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Flashcards in Autoimmune disease Deck (10)
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1
Q

What gene is mutated in IPEX syndrome? What is its role?

A

FOXP3

development of regulatory T-cells

2
Q

What are the mechanisms responsible for maintaining self tolerance?

A
  1. Central tolerance - removal of self-reactive lymphocytes in primary lymphoids tissues
  2. Peripheral tolerance - inactivation of self-reactive lymphocytes that escape central tolerance
    - mediated by Regulatory T cells
3
Q

Diversity of MHC proteins enables them to….

A

bind a wide range of antigens

4
Q

SLE is a Type __ hypersensitivity disease

A

Type III

-immune complex mediated

5
Q

What are the clinical features of SLE?

A

seizures
butterfly rash
discoid lupus
endocarditis, myocarditis

6
Q

What is the pathogenesis of SLE?

A

Infection/UV
1. apoptotic bodies form
2, apoptotic bodeis are not cleared by macrophages; they degrade
3. auto-antigens are released (from nucleus)
4. Immune complexes form

7
Q

IPEX syndrome is a ___genic disorder. IPEX stands for…

A

monogenic

Immune dysregulation
Polyendocrinopathy
Enteropathy
X-linked inheritance syndrome

8
Q

Rheumatoid arthritis is a Type _ hypersensitivity reaction. That means it is _____ mediated

A

Type IV

T-cell

9
Q

In rheumatoid arthritis, the synovium is infiltrated by self-reactive ___ T-cells. Activated ______ are consecutively involved.

A

CD4+

B cells

10
Q

What two pro-inflammatory cytokines play a key role in Rheumatoid Arthritis pathogenesis?

A

TNF-alpha

IL-1