remitting AI disease where a wrongful response to host antigens leads to immune complex formation. a lack of clearance of these complexes then causes tissue damage and inflammation

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18
Q

what is formed in SLE?

A

immune complexes

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19
Q

what type of hypersensitivity reaction is present in SLE?

A

type 3

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20
Q

what genes are associated with SLE?

A

HLA DR3

also HLA B8/DR2

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21
Q

what is the the trigger for formation of autoimmune complexes in SLE?

A

host antigens

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22
Q

how does SLE present?

A
malar rash
discoid rash
oral ulcers
arthritis 
serositis
renal disorder
hematologic disorders
immunological disorders 
antinuclear disoders
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23
Q

what drugs cause SLE?

A
sulfalazine
hydralazine 
isoniazid 
procainamide
penicillamine
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24
Q

what ix are done for SLE and what will you see?

A

bloods- anaemia, lymphopenia, thrombocytopenia
ESR high
CRP normal
autoantibodies: ANA, anti ds-DNA, anti histone
there may be lupus nephritis on renal biopsy

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25
Q

what are ESR and CRP levels in SLE?

A
ESR= raised 
CRP= normal
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26
Q

how is SLE managed?

A

maintanence= NSAIDs and hydrocychloroquine for joint and skin symptoms
mild flare= low dose steroids
moderate flaer= DMARDs or mycophenolate
severe flare= high dose steroids, mycophenolate, cyclophosphamide, rituximab

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27
Q

what is the difference between CRP and ESR and therefore when will either of them be raised?

A
CRP= rises when there is acute inflammation, ischaemia or thromboembolism
ESR= increases in a slower manner and then remains raised so with be high in chronic conditions
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28
Q

what does high CRP normal ESR indicate?

A

acute infection, inflammation or ischaemia

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29
Q

what does high ESR normal CRP mean?

A

chronic systemic inflammatory or autoimmune process

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30
Q

what autoantibodies are associated with SLE?

A

ANA

anti dsDNA

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31
Q

what is sjogrens syndrome?

A

chronic inflammatory disorder where lymphocytic infiltration destroys exocrine gland and causes extensive fibrosis

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32
Q

what glands are affected in sjogrens syndrome?

A

exocrine- mainly lacrimal and salivary

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33
Q

who is more likely to get sjogrens syndrome?

A

middle aged women

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34
Q

how does sjogrens syndrome present?

A
dry eyes- keratoconjucntivis sicca 
dry mouth- xerostomia 
parotid swelling 
polyarthritis 
raynauds phenomenon 
vasculitis 
peripheral neuropthy
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35
Q

what is keratoconjunctivits sicca? what condition is it seen in

A

dry eyes

seen in sjogrens syndrome

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36
Q

what is xeroztomia? what condition is it seen in

A

dry mouth

seen in sjogrens syndrome

37
Q

what ix are done for sjogrens syndrome? describe how they are carried out and what is positive

A

schrimers test- place a piece of paper under the eyelid for 5 mins and if less than 5mm of paper is wet in less than 5 mins this is positive
autoantibodies- anti ro and anto la (are not specific enough)
can do salivary gland biopsy

38
Q

what is schrimmers test? how is it carried out and which condition is it used for?

A

place a piece of paper under the eyelid for 5 mins and if less than 5mm of paper is wet in less than 5 mins this is positive for sjogrens syndrome

39
Q

how is sjogrens syndrome managed?

A

dry eyes= hypromellose (fake tears) and eye drops
dry mouth= salivary substitutes (sugar free gum)
severe disease= immunosupressants (steroids)

40
Q

what is systemic sclerosis? describe what happens

A

multisystemic autoimmune disease where there are functional and structural abnormalities of non inflammatory blood vessels, fibrosis of the skin and internal organs

41
Q

what is limited systemic sclerosis and how does it present?

A

systemic sclerosis that is limited to the hands, face and feet
presents with calcinosis cutis, raynauds, oesophageal dysmotility, sclerdactyly and telangiectasia

42
Q

what is diffuse systemic sclerosis and how does it present?

A

systemic sclerosis with widespread organ involvement

there is rapid progression and early visceral involvement and BP needs to be meticulously monitored

43
Q

how does systemic sclerosis present?

A

puffy taut skin with no wrinkles and finger tip pitting
renal- scleroderma hypertensive crisis
pulmonary- interstitial fibrosis, PH
GI- dysmotility, reflux and watermelon stomach
cardio- pericarditis or effusion

44
Q

what autoantibodies are associated with systemic sclerosis?

A

anti centromere in limited type

anti scl 70 and anti RNA polymerase III in diffuse type

45
Q

what type of systemic sclerosis are anti centromere antibodies associated with?

A

limited

46
Q

what type of systemic sclerosis are anti scl 70 and anti RNA polymerase III antibodies associated with?

A

diffuse

47
Q

how is systemic sclerosis managed?

A

immunosupression and treat individual symptoms

in renal crisis ACE-I or ARB with agressive hydration

48
Q

what are poly/dermatomyositis?

