Autoimmunity and Autoimmune Diseases Flashcards

1
Q

Genetic factors associated with autoimmune disease?

A

Familial Incidence

Association with Specific HLA/MHC Haplotypes

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2
Q

Origins of autoimmune disease?

A
Genetics
Failure to maintain self-tolerance
Loss of Treg
Expression of cryptic self epitopes
Inappropriate MHC II molecules
Antigen mimicry
Polyclonal B activation
Infectious Diseases
Hormonal influences
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3
Q

Autoimmune hemolytic anemia.

Rxn Type, Autoantigen and Consequence?

A

Type II
Rh blood group antigens
RBC destruction, Anemia

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4
Q

Autoimmune thrombocytopenia purpura.

Rxn Type, Autoantigen and Consequence?

A

Type II
Platelet Integrin (gpIIb:IIIa)
Abnormal Bleeding

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5
Q

Goodpasture’s Syndrome.

Rxn Type, Autoantigen and Consequence?

A

Type II
Non-collagenous basement membrane, Type 4 collagen
Glomerulonephritis, Pulmonary Hemorrhage

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6
Q

Pemphigus Vulgaris.

Rxn Type, Autoantigen and Consequence?

A

Type II
Epidermal Cadherin
Blistering

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7
Q

Pemphigus foliaceus.

Rxn Type, Autoantigen and Consequence?

A

Type II
Desmoglein
Mild Blistering

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8
Q

Acute rheumatic fever.

Rxn Type, Autoantigen and Consequence?

A

Type II
Strep Wall antigens, cross react w/ cardiac
Arthritis, Myocarditis, Late Scarring of Heart

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9
Q

Grave’s Disease.

Rxn Type, Autoantigen and Consequence?

A

Type II
TSH receptor
Hyperthyroidism

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10
Q

Myasthenia Gravis.

Rxn Type, Autoantigen and Consequence?

A

Type II
Acetylcholine Receptor
Progressive weakness

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11
Q

Type II Diabetes.

Rxn Type, Autoantigen and Consequence?

A

Type II
Insulin Receptor
Hyperglycemia, Ketoacidosis

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12
Q

Subacute bacterial endocarditis.

Rxn Type, Autoantigen, and Consequence?

A

Type III
Bacterial Antigen
Glomerulonephritis

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13
Q

Mixed essential cryoglobulinemia.

Rxn Type, Autoantigen, and Consequence?

A

Type III
Rheumatoid Factor IgG complexes
Systemic Vasculitis

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14
Q

Systemic Lupus Erythematosus.

Rxn Type, Autoantigen, and Consequence?

A

Type III
DNA, Histones, Ribosomes, snRNPs
Glomerulonephritis, Vasculitis, Arthritis

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15
Q

Type I Diabetes.

Rxn Type, Autoantigen, and Consequence?

A

Type IV
Pancreatic beta cell antigen
beta cell destruction

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16
Q

Rheumatoid arthritis

Rxn Type, Autoantigen, and Consequence?

A

Type 4
Unknown synovial joint antigen
Joint inflammation, destruction

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17
Q

MS

Rxn Type, Autoantigen, and Consequence?

A

Type 4
Myelin basic protein, proteolipid protein
Brain degeneration, Paralysis

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18
Q

How do we normally prevent autoimmunity?

A

Positive/Negative Selection of B and T cells
Regulatory CD4CD25 T Cells
Physical Separation
Limited expression of MHC II and B7

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19
Q

Examples of physically separated from lymphocytes?

A

Brain, Eye, Testis

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20
Q

How are autoimmune cells repressed by Tregs?

A

IL10, TGFbeta

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21
Q

Five Major Susceptibility Factors in Autoimmune Disease?

A
HLA Genotype/Genetic Background
Microbial Infection
Injury
Environmental Factors/Behavior
Gender and Sex hormones
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22
Q

How might microbial infection promote autoimmunity

A

Adjuvant effect
Induction of higher MHC and B7
Molecular Mimicry

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23
Q

How might injury lead to an autoimmune response?

A

Reveals antigens from a previously immune priveledged organ

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24
Q

Environmental factors that affect autoimmune disease?

A

Smoking and hygiene

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25
Q

Which gender is more prone to autoimmune concerns?

A

Women

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26
Q

Describe the pathogenesis of Sympathetic Opthalmia

A

Injury in one eye releases eye antigen into local lymph nodes
Immune responses to antigen affect both eyes
Blindness in both damaged and undamaged eye

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27
Q

What does AIRE do?

A

Allows expression of peripheral antigen in the thymus

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28
Q

Mutation of AIRE leads to….

A

Wide range of autoimmune diseases

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29
Q

Mutations in AIRE are characterized by….

A

Autoimmune
PolyEndocrynology
Candidiasis
Ectodermal Dystrophy

30
Q

What is IPEX?

A

Mutation of FOXP3
Loss of generation of Tregs
Tregs can’t suppress activation of other immune cells

31
Q

Describe the relationship of HLA allotypes with aytoimmune disease

A

Some unique HLA allotypes are very heavily assoicated with specific disease states compared to their prevalence in the control population

Ex. B27 HLA is present in 9% of people, but more than 95% of patients with ankylosing spondylitis have it

32
Q

What is Celiac disease?

