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204 > Autoimmune Diseases 1 > Flashcards

Autoimmune Diseases 1 Flashcards

1
Q

What is tolerance?

A

A state of immunological non-reactivity to an antigen

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2
Q

Why is sarcoidosis and IBS not autoimmune?

A

The adaptive system does not react to self antigens

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3
Q

What is negative selection?

A

Too strong interaction between T cells and antigen presenting cells with peptide

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4
Q

Type 2 hypersensitivity is ?

A

Pathogenic autoantibodies

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5
Q

Autoimmune cytopenias?

A

Antibodies stuck to antigens on red cells, macrophages with fcr receptors will phagocytose or complement activation

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6
Q

What happens in graves thyroiditis?

A

Antibody against TSH receptor stimulating thyroid hormone release, so TSH levels go down

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7
Q

Signs of myasthenia gravis?

A

Ptosis at rest
Muscle weakness
Facial muscles talking and swallowing affected

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8
Q

Bells syndrome is?

A

Eyes turn inwards and upwards

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9
Q

What happens in myasthenia gravis molecular level?

A

Acetylcholine receptors degraded and internalised

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10
Q

Spontaneous urticaria is caused by?

A

IgG FcER1 antibody cross links mast cel receptor causing degranulation
Presents with hives and swelling

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11
Q

Type 4 hypersensitivity is caused by?

A

T cell dependent mechanisms because they activate other elements of innate immunity or damage tissue

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12
Q

T cell mediated autoimmune diseases?

A

Coeliac
Type 1 diabetes
Multiple sclerosis

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13
Q

Autoimmune polyglandular syndrome candiasis and ectodermal dystrophy?

A

AIRE gene regulates ectopic expression of tissue specific antigens in thymus,

AIRE mutations result in failure of negative selection

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14
Q

Why candiasis?

A

Prone to developing antibodies against IL 17, which is important for fungal defence

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15
Q

DiGeorge syndrome is due to?

A

Failure migration 3/4 brachial arches

Microdeletion of chromosome 22

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16
Q

A permissive negative selection?

A

Broad repertoire
Low risk of infection
High risk of autoimmunity

17
Q

Pemphigus?

A

Organ specific autoimmune disease with blisters and sores on skin or mucous membranes

18
Q

For coeliac which antibody would you check for?

A

Tissue transglutaminase antibody

19
Q

APACED is associated with organ specific diseases such as?

A

Type 1 diabetes, vitiligo, alopecia, autoimmune adrenal disease

20
Q

Phenotype of DiGeorge syndrome?

A

Absent parathyroids( low Ca, tetany),
Cleft palate
Congenital heart defects
Thymus aplasia

21
Q

IPEX, immune dysregulation, polyendocrinopathy, enteropathic, x linked?

A

FOXP3 gene

22
Q

Key features of IPEX?

A

IBD
Dermatitis
Organ specific autoimmunity

23
Q

HLA encoded on?

A

Chromosome 6

24
Q

HLA system

A

Class I: A,B C

Class II: DR, DP, DQ

25
Q

Coeliac prevalence?

A

1% in UK

26
Q

Sign of coeliac?

A 
Loose stool
Weight loss
 Vit deficiency 
Anaemia
Poor growth in children
27
Q

Coeliac disease microscope

A

Total villus atrophy
Crypt hyperplasia
Lymphocyte infiltration

28
Q

Coeliac patient express either or both?

A

HLA DQ2

HLA DQ8

29
Q

What happens in coeliac?

A

Gliadin is degraded by transgluatmine 2 enzyme to form gliadin peptides.

HLA DQ2/8 molecules can present gliadin peptides to T cells

30
Q

What percent of Europeans have HLA DQ2/8?

A

30-50%

31
Q

What are the peripheral tolerance mechanisms?

A

Immunological heirachy- CD4 T cell wont be activated unless antigen in presented in an inflammatory context with TLR ligation

Antigen segregation

Peripheral anergy- weak signalling APC/CD4 T cell with costimulation causes T cells to become unresponsive

Regulatory T cells- cd25/foxp3

Cytokine deviation- change in phenotype from th1 to TH2

Clonal exhaustion- apoptosis post activation by activation induced cell death

32
Q

AID organ specific?

A 
Type 1 diabetes
Pemphigus
Graves
Hashmitos
Autoimmune cytopenia etc
33
Q

Multi system AID?

A

Lupus
Rheumatoid arthritis
Sjogrens

34
Q

Criteria for type 2 hypersensitivity?

A

Pathogenic antibody is identified
Removal of antibody by plasmapheresis is beneficial
Diseases can be transferred between experimental animal/gestation

35
Q

Type 2 hypersensitivity, antibody mediated?

A 
Graves
Autoimmune thrombolysis
Autoimmune haemolytic anaemia
Myasthenia gravis (ptosis)
Spontaneous urticaria
36
Q

Why is it hard to check for T cell mediated conditions?

A

More difficult to demonstrate auto reactive T cells in vitro than it is to demonstrate antibody

Experimental models rely on genetically susceptible animals that are sensitised, often by exposure to a self antigen with an adjuvant

37
Q

T cell mediated conditions?

A

Hashimotos
Type 1 diabetes
Coeliac

38
Q

IPEX cause?

A

Foxp3 mutation
Abrogates production of CD4 cd25 and foxp3 reg T cells

Key features:
IBD dermatitis organ specific autoimmunity

39
Q

Which are the monogenic autoimmunity conditions?

A

IPEX
DiGeorge
APACED

204 (58 decks)

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