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MSK > multi system autoimmune disease > Flashcards

multi system autoimmune disease Flashcards

1
Q

what are examples of connective tissue diseases ?

A 
SLE
scleroderma
Sjorgen's syndrome
auto-immune myositis
mixed connective tissue disease
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2
Q

what are examples of systemic vasculitis ?

A

giant cell arteritis
granulomatosis polyangitis - Wegener’s
microscopic polyangitis
eoisinophillic granulomatosis polyangitis - Churg-Strauss

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3
Q

how are autoimmune diseases diagnosed ?

A 
cardinal clinical features 
immunology
imaging
tissue
exclusion of differentials
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4
Q

what can mimic an autoimmune disease ?

A 
drugs - coke, minocyline, PTU
infection - HIV, IE, hep, TB
malignancy - lymphoma
cardiac myxoma
cholesterol emboli
scurvy
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5
Q

who gets SLE ?

A

young people
F9:1M
afro-caribbeans>asians>whites

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6
Q

name any 4 classification criteria for SLE (diagnostic) ?

A 
malar rash - butterfly
discoid rash - alopecia, scarring
photosensitivity
oral ulcers
arthritis
serositis - pleurisy, pericarditis
renal - proteinuria, casts
neurological - seizures, psychosis
haematology - low stuff, haemolytic anaemia
immunity - low shit
ANA
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7
Q

who gets scleroderma ?

A

F3:1M

30-50

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8
Q

what are features of scleroderma ?

A 
morphea - grey blotches abdo
raynauds syndrome
RA
tight skin
digital ulcers
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9
Q

what are complications of scleroderma ?

A

limited - pulmonary hypertension

diffuse - pulmonary fibrosis, renal crisis, small bowel bacterial overgrowth

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10
Q

what are features of Sjogren’s syndrome ?

A

dry eyes and mouth
parotid gland enlargement
1/3rd have systemic upset - fatigue, fever, myalgia, arthralgia

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11
Q

what are complications of Sjogren’s syndrome ?

A 
lymphoma
neuropathy
purpura
interstitial lung disease
renal tubular acidosis
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12
Q

what are features of auto-immune myositis ?

A

rare
muscle weakness - symmetrical, diffuse, proximal

polymyositis
dermatomyositis - Gottron’s papules, heliotrope rash

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13
Q

what are complications on auto-immune myositis ?

A

cancer

interstitial lung disease

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14
Q

what are overlap syndromes ?

A

soft tissue swelling
raynauds
myositis
arthralgia

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15
Q

what are the large vessel vasculitides ?

A

Takayasu arteritis

giant cell arteritis

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16
Q

what are the medium vessel vasculitides ?

A

polyarteritis nodosa

kawasaki disease

17
Q

what are the small vessel vasculitides ?

A

ANCA - associated

18
Q

what are the diagnostic criteria for GCA ?

A 
3 of;
age 50+
new headache
temporal artery tenderness/reduced pulsation
ESR 50+
abnormal temporal biopsy
19
Q

what are features of granulomatosis with polyangitis (Wegener’s) ?

A

necrotising granulomatous inflammation
involves upper and lower respiratory tract
small to medium vessels
necrotising glomerulonephritis is common

20
Q

what are features of microscopic polyangiitis ?

A

necrotising polyangitis, few or no immune deposits, small vessels
necrotising arteritis and small and medium vessels
necrotising glomerulonephritis is very common
pulmonary capillarities common
granulomatous inflammation absent

21
Q

what are features of eosiniphillic granulomatosis with polyangitis (Churg Strauss) ?

A

eosinophil rich and necrotising granulomatous inflammation involving respiratory tract
necrotising vasculitis predominately involving small to medium vessels
associated with asthma
ANCA more frequent when glomerulonephritis present

22
Q

what are common consequences of ANCA associated vasculitides ?

A 
hearing loss
nerve damage
lung disease
end stage renal disease
malignancy
23
Q

what is the specific ANA profile for SLE ?

A

dsDNA
Ro
Sm

24
Q

what is the specific ANA profile for scleroderma ?

A

Scl-70

centromere

25
Q

what is the specific ANA profile for polymyositis ?

A

Jo-1

26
Q

what is the specific ANA profile for Sjogren’s disease ?

A

Ro

La

27
Q

what are the different classes of lupus nephritis ?

A 
1 - minimal mesangial
2 - mesangial proliferative
3 - focal 
4 - diffuse
5 - membranous
6 - advanced sclerosing
28
Q

how do you treat mild autoimmune disease ?

A

hydroxychloroquine

29
Q

how do you treat moderate autoimmune disease ?

A

azathioprine
methotrexate
mycophenolate

30
Q

how do you treat severe autoimmune disease ?

A

cyclophosphamide

rituximab

MSK (29 decks)

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