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Flashcards in Immunology Deck (44):
1

HLA-DR2

Goodpastures

2

Graves disease HLA?

HLA-DR3

3

SLE HLA?

HLA-DR3

4

Type 1 diabetes HLA?

HLA-DR3/DR4

5

Rheumatoid arthritis HLA?

HLA-DR4

6

Which type of hypersensitivity is SLE?

Type III hypersensitivity

7

Symptoms of IPEX disease

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

8

Clinical classification of auto-immune diseases?

Organ-specific disease
Non-organ specific disease

9

Eating cells seen in type III hypersensitivity?

Macrophages and neutrophils

10

B or T cell dysregulation in SLE and antibodies?

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

11

Name two ENAs?

anti-Ro
anti-La

12

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

speckled!!!

13

What is anti-centromere antibody very specific for?

Scleroderma

14

What binds to kinetochore (chromosomes)?

Anti-centromere antibody

15

Nucleolar ANA?

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

16

Name 2 proteins associated with nucleolar RNA?

Fibrillarin, U1RNP

17

What is anti-smith antibody very specific for?

SLE

18

Anti-DNA antibodies in SLE

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.

19

Characteristic biopsy features of type III hypersensitivity?

Immune complexes, macrophages and neutrophils, products of complement activation

20

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

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

21

Type III hypersensitivity management

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

22

Jaccouds arthritis?

SLE

23

Libmansach endocarditis?

SLE

24

SLE glomerulonephritis?

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

25

SLE cardiac features

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

26

SLE pulmonary features

Pleurisy
Infections
Diffuse lung infiltration and fibrosis
Pulmonary hypertension
Pulmonary infarct

27

SLE neurological features

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

28

SLE haemtological features

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

29

SLE drug treatment

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

30

How is cyclophosphamide administered?

IV

31

IPEX inheritence

Autosomal dominant

32

Ankylosing spondylitis HLA

HLA B27

33

Anti-nuclear antibodies associated with which conditions?

SLE
Rheumatoid arthritis/autoimmune conditions
HIV
Hepatitis
Females
Older age

34

Speckled antibody

Ro and La
(SLE, Sjogrens)

35

Anti-centromere

Limited scleroderma (CREST)

36

Nucleolar ANA (fibrillarin, U1RNP)

Scleroderma

37

Anti-RNP

Mixed connective tissue disease and SLE

38

Anti-toposiomerase (Anti-Scl70)

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

39

Shows activity in SLE?

Anti-dsDNA
C3 and C4 (negatively correlate)

Unactivated C3 and C4 measured in Ninewells)

40

GN appearance in SLE

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

41

GN in goodpastures?

Linear deposition

42

RA is which type of reacion?

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

43

Pro-inflammatory cytokines in RA

TNF and IL-1

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

44

Major indications for rheumatoid factor testing?

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