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Year 2 - Musculoskeletal (DP) > Immunology > Flashcards

Flashcards in Immunology Deck (47)
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1

A 2 year old patient presents as the mother is concerned about his growth. The mother is also concerned about the scratch marks on her son's ankles and wrists. He looks tired and is very quiet. On urine dipstick glucose is +++ and ketones are +. You examine the excoriated areas and diagnose them as eczema. The mother explains that he has a number of food allergies and has suffered regularly from URTIs.

IPEX Syndrome:
- Immune dysregulation
- Polyendocrinopathy
- Enteropathy
- X-Linked -> Fatal

2

What causes IPEX syndrome and what does it stand for?

Immune dysregulation
Polyendocrinopathy
Enteropathy
X-Linked -> Fatal
Cause:
- FOXP3 gene defect -> Reduced regulator T cells

3

What cells is HLA Class i found on?

All nucleated cells

4

What is the function of HLA Class i?

Present intracellular peptides:
- Infected cells display virus

5

What types of HLA Class i are there?

HLA-A
HLA-B
HLA-C

6

What cells is HLA Class ii found on?

Antigen-presenting cells

7

What is the function of HLA Class ii?

Present extracellular peptides

8

What types of HLA Class ii are there?

HLA-DR
HLA-DQ
HLA-DP

9

How many variants of each HLA does every person have?

Two

10

What does HLA diversity allow?

Maximises peptide binding since only a few peptides bind to each HLA

11

What are the susceptibility response genes associated with each of the following:
1. Ankylosing spondylitis
2. Goodpasture's Syndrome
3. Grave's Disease
4. SLE
5. T1DM
6. RA

1. HLA-B27
2. HLA-DR2
3. HLA-DR3
4. HLA-DR3
5. HLA-DR3/4
6. HLA-DR4

12

How does Type iii H/S arise?

1. AutoAB + Circulating Ag = Immune complex
2. Complexes deposit in small vessels
- eg. Bowman's capsule
3. Complement activation

13

What type of H/S is SLE?

Type iii

14

A 19 year old female is referred with arthralgia. She has a 4 months history of fatigue. The arthralgia is in her hands and she has some mouth ulcers. On examination you note a rash on her face sparing the nasolabial folds, there are no joint swellings. She has vasculitis present on her fingers and soft heart sounds. Her ANA titre is > 1:640, and she has positive Anti-Ro and Anti-La antibodies.

SLE

15

What do Anti-Nuclear Antibodies do?

Bind nuclear proteins

16

True or false; Any titre of ANA is indicative of autoimmune disease?

False
A titre of

17

What are speckled autoantibodies associated with?

ENA Abs:
- Anti-Ro
- Anti-La
SLE and Sjogren's

18

What dose Anti-Centromere Antibody do?

Binds to chromosomes (Kinetochore)

19

What is nucleolar ANA associated with?

Proteins associated with nuclear RNA:
- Fibrillarin
- U1RNP
Scleroderma (Diffuse)

20

What conditions is Anti-Jo-1 associated with?

Polymyositis
Dermatomyositis
(ILD in both of these)

21

What is Anti-La associated with?

Sjogren's
SLE

22

What is Anti-Ribonucleoprotein (RNP) associated with?

MCTD
SLE

23

What is Anti-Ro associated with?

SLE
Sjogren's

24

What autoimmune features does Anti-Ro positive SLE tend to present?

Photosensitivity
Thrombocytopaenia
Cutaneous LE

25

What is Anti-Smith associated with?

SLE

26

What is Anti-Topoisomerase I (Scl-70) associated with?

Diffuse scleroderma

27

What do high titres of Anti-DNA binding Ab suggest?

SLE with:
- Renal features
- CNS features

28

What dose the formation of immune complexes activate?

Classical complement pathway:
- C1, C4 + C2 -> C3 -> MAC
Tissue inflammation where complexes deposit

29

When we measure C3 and C4 are we measuring the activated or unactivated levels? What will the result we get indicate disease activity?

Unactivated
Low levels suggest lots of complement activation:
- Very active disease

30

What biopsy features will be present in Type iii H/S?

Immune complexes
Macrophage + Neutrophil infiltrate
Products of complement activation