Autoimmune Basics Flashcards

1
Q

What is an Autoimmune Disease?

A

Immune reaction directed against a self antigen that is responsible for the pathologic condition

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2
Q

Autoimmune diseases can be caused by what type of things?

A

Genetics
Environment
- Infection = Molecular mimicry
- Damaged tissues = Epitope spreading

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3
Q

What are the autoimmune diseases we discussed?

A

Systemic Lupus Erythematosus
Sjogren Syndrome
Systemic Sclerosis
IgG4-related disease

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4
Q

What is normal immunologic tolerance?

A

Tolerating self antigens

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5
Q

When is central tolerance learned and where does it take place?

A

It is learned before lymphocyte release from the generative organs - at the thymus and bone marrow

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6
Q

If T cells cross-react with self antigens in the thymus, what happens to them?

A

Apoptosis

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7
Q

If B cells recognize self antigens in the bone marrow, what happens to them?

A

Receptor editing OR Apoptosis

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8
Q

What 2 things control peripheral tolerance?

A

T regulatory cell suppression

Anergy

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9
Q

What receptors on the T cell are engaged to induce T cell anergy (no response)?

A

PD-1

CTLA

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10
Q

What is anergy?

A

Failure/downregulation of T cell response when their inhibitory ligands (CTLA and PD-1) are engaged

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11
Q

What autoimmune disease is associated with HLA-B27?

A

Ankylosing Spondylitis

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12
Q

What are the symptoms with Ankylosing Spondylitis and what gene is it associated with?

A
  • Associated with HLA-B27

= Spinal inflammatory condition that causes degeneration and fusion of the vertebra

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13
Q

Degeneration and fusion of the vertebrae?

A

Ankylosing Spondylitis

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14
Q

What autoimmune disease is associated with PTPN22 variants?

A

Rheumatoid Arthritis

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15
Q

Rheumatoid Arthritis is associated with variants of what gene?

A

PTPN22

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16
Q

PTPN22 stands for?

A

Protein Tyrosine Phosphatase

17
Q

Variants of PTPN22 (rheumatoid arthritis) directly cause?

A

Decreased B cell and T cell self tolerance!

18
Q

What autoimmune disease is associated with NOD-2 variants?

A

Crohn’s Disease

19
Q

Crohn’s Disease is associated with variants of what gene?

A

NOD-2

20
Q

What do NOD-2 variants do?

A

Render intestinal Paneth cells ineffective at microbial killing
= Accumulation of bacteria

21
Q

This gene variant renders intestinal Paneth cells ineffective, which results in accumulation of bacteria and an exaggerated immune response

A

NOD-2 – Crohn’s Disease

22
Q

Describe Molecular Mimicry

A

Antigenic similarity between true pathogens are native tissues

23
Q

What is an example of molecular mimicry that leads to an autoimmune disease?

A

Rheumatic Heart disease

  • Streptococcal Ag looks like Myocardial Ag
    • Antibodies attack Myocardial Ag
24
Q

What is an example of Epitope spreading with damaged tissues?

A

Oral Lichen Planus

25
Q

Describe Epitope spreading with Oral Lichen Planus

A
  • T cell response leads to lesions in oral mucosa
  • Basement membrane disruption exposes more antigens (new)
  • B cell response occurs to those antigens
26
Q

What is an ANA test?

A

Anti-Nuclear Antibody

- If (+) could indicate a non-specific autoimmune disease

27
Q

A (+) ANA test will prompt?

A

More specific testing to figure out which autoimmune disease may be present

28
Q

Systemic Lupus Erythematous has what Antibodies?

A

Anti-DS DNA

Anti-Smith

29
Q

How does SLE stain?

A

Homogenous

30
Q

Sjogren has what Antibodies?

A

Anti-RO/ss-B

Anti-LA/ss-A

31
Q

How does Sjogren stain?

A

Speckled

32
Q

Systemic Sclerosis has what Antibodies?

A

Anti-DNA Topoisomerase

Anti-Scl-70

33
Q

How does Systemic Sclerosis stain?

A

Speckled or Nucleolar

34
Q

How does CREST syndrome stain?

A

Centromere

35
Q

Non-specific test for an Autoimmune disease?

A

ANA

Anti-Nuclear Ab