autoimmune disorders Flashcards

1
Q

what type of hypersensitivity is associated with SLE ?

A

Type 3

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2
Q

what are the serological tests used for the identification of SLE ?

A

ANA non specific
Anti DsDNA specific
Anti smith

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3
Q

what is anti smith directed against ?

A

snRNP

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4
Q

what are all the penias in SLE due to ?

A

type 2 hypersensitivity reaction

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5
Q

which type of lupus nephropathy is the most common type in SLE ?

A

diffuse proliferative GN
which presents as nephritic syndrome

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6
Q

what type of endocarditis is associated with SLE ?

A

libman sacks endocarditis

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7
Q

what is very distinctive about libman sacks endocarditis ?

A

affects both sides of the valve

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8
Q

what are the three clinical consequences of antiphospholipid antibody ?

A

antiphospholipid syndrome
increased PTT
false positive syphillis

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9
Q

what are the three types of antiphospholipid antibodies ?

A

anti cardiolipin
lupus anticoagulant
anti b2 glycoprotein

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10
Q

what other disease is associated with anti-cardiolipin ?

A

syphillus

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11
Q

which antibody is associated with a false elevation of PTT ?

A

lupus anticoagulant

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12
Q

which antibody is associated with drug induced lupus ?

A

anti histone ab

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13
Q

which drugs are associated with drug induced lupus ?

A

isoniazid
hydralaziine
procinamide

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13
Q

what is the pathophysiology in rheumatoid arthritis ?

A

inflammation of the synovium due too TNF and IL 6
Type 3 HS
causing the formation of pannus

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14
Q

what is the classical presentation of RA ?

A

symmetrical joint inflammation
MCP
PIP
but the DIP is spared
Ulnar deviation
Swan Neck deformity - Hyperextended PIP , hyper flexed DIP

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15
Q

what antibodies are associated with RA ?

A

RF ( seroo positive)
anti-ccp

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16
Q

whats the target for RF ?

A

Fc portion of IgG Ab

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17
Q

what HLA is associated with RA ?

A

HLA-DR4

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18
Q

what are the long term complications of RA ?

A

osteoporosis
increased risk of coronary heart disease
secondary amyloidosis

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19
Q

what drugs are used for the treatment of RA ?

A

steroids
NSSAIDS
DMARDS

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20
Q

what is the function of infliximab ?

A

a monoclonal antibody which is directed against TNF alpha

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21
Q

what is the problem with the se of infliximab ?

A

there is a risk of reactivation off TB so a PD test must be done before administration

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22
Q

what is thee function of etanercept ?

A

acts as tumor necrosis factor inhibitor

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23
Q

what is felty syndrome ?

A

rare complication of RA
splenomegaly
anemia
thrombocytopenia
arthiritis

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24
Q

what is thee pathophysiology of scleroderma ?

A

fibroblast activation leading to excess collagen deposition

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25
Q

what are the 2 clinical syndromes associated with scleroderma ?

A

diffuse
limited CREST

Calcinosis
Raynauds
Oesophageal motility
sclerodactyly
telengectasia

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26
Q

what is the main risk associated with limited scleroderma ?

A

pulmonary disease

27
Q

how is renal crisis in association with diffuse scleroderma treated ?

A

ACE inhibitor

28
Q

what are the antibodies associated with diffuse scleroderma ?

A

anti scl 70
which is ant topoisomerase 1
anti RNA polymerase II

29
Q

which antibody is associated with limited scleroderma ?

A

anti centromere

30
Q

if a patient with scleroderma starts presenting with fatigue itching and jaundice what is the most likely diagnosis ?

A

primary biliary cirrhosis

31
Q

what are the antibodies associated with sjogren syndrome ?

A

anti ro
anti la

32
Q

biopsy off salivary gland off patient with sjogren ?

A

lymphocytic sialadenitis

33
Q

how do we treat sjogren ?

A

muscarinic agonists

34
Q

what is the test for thee sicca symptoms of sjogren ?

A

schrimer test
salivary gland scintigraphy

35
Q

if a patient with sjogren presents with persistent unilateral swelling what is this a sign off ?

A

most probably a b cell lymphoma

36
Q

what are the large vessel vasculitis ?

A

temporal arteritis
takayasu arteritis

37
Q

what are the medium vessel vasculitis ?

A

polyarteritis nodosa
kawasaki disease
beurgers disease

38
Q

what are the small vessel vasculitis ?

A

churg strauss
wegners granulomatosis
microscopic polyangitis
henoch- schonlein purpra

39
Q

what is the main symptom associated with vasculitis ?

A

palpable purpra
do not blanch on pressure

40
Q

what are thee consequences of untreated temporal arteritis ?

A

blinndness due to affection of the occcular artery

41
Q

where is the affection in takayasu arteritis ?

A

aortic arch with granulomatous thickening

42
Q

what is thee presentation of takayasu arterritiis ?

A

weak unilateral pulses
BP difference between arm and legs

43
Q

what is thee presentation in kawasaki disease ?

A

young asian kids
strawberry tongue

44
Q

what is the feared complication in kawasaki disease ?

A

coronary aneurysm

45
Q

what is the treatment for kawasaki ?

A

IVIg
aspirin

46
Q

what disease is commonly confused with kawasaki diseease ?

A

scarlet disease
but the difference is that thee patent present with a sore throat before thee presentation of the strawberry tongue

47
Q

what is thee presentation of reye syndrome ?

A

encephalopathy
confusion
often coma
after the administration of reye syndrome

48
Q

what group is more liable to get beurgers disease ?

A

male smokers
thromboangitis obliterans

49
Q

what disease is associated with polyarteritis nodosa ?

A

hepatitis B

50
Q

what is the pathophysiology off polyarteritis nodosa ?

A

type 3 Hypersenitivty

51
Q

what sign is oft3en seen with polyarteritis nodosa ?

A

rosary sign seen on arteriogram

52
Q

what is the other name for pANCA and ANCA ?

A

pANCA - PR3
cANCA - MPO

53
Q

which vasculitis is pANCA and which are cANCA ?

A

cANCA - Wegner
pANCA - Churg Straus
microsccopic polyangitis

54
Q

what is thee presentation of churg strauss ?

A

asthma for thee first time as an adult
bizarre presentation of asthma
sinusitis and neuropathy

55
Q

what labs are associated with churg strauss ?

A

high eosinophils
high IgE
p-ANCA

56
Q

what is the presentation off wegners granulomatosis ?

A

triad off hemoptysis
sinusitis
otitis media

inflammation of the upper airway and kidney involvement

57
Q

what is thee classic case of polyarteritis nodosa ?

A

a patient with hep b that starts presenting with neuropathy and renal failure

58
Q

what is seen on biopsy of polyarteritis nodosa ?

A

fibrinoid necrosis

59
Q

what is the pathophysiology fo henoch schonlein purpra ?

A

IgA associated nephropathy
with C3 complement deposition

60
Q

what i9s the classic presentation of henoch scchonlein purpra ?

A

childhood vasclitis
URI
Melena

61
Q

what do all ANCA diseases have in common ?

A

they all have some form of lung and renal involvement
the renal involvement is cresenteric RPGN

62
Q

what is seen on IF in cresenteric RPGN ?

A

paucimmune ( lack of Ig )

63
Q

what is the presentation off microscopic polyangitis ?

A

just like wegner but no upper airway affection

64
Q

what is the pathophysiology of goodpasteur syndrome ?

A

type 2 hypersenitivity
anntibodyy too collagen type 4
anti basement membrane
immunoflurosence is positive