A

insidious onset progressive symmetrical proximal muscle weakness (quads, triceps, biceps) due to autoimmune mediated inflammation of straited muscle

49
Q

what muscles are weakened in poly/dermatomyositis?

A

proximal muscles like quads, biceps, triceps etc

50
Q

what cancer is dermatomyositis associated with?

A

ovarian

51
Q

what type of inflammation is present in polymyositis?

A

endomysial inflammation

52
Q

what type of inflammation is present in dermatomyositis?

A

perimysial

53
Q

what cells are involved with polymyositis?

A

CD8+ T cells

54
Q

what cells are involved with dermatomyositis?

A

CD4+ T cells

55
Q

what muscle group does polymyositis often affect?

A

shoulder girdle

56
Q

how does polymyolitis present?

A

progressive symmetrical muscle weakness

57
Q

how does dermatomyositis present?

A
progressive symmetrical muscle weakness 
grottons papules 
facial erythema 
shawl rash 
mechanics hands
58
Q

what are grottons papules?

A

rough red papules across knuckles, knees and elbows

59
Q

what is shawl rash?

A

shoulders and chest darkening

60
Q

what are mechanics hands in medicine?

A

dark and thick irregular patches

61
Q

what ix are done for poly/dermatomyositis and what will you see?

A

CK and adolase enzymes are elevated due to muscle breakdown
EMG shows fibrillation potentials
msucle biopsy to confirm diagnosis and visualise inflammation
autoantibodies; anti srp, anti mi2, anti jo1

62
Q

how are poly/dermatomyositis managed?

A

corticosteroids

63
Q

what is giant cell arteritis?

A

Large vessel granulomatous vasculitis

64
Q

what is polymyalgia rheumatica?

A

inflammatory condition which manifests as pain and morning stiffness in the neck, shoulder and hip girdle

65
Q

what vessel is commonly affected in giant cell arteritis?

A

external carotid

66
Q

who usually gets giant cell arteritis?

A

elderly females

67
Q

how does giant cell arteritis present?

A
unilateral headache
sclap tendrness
tongue/jaw claudication
amaurosis fugax
unilateral blidness
68
Q

what is amaurosis fugax?

A

vision gradually going away

69
Q

how does polymyalgia rheumatica present?

A
weakness is not a feature 
subacute onset (2 weeks) of bilateral aching, tenderness and morning stiffness
fever
weight loss
anorexia
70
Q

what is not a feature of polymyalgia rheumatica and helps distinguish it from other conditions?

A

weakness

71
Q

what ix are done for giant cell arteritis and what do you see/

A

raised ESR
raised CRP
normocytic anaemia
temporal artery biopsy but only after management- will show focal granulomtous inflammation

72
Q

what ix are done for polymyalgia rheumatica and what will you see?

A

raised ESR

raised CRP

73
Q

in giant cell arteritis what do you do first and second?

A

first give steroids

second do ix like bloods

74
Q

how is giant cell arteritis managed?

A

high dose methylprednisolone if blindness is starting

prednisolone if suspected

75
Q

how is polymyalgia rheumatic managed?

A

give steroids immediately and there should be a response within a week
also give bone protection as steroids will be needed for at least 2 years

76
Q

in giant cell arteritis when is methyprednisolone given and when is normal prednisolone given?

A

methylprednisolone= given if blindness has started

normal prednisolone= give if disease is suspected

77
Q

what is bechets disease?

A

systemic inflammatory vasculitis

78
Q

what gene is bechets disease associated with?

A

B51

79
Q

where is bechets disease found and who is most likely to have it?

A

old silk road along mediterrean to china

most likely a turkish male

80
Q

how does bechets disease present?

A
recurrent oral ulcers 
genital ulcerations 
erythema nodosum 
thrombophlebitis 
uveitis
81
Q

what ix are done for bechets disease and what will you see?

A

usually diagnosis is clinical

can also perform pathology testing by sticking a needle into their arm and a papule will form in 48 hrs

82
Q

how is bechets disease managed?

A

topical steroids for orogenital ulceration

prednisolone plus immunosupressant (azathioprine) if there is organ involvement

83
Q

what is polyarteritis nodosa?

A

necrotising inflammation of medium sized arteries

aneurysms lead to thrombosis and infarction

84
Q

what virus can cause polyarteritis nodosa?

A

HBV

85
Q

how does polyarteritis nodosa present?

A

systemic features
skin- rash, punched out ulcers, nodules
renal- artery narrowing, ischaemia and hypertension
livedo reticularis

86
Q

what is livedo reticularis? what does it look like? in what condition is it seen?

A

a skin symptom where there is mottled appearance of blood vessels often on the leg
can be seen in polyarteritis nodosa

87
Q

what ix are done for polyarteritis nodosa and what is seen?

A

FBC- high WCC
HBV serology
ESR and CRP high
MRA- shows string of pearls appearance due to micoraneurysms and spasm
renal biopdy might show transmural inflammation and necrosis

88
Q

how is polyarteritis nodosa managed?

A

prednisolone and if its severe then a DMARD