Autoimmunity (Denzin)

Question 1

IPEX (Immune dysregulation Polyendocrinopathy Enteropathy X-linked)

Answer 1

Caused by lack of FOXP3

• Associated with type I diabetes
• Diarrhea
• Failure to thrive
• Dermatitis
• DEATH in first 2 years of life

Question 2

APC/APCED

Autoimmune Polyglandular Syndrome/Autoimmune Polyendocrinopathy Candidias Ectodermal Dystrophy

Answer 2

APS/APCED patients have mutation in AIRE (autoimmune regulator) gene, makes transcription factor that causes ectopic expression of body proteins in thymus so T-cells can be exposed to non-thymus self antigens in development.

• Parathyroid failure, affecting Ca metabolism–>weird tooth enamel/nails
• Susceptibility to Candida (yeast) infection
• Addison’s Disease (adrenal failure)

Question 3

Molecular Mimicry

Answer 3

Pathogen expresses epitopes similar to self-antigens, causing activation of self-reactive T-cells, leading to autoimmunity

Question 4

Drug Hypersensitivity

Answer 4

Drug metabolite complexes with host protein, causing immune reaction and autoimmunity. Reversible when remove drug (since stop making complex)

Question 5

Abacavir Hypersensitivity Syndrome

Answer 5

In rare subset of people, Abacavir (HIV med, anti-retroviral) irreversibly binds MHC-1 peptide binding site. Causes normal peptide to bind differently, allows new ones to bind. This causes autoimmunity.

Question 6

Sifalimumab

Answer 6

anti-IFN-å antibody (Lupus treatment, stops immune activation and inflammation)

Question 7

Rituximab

Answer 7

• Lupus treatment, RA treatment

- Monoclonal antibody vs. CD-20 (on B-cells, so kills them)

Question 8

Tabalumab

Answer 8

• Lupus treatment

- Anti-BAFF antibody (B-cells need BAFF to survive)

Question 9

Etanercept, Infliximab, Adalumimab, Golimumab, Centolizumab

Answer 9

• Rheumatoid arthritis treatment

- anti-TNF antibodies

Question 10

Anakinra

Answer 10

• Rheumatoid arthritis treatment

- IL-1 inhibitor

Question 11

Tocilizumab

Answer 11

• Rheumatoid arthritis treatment

- Anti-IL-6 receptor antibody

Question 12

CTLA4 Fusion Protein

Answer 12

• Rheumatoid arthritis drug

- Binds B7 better than CD28 on T-cells, preventing their activation

Question 13

Secukinumab

Answer 13

• Rheumatoid arthritis treatment

- Anti IL-17 antibodies

Question 14

Treg Creation

Answer 14

Mature T-cell recognizing self antigen, becomes Treg via IL-2 and FoxP3 (transcription factor), goes on to suppress immune repsonse