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Flashcards in Immunology Deck (29):
1

what are monogenic autoimmune disorders?

autoimmune disease caused by single gene defects
(rare)

2

What is IPEX syndrome?

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

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

3

what symptoms do children with IPEX present with?

type 1 diabetes (

4

what is the pathogenesis of IPEX syndrome?

mutation in a gene essential for development of regulatory T cells

5

what type of HLA increases the susceptibility of ankylosing spondylitis?

HLA B27

6

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

HLA DR3/DR4

7

what class of HLA do all nucleated cells express?

HLA class I

8

what type of cells do HLA class I control?

cytotoxic T cells

9

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

HLA class II

10

what type of hypersensitivity is lupus?

type III

11

what is the fundamental abnormality in SLE?

disturbed regulation of B cell activity

12

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

due to pericardial effusion

13

what antibody is specific for limited systemic scleroderma?

anti-centromere antibody

14

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

low complement levels

(because you have used up the store for activation)

15

what is the criteria classification of SLE?

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

what type of hypersensitivity is rheumatoid arthritis?

type IV

17

what cell infiltrate in the synovium characterises rheumatoid arthritis?

initially- helper T cells
secondary- phagocytes, activated B cells and antibody

18

what antibodies can be present in rheumatoid arthritis?

anti-CCP
Rheumatoid Factor

19

what are the associated conditions with anti-CCP antibody?

rheumatoid arthritis

20

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

SLE
Sjogren's syndrome
Systemic Sclerosis
Liver Disease

21

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

SLE

22

what are the associated conditions with anti-Sm?

SLE

23

what are the associated conditions with anti-Ro?

SLE, Sjogren's syndrome

24

what are the associated conditions with anti-La?

Sjogren's syndrome

25

what are the associated conditions with anti-centromere?

limited systemic sclerosis

26

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

diffuse systemic sclerosis

27

what are the associated conditions with anti-RNP?

SLE
mixed connective tissue disease

28

what are the associated conditions with anti-cardiolipin antibody?

anti-phospholipid syndrome

29

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

small vessel vasculitis
(GPA, EGPA, MPA)