autoimmunity/Autoinflammatory Flashcards

1
Q

Components of the inflammasome complex

A

ASC + procaspase 1 -> caspase 1 -> IL-1 + HK-kB + apoptosis

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2
Q

+ve and -ve stimulants for the inflammasome complex

A

cryopyrin = + ve (inc ASC prod)

pyrin marenostrin = -ve

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3
Q

MEFV mut

A

Familial med fever

mut -> dec pyrin marenostrin -> inc inflammasome complex

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4
Q

fam med fever - inc risk of what

A

inc risk AA amyloidosis

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5
Q

most common cause of fever that comes and goes

A

subacute inf endocarditis (not FMF)

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6
Q

tx for FMF

A

colchicine
anakira (anti-IL-1R)
TNF alpha inhib

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7
Q

hypopara, addisons, chronic thrush inf

AIRE defect

A

APS-1/APECED

defect -> dec apoptosis of autoreg t cells -> failure of central tolerance

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8
Q

diarrhoea, T1DM, dermatitis, hypothy

mut in foxp3

A

IPEX

x-linked mut to foxP3 -> dec Treg dev -> failure of periph tolerance

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9
Q

v high, double neg lymphocytes

splenomeg, lymphadenopathy, cytopenias

A

ALPS

mut in FAS path -> dec apoptosis of lymphocytes

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10
Q

inc risk of what in ALPS

A

lymphomas

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11
Q

NOD2/CARD15 mut

A

-> inc risk crohns

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12
Q

tx for crohns

A

azathioprine, steroids, anti-TNFa, anti-IL12/23

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13
Q

lower back pain in the morning that gets better as you move about
strong FHx
HLAB27+

A
ank spond (seroneg) 
-> enthesitis (inflam where tendons join bone)
sacroilitis
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14
Q

Tx for ank spond

A

NSAIDs, physio, anti-TNFa, Anti-IL17

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15
Q

HLADR-15 allele

A

inc risk goodpastures

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16
Q

HLADR-3

A

inc risk graves, SLE, T1DM

17
Q

HLADR-4

A

RA

18
Q

effect of PYPN22 or CTLA4 mut

A

dec periph tolerance -> polygenic autoimmune

19
Q

anti-GAD abs

anti-islet cell

A

T1DM (T1/4)

20
Q

antibodies in pernicious anaemia (T2)

A

anti-IF = most specific

anti-gastric parietal cell = most sensitive

21
Q

anti-nAChR ab

A

myaesthenia gravis (T2)

22
Q

ab = anti-basement memb of Type IV collagen

A

Goodpastures (T2)

23
Q

most specific ab in rheumatoid arth

A

anti-ccp abs

24
Q

anti-dsDNA

A

v specific for SLE

diag + monitor

25
Q

anti-Ro/La

A

Sjogrens

26
Q

anti-Scl170

A

diffuse scleroma

27
Q

anti-centromere

A

limited scleroma

28
Q

what can be used to monitor SLE

A

anti-dsDNA

C3/C4 levels

29
Q

long APTT
anti-cardioliptin ab
recurrent venous/arterial thrombosis

A

anti-phospholipid syndro

-> also recurrent miscarriages

30
Q

what mediates the inflam in systemic sclerosis

A

Th2/Th17

31
Q

muscle weakness, skin rash

anti Jo1

A

dermatomyositis

32
Q

muscle weakness

anti SRP

A

polymyositis

33
Q

classification of vasculitis

A

Chapel hill clasification