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Pedi Rheum Boards > Periodic Fever > Flashcards

Periodic Fever Flashcards

1
Q

FMF mechanism of disease

A

Activation of pyrin inflammasome; IL-1beta up

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2
Q

TRAPS mechanism of disease

A

Misfolding of mutant protein; unfolded protein response

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3
Q

HIDS mechanism of disease

A

Defect in geranyl-geranylation of RhoA resulting in activation of pyrin inflammasome; IL-1beta up

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4
Q

FCAS/MWS/NOMID mechanism of disease

A

Constitutive activation of the NLRP3 inflammasome: IL-1beta up

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5
Q

PAPA mechanism of disease

A

Constitutive activation of the pyrin inflammasome; IL-1beta up

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6
Q

DADA2 mechanism of disease

A

Lack of antiinflammatory (M2) macrophages; TNF & IL-1 up

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7
Q

SCAN4 mechanism of disease

A

Activation of the NLRC4 inflammasome; IL-1beta & IL-18 up

Syndrome of enteroColitis and auto-inflammation NLRC 4

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8
Q

HA20 mechanism of disease

A

Defect in hydrolysis of Lys63-linked ubiquitin chains; NF-kappaB up, multiple cytokines

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9
Q

CANDLE mechanism of disease

A

Defects in proteasome assembly; type 1 interferon up

Chronic atypical neutrophilic dermatosis with lipodystrophy and elevatedtemp

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10
Q

SAVI mechanism of disease

A

Constitutive activation of STING; type 1 IFN up

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11
Q

Monoarthritis, peritonitis, erysipelas-like rash

A

FMF

Gene/Protein
MEFV/pyrin

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12
Q

Migratory myalgia and erythema, periorbital edema

A

TRAPS

Gene/Protein
TNFRSF1A/“

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13
Q

Cervical adenopathy and aphthous ulcers

A

HIDS

Gene/Protein
MVK/“

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14
Q

Cold induced urticarial like rash

A

FCAS

NLRP3

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15
Q

Sensorineural hearing loss

A

MWS

NLRP3

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16
Q

Aseptic meningitis and arthropathy

A

NOMID

NLRP3

17
Q

Scarring cystic acne, PG, and pyogenic sterile arhritis

A

PAPA

PSTPIP1

18
Q

Ischemic lacunar strokes

A

DADA2

ADA2

19
Q

Recurrent MAS, cold-induced fever

A

SCAN4

Enterocolitis and autoinflammation

NLRC4

20
Q

Fluctuating presence of autoantibodies

A

HA20

TNFAIP3 (A20)

21
Q

Myositis, lipodystrophy, lymphadenopathy

A

CANDLE

PSMB8

22
Q

Interstitial lung disease

A

SAVI

Gene/Protein
TMEM173/STING

Pedi Rheum Boards (4 decks)

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