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Flashcards in Immunology Deck (29)
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1
Q

what are monogenic autoimmune disorders?

A

autoimmune disease caused by single gene defects

rare

2
Q

What is IPEX syndrome?

A

Immune-dysfunction, Polyendocrinopathy, Enteropathy and X-linked inheritance syndrome:

x-linked, autoimmune, recessive disorder that presents early in childhood

3
Q

what symptoms do children with IPEX present with?

A

type 1 diabetes (

4
Q

what is the pathogenesis of IPEX syndrome?

A

mutation in a gene essential for development of regulatory T cells

5
Q

what type of HLA increases the susceptibility of ankylosing spondylitis?

A

HLA B27

6
Q

what type of HLA increases the susceptibility of type 1 diabetes?

A

HLA DR3/DR4

7
Q

what class of HLA do all nucleated cells express?

A

HLA class I

8
Q

what type of cells do HLA class I control?

A

cytotoxic T cells

9
Q

what class of HLA do specialised antigen-presenting cells express?

A

HLA class II

10
Q

what type of hypersensitivity is lupus?

A

type III

11
Q

what is the fundamental abnormality in SLE?

A

disturbed regulation of B cell activity

12
Q

why might there by very quiet heart sounds in a patient with SLE?

A

due to pericardial effusion

13
Q

what antibody is specific for limited systemic scleroderma?

A

anti-centromere antibody

14
Q

what complement levels would you expect to find in a patient with SLE?

A

low complement levels

because you have used up the store for activation

15
Q

what is the criteria classification of SLE?

A
Serositis
Oral ulcers
Arthritis (non-erosive)
Photosensitivity
Blood disorders
Renal involvement
ANA
Immunologic phenomena (ie anti-dsDNA, anti-Sm)
Neurological disorders
Malar rash
Discoid rash
16
Q

what type of hypersensitivity is rheumatoid arthritis?

A

type IV

17
Q

what cell infiltrate in the synovium characterises rheumatoid arthritis?

A

initially- helper T cells

secondary- phagocytes, activated B cells and antibody

18
Q

what antibodies can be present in rheumatoid arthritis?

A

anti-CCP

Rheumatoid Factor

19
Q

what are the associated conditions with anti-CCP antibody?

A

rheumatoid arthritis

20
Q

what are the 4 main associated conditions with ANA? (anti-nuclear antibody)

A

SLE
Sjogren’s syndrome
Systemic Sclerosis
Liver Disease

21
Q

what are associated conditions with anti-dsDNA? (anti-double stranded DNA)?

A

SLE

22
Q

what are the associated conditions with anti-Sm?

A

SLE

23
Q

what are the associated conditions with anti-Ro?

A

SLE, Sjogren’s syndrome

24
Q

what are the associated conditions with anti-La?

A

Sjogren’s syndrome

25
Q

what are the associated conditions with anti-centromere?

A

limited systemic sclerosis

26
Q

what are the associated conditions with anti-Scl-70?

A

diffuse systemic sclerosis

27
Q

what are the associated conditions with anti-RNP?

A

SLE

mixed connective tissue disease

28
Q

what are the associated conditions with anti-cardiolipin antibody?

A

anti-phospholipid syndrome

29
Q

what are the associated conditions with anti-neutrophil cytoplasmic antibody? (ANCA)

A

small vessel vasculitis

GPA, EGPA, MPA