A

Wheat flour gluten peptide-specific CD4+ T cells
Later anti-transglutaminase autoantibodies
Destruction of villi structure

33
Q

How does Celiac Happen

A
Gluten degraded in lumen to resistant fragment
Fragment enters gut tissue
Deaminated by transglutaminase
Native CD4 t cell responds via HLA-DQ
Inflammatory T cell response
34
Q

What happens in molecular mimicry?

A

Antibodies to pathogens cross-react against host antigens

35
Q

Group A Strep

HLA Association? Consequence?

A

Not Known

Rheumatic Fever

36
Q

Chlamydia trachomatis

HLA Association? Consequence?

A

HLA-B27

Reiter’s Syndrome (arthritis)

37
Q

Campylobacter jejuni

HLA Association? Consequence?

A

HLA-B27

Reactive Arthritis

38
Q

Borrelia Burgdorferi

HLA Association? Consequence?

A

HLA-DR2, DR4

Chronic Arthritis in Lyme Disease

39
Q

Cocksackie A and B

HLA Association? Consequence?

A

HLA DQ2, DQ8, DR4

Type I Diabetes

40
Q

Mechanism of induced autoimmune thyroid disease?

A

IFNg from infection/inflammation induces MHC II
Activated T cells recognize thyroid peptides
Induce Autoimmune thyroid disease

41
Q

How does the thyroid typically avoid immune response?

A

Thyroid does not normally express HLA molecules

42
Q

What is autoimmune hemolytic anemia (type 2)

A

Erythrocytes bind anti-RBC autoantibodies

Destruction through phagocytosis or completment

43
Q

How do you treat autoimmune hemolytic anemia (type 2)

A

Splenectomy to reduce Blood cell loss

44
Q

What happens in Immune Thrombocytopenic Purpura (ITP)

A

Antibodies attach to blood platelets for destruction

45
Q

What is Goodpasture’s syndrome?

A

IgG agains alpha3 chain of type 4 collagen in BM

Renal Glom is most sensitive to the antibody deposits/inflam

46
Q

HLA types associated with Type I diabetes?

A

HLA-DR3, DR4

47
Q

Grave’s disease is associated with what HLA type?

A

HLA-DR3

48
Q

Difference in presentation of Hashimoto’s and Grave’s?

A

Hashi - Destroys Thyroid

Graves – Hyperproduction of thyroid

49
Q

Autoimmune disease of adrenal gland?

A

Addison’s

50
Q

Treatment for Grave’s disease?

A

Thyroidectomy or destruction of the gland by radioactive I

51
Q

Concern for pregnant women with Grave’s

A

Antibodies to TSHR can cross placenta
Newborn also suffers from Grave’s
Plasmaphoresis should cure the disease

52
Q

What happens in Hashimoto’s thyroiditis?

A

Antibodies against effector T cells specific for thyroid
Destroy Thyroid, loss of T3/T4 production
Th1 type disease

53
Q

Treatment for hashimoto’s

A

administration of thyroid hormone

54
Q

List three autoimmune liver/GI diseases

A

IBD
Pernicious Anemia
Autoimmune Chronic Active Hepatitis

55
Q

Autoimmune Chronic Active Hepatitis is assocaited with which HLA antigen?

A

HLA-B8/DR3

56
Q

Three autoimmune rheumatic diseases? HLA? Sensitivity?

A

Rheumatoid Arthritis - DR4 – (III and IV)
Systemic Lupus Erythematosus – DR2,3 – II and III
Polymyositis/Dermatomyositis – DR3, DR – IV

57
Q

Immediately apparent sign of systemic lupus

A
Wolf Erythematosus (Skin Rash)
Caused by antibodies to histone and DNA
58
Q

Two big problems with Systemic Lupus Erythematosus?

A

IgG binding to cell surfaces –> Inflammatory responses

Immune complexes deposited in vessels, kidneys

59
Q

Cause of rheumatoid arthritis?

A

Rheymatoid Factor = IgM/G/A against the Fc region of IgG

Leukocyte infiltration in the joint synovium

60
Q

What cells make rheumatoid factor?

A

Plasma B cells

61
Q

How is Rheumatoid arthritis treated?

A

Anti inflamamtory

Immunosuppressive drugs

62
Q

How does smoking affect Rheumatoid arthritis?

A

Smoking –> Injury –> Generation of Citruline Residues

Citruline residues –> CD4 T Cell Activation -> RA

63
Q

How can RA be treated with NK cells?

A

Add in an anti-CD20 antibody (Rituximab) to promote lysis by NK cells

64
Q

Four autoimmune neurologic diseases.

A

MS
Acute disseminated encephalomyelitis
Acute Inflammatory Polyneuropathy/Guillon Barre
Myasthenia Gravis

65
Q

MS is what type of hypersensitivity?

A

2 and 4

66
Q

Acute disseminated encephaloyelitis is what type of hypersensitivity?

A

4

67
Q

Guillon barre is what type of hypersensitivity?

A

II and IV

68
Q

Myasthenia gravis is is what type of hypersensitivity?

A

II

69
Q

T cells implicated in MS?

A

Th17 or Th1

70
Q

Cytokines associated with CNS inflammation and demyelination

A

IL-17, IFNg