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MICR2221 - Medical Immunology > L7 - Autoinflammatory disease > Flashcards

L7 - Autoinflammatory disease Flashcards

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USMLE® Step 1 flashcards
1
Q

What are auto-inflammatory disease?

A

heterogenous group of inherited disorders

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2
Q

what is there an absence of in auto-inflammatory diseases?

A

high-titre antibodies

antigen specific auto-reactive T cells

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3
Q

What are some Autosomal DOMINANT hereditary periodic fevers?

A

TRAPS

MWS

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4
Q

What are some Autosomal RECESSIVE hereditary periodic fevers?

A

DIRA

DITRA

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5
Q

What causes the persistence of triggers in auto inflammatory disease?

A

Dysregulated cell activation/feedback

lack of counter-regulatory pathways

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6
Q

What auto-inflammatory diseases are caused by issues with IL-1beta?

A

CAPS

DIRA

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7
Q

What auto-inflammatory diseases are caused by issues with IL-36?

A

DITRA

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8
Q

What is receptor antagonist?

A

regulation

high receptor antagonist = competitive blocking of receptor

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9
Q

What is DIRA?

A

Deficiency of IL-1RA

sterile multifocal osteomyelitis

periotstitis

pustulosis

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10
Q

What is used to treat DIRA?

A

Anakinra

Injection IL-1RA

daily injections

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11
Q

What are an important source of IL-1?

A

Keratinocytes

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12
Q

What cells are important responders to IL-1?

A

T-cells

upregulate the adaptive immune response

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13
Q

What is gout?

A

urate crystals at joints

commonly big toe joint

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14
Q

What is psoriasis?

A

scaly red plaques
itchy
infiltration of lymphocytes and inflammatory cells into the epidermis

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15
Q

What IL is strongly up-regulated in psoriatic lesions?

A

IL36y

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16
Q

What does IL-36 induce?

A

IL-36 induces IL-8 in FIBROBLASTS and KERATINOCYTES

IL-8 attracts neutrophils

17
Q

Is IL-36 active in its full length?

A

NO

No production of IL-8

18
Q

What is the Koebner phenomenom?

A

psoriatic skin triggered by microtrauma or irritation

19
Q

What is DITRA?

A

Deficiency in IL-36 antagonist

without RA, binding of accessory protein cannot occur

20
Q

What is pustular psoriasis?

A

skin function impaired

electrolyte disturbance due to skin loss

21
Q

What is the importance of processing in IL-1 cytokines?

A

require post translational cleavage for activation

22
Q

What does IL-36y also require for activation?

A

N- terminal truncation

23
Q

What is the molecule that cleaves IL-36?

A

Cathepsin S

MICR2221 - Medical Immunology (16 decks)

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