Hypersensitivity Disorders - HLA Associations and Other Diseases Flashcards Preview

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Flashcards in Hypersensitivity Disorders - HLA Associations and Other Diseases Deck (28)
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1

HLA Associations - Ankylosing spondylitis -Susceptibility Alelle -Relative Risk (fold)

-Susceptibility Alelle - HLA B27 -Relative Risk (fold) - 87

2

HLA Associations - Goodpasture's Syndrome -Susceptibility Alelle -Relative Risk (fold)

-Susceptibility Alelle - HLA DR15/DR2 -Relative Risk (fold) - 10

3

HLA Associations - Graves Disease -Susceptibility Alelle -Relative Risk (fold)

-Susceptibility Alelle - HLA - DR3 -Relative Risk (fold) - 4

4

HLA Associations - SLE -Susceptibility Alelle -Relative Risk (fold)

-Susceptibility Alelle - HLA - DR3 -Relative Risk (fold) - 6

5

HLA Associations - T1DM -Susceptibility Alelle -Relative Risk (fold)

-Susceptibility Alelle - HLA DR3/DR4 -Relative Risk (fold) - 25

6

HLA Associations - Rheumatoid Arthritis -Susceptibility Alelle -Relative Risk (fold)

-Susceptibility Alelle - HLA-DR4 -Relative Risk (fold) - 4

7

PTPN22

Tyrosine phosphatase expressed in lymphocytes, associated development of RA, SLE, and T1DM

8

CTLA4

receptor for CD80/CD86 expressed by T cells, transmits inhibitory signal to control T cell activation, Associated with SLE, T1DM, Autoimmune thyroid disease

9

CREST syndrome - Limited Cutaneous Scleroderma - what does CREST stand for

Calcinosis Raynaud's (O)Esophageal dysmotility Sclerodactyly Telangiectasia (+ primary pulmonary hypertension)

10

CREST syndrome - Limited Cutaneous Scleroderma - Antibodies for diagnosis 

Anti-centromere Antibodies for diagnosis

 

11

CREST syndrome - Limited Cutaneous Scleroderma -increases risk for what?

High risk of Lung Fibrosis and Renal Crisis

12

Diffuse Cutaneous Scleroderma - what does it involve?

CREST + GIT+ interstitial pulmonary disease + renal problems

13

Diffuse Cutaneous Scleroderma - Antibodies?

Anti- Topoisomerase/ Scl70, RNA Pol 1,2 and 3, Fibrillarin Antibodies

14

Diffuse Cutaneous Scleroderma - who is affected?

Females: males 4:1

15

Sjogren's Syndrome - who is affected?

M:F 1:9, onset in late 40s

16

Sjogren's Syndrome - symptoms?

Dry mouth (xerostomia), eyes (keratoconjunctivitis sicca), nose and skin May affect kidneys, blood vessels, lungs, liver, pancreas and PNS, may get parotid or salivary gland enlargement 

17

Sjogren's Syndrome - Ab present?

Anti-Ro and anti-La antibodies present 

18

Sjogren's Syndrome - what test is used?

Use Schirmer test to measure production of tears- assessing for dry eyes

19

IPEX syndrome - what does it stand for?

Immune dysregulation, Polyendocrinopathy, Enteropathy and X-linked inheritance syndrome + autoimmune diseases

20

IPEX syndrome -Symptoms?

Eczematous dermatitis, nail dystrophy and autoimmune skin conditions such as alopecia universals and bullous pemphigoid

21

IPEX syndrome -age of death?

Most affected children die within the first 2 years of life

22

IPEX syndrome -inheritance?

X-linked recessive disorder with exclusive expression in males

23

IPEX syndrome -treatment?

Bone marrow transplant is the only cure. can use immunomodulators to help

24

Coeliac Disease -what is it?

Failure of tolerance to gluten. Villous atrophy and enteropathy

25

Coeliac Disease -Symptoms?

GIT discomfort, constipation, diarrhoea, bloating, fatigue

26

Coeliac Disease -what does it go on to cause deficiencies in?

Iron, B12, Folate, Fat, Vitamins A, D, E & K and calcium deficiencies

27

Coeliac Disease -Immunogloblins involved?

IgA EMA (anti- endomysial antibody) disappears with exclusion diet (95% specific, 85% sensitive) IgA TGT (anti- transglutaminase antibody) - 95% specific, 90-94% sensitive IgG anti-gliadin antibody - most persistent (30-50% specific, 57-80% sensitive)

28

Coeliac Disease -Test

Gold Standard test is to do a duodenal biopsy but it is not first line