Immunology of AI disease

Question 1

Does having autoimmune phenomena mean you have the disease?

Answer 1

No

Question 2

What is HLA?

Answer 2

Human leukocyte antigen complex

Question 3

What are the genetic influences in AI disease?

Answer 3

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

Question 4

What is IPEX syndrome?

Answer 4

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

Question 5

What do all nucleated cells express?

Answer 5

HLA class 1

Question 6

How many HLA molecules can antigen presenting cells express?

Answer 6

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

Question 7

How many variants of HLA does each individual posses?

Answer 7

2

Question 8

What can disease clustering indicate?

Answer 8

a genetic basis or relate to a common immune immune defect

Question 9

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

Answer 9

EBV, Parovirus, Coxsackie, Klebsiella

Question 10

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

Answer 10

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

Question 11

Are all autoantibodies pathogenic?

Answer 11

No

Question 12

What do circulating auto-antibodies in autoimmune disease activate

Answer 12

complement
phagocytes
natural killer cells

Question 13

What are the 2 clinical classifications of AI disease?

Answer 13

Organ specific AI disease

Multi-system AI disease

Question 14

What kind of hypersensitivity reaction is SLE?

Answer 14

Type 3

Question 15

What is the “fundamental abnormality” in SLE?

Answer 15

Disturbed regulation of B cell activity

Question 16

In SLE what do the auto-antibodies act against?

Answer 16

Dead and dying nuclear material including extractable nuclear antigens

Question 17

What does Normocytic normochromic anaemia indicate?

Answer 17

Chronic disease

Question 18

What are anti-nuclear antibodies?

Answer 18

Group of antibodies that bind to nuclear proteins

Question 19

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

Answer 19

Anti- Ro
Anti - La
SLE and Sjogrens

Question 20

What is the anti-centromere antibody specific for?

Answer 20

Limited scleroderma

Question 21

What is the NucleolR ANA associated with?

Answer 21

Scleroderma

Question 22

What investigations ca be used for Type 3 HSR?

Answer 22

Specific IgG to putative antigen
Low serum complements
Characteristic biopsy features

Question 23

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

Answer 23

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

Question 24

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

Answer 24

4

Question 25

What kind of HSR is rheumatoid arthritis?

Answer 25

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

Question 26

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

Answer 26

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

Question 27

What can rheumatoid factor be used for?

Answer 27

evaluate prognosis in patiets with rheumatoid arthritis

may be helpful in the diagnosis of Sjogrens syndrome

Question 28

What is a more specific test for RA?

Answer 28

Anti-CCP antibody

It is also a better predictor d an aggressive disease course