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MSK > Immunology of autoimmune disease > Flashcards

Immunology of autoimmune disease Flashcards

1
Q

What is a monogenic disorder?

A

Single gene defect which causes the autoimmune disease.

Rare.

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2
Q

What is IPEX syndrome?

A

Immune dysregulation Polyendocrinopathy Enteropathy and X-linked inheritance syndrome.

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3
Q

What kind of inheritance does IPEX syndrome have?

A

Fatal autosomal recessive disorder

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4
Q

When does IPEX syndrome present?

A

Early childhood

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5
Q

What are the symptoms of IPEX syndrome?

A 
IDDM
Malabsorption 
Eczema
Autoimmune thyroid and haemolytic anaemia
Severe infections
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6
Q

What is the pathogenesis of IPEX syndrome?

A

Mutation in FOXP3 gene which is essential to develop T cells.

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7
Q

What does HLA stand for?

A

Human Leukocyte Antigen complex

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8
Q

What does HLA do?

A

???

On the surface of antigen and recognises peptides.

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9
Q

What are the 3 types of class I HLA?

A

HLA-A, HLA-B and HLA-C.

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10
Q

What are the specialised class II HLA?

A

HLA-DR
HLA-DQ
HLA-DP

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11
Q

True or False.

Each person has only 1 type of HLA molecule

A

False.

Each individual possesses 2 variants of each HLA molecule.

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12
Q

In what way is HLA like an enzyme?

A

Like lock and key mechanism.

Only a few peptides can bind to a specific molecule

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13
Q

What is the susceptible allele of ankylosing spondyltitis?

A

HLA B27

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14
Q

What is the susceptible allele of rheumatoid arthritis?

A

HLA-DR4

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15
Q

Which rheum disease is a type III hypersensitivity diseases?

A

SLE

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16
Q

What is a type III hypersenstivity?

A

Antibody-antigen complex deposition in the small vessels activating complement and macrophages to the site.

17
Q

Which antibody is characterised in SLE?

A

ANA- anti nuclear antibodies.

18
Q

What are the clinical features of SLE?

A 
??
malar rash
fatigue
arthralgia
weight loss
depression
19
Q

What other diseases is anti-ANA associated with?

A

Scleroderma

Sjogrens

20
Q

When would you find an anti-centromere antibody?

A

Systemic sclerosis/ CREST.

21
Q

What is the classical pathway of complement?

A

Centres around C3.

Formation of antibody-antigen complexes.

22
Q

Why would you use C3 as a marker for disease?

A

It measures unactivated C3.

If C3 levels are low then C3 has been used up and would be a good marker of disease activity.

23
Q

What other investigations would you do if still not sure from bloods?

A

Biopsy for presence of immune complexes

24
Q

How does glomerulonephritis in SLE differ from that in Goodpastures (a type II)?

A

Goodpastures glomerulonephritis has a linear deposition of IgG on basement membrane.
SLE is bumpy.

25
Q

What is the treatment for SLE?

A

Steroids.

Immunosuppressants

26
Q

Which rheum condition is a type IV hypersensitivity?

A

RA

27
Q

What is a type IV hypersensitivity?

A

Delayed type hypersensitivity.

28
Q

What is RA?

A

Autoimmune inflammatory disease characterised by destruction of joint cartilage and inflammation of synovium

29
Q

What systemic effects can poorly managed RA cause?

A

Osteoporosis
Bone resorption
Destruction of joint cartilage

30
Q

What is the immunopathogenesis of RA?

A

Infiltration of CD4+ cells to synovium which produces cytokines. Causes cascade and phagocytes are recruited along with B cells.

31
Q

How does the immunopathogenesis actually cause a diseased joint?

A

Cytokines and matrix metalloproteinase formed which directly stimulate breakdown of bone and cartilage.

32
Q

Why are TNF and IL1 important in RA?

A

Stimulators of fibroblasts, osteoclasts and chrondrocytes and stimulate release of matrix metalloproteinases.

33
Q

How does anti-TNF work?

A

Ab to cytokine so cytokine cannot attach to receptor.

34
Q

What is rheumatoid factor?

A

An antibody directed against Fc region of human IgG.

35
Q

True or False

If RF is negative this means the diagnosis definitely is not RA

A

False.

Not specific enough

MSK (30 decks)

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