Immunology

Question 1

HLA-DR2

Answer 1

Goodpastures

Question 2

Graves disease HLA?

Answer 2

HLA-DR3

Question 3

SLE HLA?

Answer 3

HLA-DR3

Question 4

Type 1 diabetes HLA?

Answer 4

HLA-DR3/DR4

Question 5

Rheumatoid arthritis HLA?

Answer 5

HLA-DR4

Question 6

Which type of hypersensitivity is SLE?

Answer 6

Type III hypersensitivity

Question 7

Symptoms of IPEX disease

Answer 7

• early onset insulin dependent diabetes mellitus
• severe enteropathy
• eczema
• autoimmune thyroid disease, autoimmune haemolytic anaemia,
• severe infections

Question 8

Clinical classification of auto-immune diseases?

Answer 8

Organ-specific disease

Non-organ specific disease

Question 9

Eating cells seen in type III hypersensitivity?

Answer 9

Macrophages and neutrophils

Question 10

B or T cell dysregulation in SLE and antibodies?

Answer 10

B cell
Antibodies to nuclear proteins:
-Anti-DNA
-Anti-nuclear
-Anti-ENA

Question 11

Name two ENAs?

Answer 11

anti-Ro

anti-La

Question 12

What do anti-Ro and anti-La look like?

Answer 12

speckled!!!

Question 13

What is anti-centromere antibody very specific for?

Answer 13

Scleroderma

Question 14

What binds to kinetochore (chromosomes)?

Answer 14

Anti-centromere antibody

Question 15

Nucleolar ANA?

Answer 15

Scleroderma!!

Nucleolar ANA: Autoantigens = proteins associated with nucleolar RNA including fibrillarin, U1RNP

Question 16

Name 2 proteins associated with nucleolar RNA?

Answer 16

Fibrillarin, U1RNP

Question 17

What is anti-smith antibody very specific for?

Answer 17

SLE

Question 18

Anti-DNA antibodies in SLE

Answer 18

Very high titres are often associated with more severe disease, including renal or CNS involvement!!

Useful in disease monitoring
an increase in antibody titre is associated with disease activity and may precede disease relapse.

Question 19

Characteristic biopsy features of type III hypersensitivity?

Answer 19

Immune complexes, macrophages and neutrophils, products of complement activation

Question 20

Immune complex (type III) vs antibody (type II) mediated disease pathology

Answer 20

-Granular lumpy bumpy pattern of IgG deposition in type III
-Linear deposition along basement membrane in type II
(this can be seen in glomerulonephritis

Question 21

Type III hypersensitivity management

Answer 21

Decrease inflammation
Corticosteroids
Decrease production of antibody
Immunosuppressive agents
Anti-proliferative agents
Azathioprine
Mycophenolate
Cyclosphosphamide

Question 22

Jaccouds arthritis?

Answer 22

SLE

Question 23

Libmansach endocarditis?

Answer 23

SLE

Question 24

SLE glomerulonephritis?

Answer 24

Proteinuria
Urine sediments
Urine RBC and casts
Hypertension
Acute renal failure
Chronic renal failure

Question 25

SLE cardiac features

Answer 25

Pericarditis Cardiomyopathy Pulmonary hypertension Libman Sach endocarditis (non-bacterial endocarditis)

Question 26

SLE pulmonary features

Answer 26

``` Pleurisy Infections Diffuse lung infiltration and fibrosis Pulmonary hypertension Pulmonary infarct ```

Question 27

SLE neurological features

Answer 27

``` Depression/psychosis Not always related to disease activity Migranous headache Cerebral ischaemia TIAs or stroke Cranial or peripheral neuropathy Cerebellar ataxia ```

Question 28

SLE haemtological features

Answer 28

``` Lymphadenopathy ~25% of all patients during their course of illness Leucopenia (low white cells) Anaemia haemolytic normochromic normocytic Thrombocytopenia (low platelets) ```

Question 29

SLE drug treatment

Answer 29

-NSAID and simple analgesia -Anti-malarials -Steroids -Aziathioprine -Cyclophosphamide (IV) -Methotrexate -Miycophenalate mofetil Calcineurin inhibitors (ciclosporin A, tacrolimus) Biologics: rituximab,

Question 30

How is cyclophosphamide administered?

Answer 30

IV

Question 31

IPEX inheritence

Answer 31

Autosomal dominant

Question 32

Ankylosing spondylitis HLA

Answer 32

HLA B27

Question 33

Anti-nuclear antibodies associated with which conditions?

Answer 33

``` SLE Rheumatoid arthritis/autoimmune conditions HIV Hepatitis Females Older age ```

Question 34

Speckled antibody

Answer 34

Ro and La | SLE, Sjogrens

Question 35

Anti-centromere

Answer 35

Limited scleroderma (CREST)

Question 36

Nucleolar ANA (fibrillarin, U1RNP)

Answer 36

Scleroderma

Question 37

Anti-RNP

Answer 37

Mixed connective tissue disease and SLE

Question 38

Anti-toposiomerase (Anti-Scl70)

Answer 38

Diffuse scleroderma, associated with more severe organ involvement, including pulmonary fibrosis

Question 39

Shows activity in SLE?

Answer 39

Anti-dsDNA C3 and C4 (negatively correlate) Unactivated C3 and C4 measured in Ninewells)

Question 40

GN appearance in SLE

Answer 40

Granular "lumpy-bumpy" (because type III duh)

Question 41

GN in goodpastures?

Answer 41

Linear deposition

Question 42

RA is which type of reacion?

Answer 42

Type IV hypersensitivity response Characterised initially by infiltration of synovium by CD4+ T cells Secondary involvement of activated B cells and antibody

Question 43

Pro-inflammatory cytokines in RA

Answer 43

TNF and IL-1 High synovial concentrations Potent stimulators of fibroblasts, osteoclasts and chondrocytes Stimulate release of matrix metalloproteinases Important mediators of joint damage

Question 44

Major indications for rheumatoid factor testing?

Answer 44

Evaluate prognosis in patients with rheumatoid arthritis Associated with more severe erosive disease Associated with extra-articular disease manifestations eg nodules and vasculitis May be helpful in the diagnosis of Sjogren’s syndrome cryoglobulinaemia ANTI-CCP: More specific test for rheumatoid arthritis than rheumatoid factor and better predictor of an aggressive disease course In patients with undifferentiated arthritis, anti-CCP antibodies may predict those who are likely to develop rheumatoid arthritis