Autoimmune disease

Question 1

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

Answer 1

FOXP3

development of regulatory T-cells

Question 2

What are the mechanisms responsible for maintaining self tolerance?

Answer 2

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

Question 3

Diversity of MHC proteins enables them to….

Answer 3

bind a wide range of antigens

Question 4

SLE is a Type __ hypersensitivity disease

Answer 4

Type III

-immune complex mediated

Question 5

What are the clinical features of SLE?

Answer 5

seizures
butterfly rash
discoid lupus
endocarditis, myocarditis

Question 6

What is the pathogenesis of SLE?

Answer 6

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

Question 7

IPEX syndrome is a ___genic disorder. IPEX stands for…

Answer 7

monogenic

Immune dysregulation
Polyendocrinopathy
Enteropathy
X-linked inheritance syndrome

Question 8

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

Answer 8

Type IV

T-cell

Question 9

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

Answer 9

CD4+

B cells

Question 10

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

Answer 10

TNF-alpha

IL-1