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Flashcards in Immunology of AI disease Deck (28):
1

Does having autoimmune phenomena mean you have the disease?

No

2

What is HLA?

Human leukocyte antigen complex

3

What are the genetic influences in AI disease?

- Monogenetic disorders
- Complex genetic interplay between different immune regulating genes
- Genes determining sex

4

What is IPEX syndrome?

Immune dysregulation, polyendocrinopathy. enteropathy and X -linked inheritance syndrome
fatal autosomal recessive disorder presenting early in childhood

5

What do all nucleated cells express?

HLA class 1

6

How many HLA molecules can antigen presenting cells express?

12
6 HLA class 1 molecules and 6 HLA class 2 molecules

7

How many variants of HLA does each individual posses?

2

8

What can disease clustering indicate?

a genetic basis or relate to a common immune immune defect

9

Give some examples of initiating events in the pathogenesis of AI diseases.

EBV, Parovirus, Coxsackie, Klebsiella

10

What are the 3 main factors which contribute to AI disease?

- immune regulation
- infection
- HLA - association but not a prerequisite for disease

11

Are all autoantibodies pathogenic?

No

12

What do circulating auto-antibodies in autoimmune disease activate

complement
phagocytes
natural killer cells

13

What are the 2 clinical classifications of AI disease?

Organ specific AI disease
Multi-system AI disease

14

What kind of hypersensitivity reaction is SLE?

Type 3

15

What is the "fundamental abnormality" in SLE?

Disturbed regulation of B cell activity

16

In SLE what do the auto-antibodies act against?

Dead and dying nuclear material including extractable nuclear antigens

17

What does Normocytic normochromic anaemia indicate?

Chronic disease

18

What are anti-nuclear antibodies?

Group of antibodies that bind to nuclear proteins

19

Which autoantibodies are "speckled antibodies"? And what are they more specific for?

Anti- Ro
Anti - La
SLE and Sjogrens

20

What is the anti-centromere antibody specific for?

Limited scleroderma

21

What is the NucleolR ANA associated with?

Scleroderma

22

What investigations ca be used for Type 3 HSR?

Specific IgG to putative antigen
Low serum complements
Characteristic biopsy features

23

What is the difference between "lumpy-bumpy" and linear deposition glomerulonephritis?

lumpy bumpy - type 3 HSR - deposition of IgG ad C3 in granular pattern

linear deposition - type 2 HSR - IgG depositied along the glomerular basement membrane

24

How many out of 11 does a patient need to have to have according to the American Rheumatological Association criteria for SLE?

4

25

What kind of HSR is rheumatoid arthritis?

Type 4 - infiltartion of synovium by activated CD4+ cells --> production of cytokines, recruitment of phagocytes, activated B cells and synovial fibroblasts

26

Is rheumatoid factor a good/useful investigation in he diagnosis of RA?

No - Only 50% of patients with rheumatoid arthritis are positive for rheumatoid factor at the time of diagnosis

27

What can rheumatoid factor be used for?

evaluate prognosis in patiets with rheumatoid arthritis
may be helpful in the diagnosis of Sjogrens syndrome

28

What is a more specific test for RA?

Anti-CCP antibody
It is also a better predictor d an aggressive disease course