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Leila's Yr 5 Pathology > Immunology 1A: Autoimmune and autoinflammatory diseases 1 > Flashcards

Immunology 1A: Autoimmune and autoinflammatory diseases 1 Flashcards

1
Q

What is immunopathology?

A

Damage to the host caused by the immune response

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2
Q

What is autoimmunity/autoinflammatory?

A

immunopathology in the absence of infection

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3
Q

Difference between autoimmunity and autoinflamamtory?

A

Autoinflammatory - disease driven by components of innate immune system
Autoimmunity = disease driven by components of the adaptive immune response

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4
Q

Examples of rare monogenic autoinflammatory disorders

A

Familial mediterranean fever

TRAPS

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5
Q

Examples of polygenic autoinflammatory diseases

A 
Crohn's
UC
Osteoarthritis 
GCA
Takayasu's
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6
Q

Example of mixed pattern diseases

A

Ank spond
Psoriatic arthritis
Behcet’s

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7
Q

Examples of rare monogenic autoimmune disorders

A

APECED/APS-1, IPEX, ALPS

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8
Q

Examples of polygenic autoimmune diseases

A 
RA
MG
SLE
Pernicious anaemia
Coeliac
Graves
Goodpastures
PBC
ANCA mediated vasculitis
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9
Q

2 cytokines which are often affected in monogenic auto inflammatory conditions?

A

TNFa and iL-1

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10
Q

Which gene encodes cryopyrin?

A

NLRP3

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11
Q

Which gene is mutated in familial mediterranean fever?

A

MEFV (leads to loss of function mutation of pyrin-manenostrin)

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12
Q

What protein doe MEFV encode?

A

Pyrin-Manenostrin

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13
Q

Function of pyrin-manenostrin?

A

Negative regulator of inflammasome complex

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14
Q

Familial mediterranean fever inheritance

A

AR

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15
Q

Which cell type is the inflammation driven by in FMF?

A

Neutrophils

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16
Q

What does procaspase 1 lead to?

A

Increased IL-1
NFKb (encodes TNFa)
Apoptosis

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17
Q

Clinical presentation of FMF

A 
Periodic fevers lasting 48-96 hours
Peritonitis
Pleuritis
Carditis
Arthritis
Rash
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18
Q

Long term risk of FMF?

A

AA Amyloidosis (in kidney –> nephrotic syndrome)

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19
Q

Management of FMF?

A

Colchicine and etanercept OR anakinra

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20
Q

Name an IL-1 inhibitor

A

Anakinra

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21
Q

Name a TNFa inhibitor

A

Etanercept

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22
Q

MOA colchicine

A

binds to tubilin in neutrophils and disrupts function in migration /cytokine secretion

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23
Q

Autoimmune diseases associated with AA amyloidosis

A

RA, AS, IBD

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24
Q

Three types of pathogenesis of monogenic autoimmune disease

A

abnormality of central tolerance
abnormality of Tregs
abnormality of lymphocyte apoptosis

25
Q

APECED pathophysiology

A

Defect in central tolerance
AIRE gene (Autoimmune regulator)
Autosomal recessive

26
Q

Function of AIRE gene

A

Upregulates the expression of self-antigens bythymic cells which T cells are selected against –> auto-reactive T and B-cells

27
Q

Autoimmune diseases associated with APECED

A

Hypothyroidism
Addisons
Candidiasis

28
Q

Why candidiasis with APECED?

A

Auto-antibodies to IL-17 and il-22

29
Q

IPEX pathophysiolpogy

A

X-linked
Impaired Tregs, mutation in FOXP3
Auto-reactive B cells

30
Q

Symptoms of IPEX

A

Diarrhoea (enteropathy), dermatitis, diabetes

31
Q

What does ALPS stand for?

A

Autoimmune lymphoproliferative syndrome

32
Q

ALPS pathogenesis

A

Abnormality of lymphocyte apoptosis due to mutations in FAS pathway –> failure of tolerance as autoreactive T cells do not die

33
Q

Symptoms of ALPS

A 
Lymphocytosis
Splenomegaly
Lymph nodes
AI diseases e.g. cytopaenias
Increased risk of lymphoma
34
Q

Flow cytometry feature of ALPS

A

Double negative T cells (CD4 - CD8-)

35
Q

Major mutation often seen in Crohn’s?

A

NOD2 (IBD1 gene on chr16)

36
Q

What other diseases have NOD2 mutation?

A

Blau syndrome and some types of sarcoidosis

37
Q

Where is NOD2 expressed?

A

Cytoplasm of myeloid cells e.g. macropahges, neutrophils, DCs

38
Q

Function of NOD2

A

Cytoplasmic microbial sensor (muramyl dipeptide)

39
Q

Normal function of HLA-B27

A

Ag presentation to CD8+ T cells

40
Q

Normal function of IL-23R

A

Differentiation of TH17 cells

41
Q

Where does AS tend to occur?

A

insertion point of ligaments + tendons (entheses)

42
Q

Are autoantibodies usually a feature of mixed pattern disease and polygenic autoimmune disease?

A

Mixed disease - NO

Polygenic autoimmune - YES

43
Q

HLA-DR15 which disease?

A

Goodpastures

44
Q

HLA DR-3 which diseases?

A

T1DM
Graves
SLE

45
Q

2 GENETIC Polymorphisms involved in T cell activation

A 
PTPN 22 (suppresses T cell activation normally)
CTLA4 (Inhibitory signal to control T-cell activation)
46
Q

3 aspects of peripheral toelranceq

A

T-cells need co-stimulation
Tregs
Sites of immune privelege

47
Q

What are 3 sites of immune privelege?

A

Eyes
Testes
CNS

48
Q

Gel and coombs classification

A

Type I = Immediate hypersensitivity which is IgE mediated
Typw II - Antibody reacts with cellular antigen
Type III = Antibody reacts with soluble antigen to form an immune complex
Type IV = Delayed-type hypersensitivity = T-cell mediated response

49
Q

Outline Type I hypersensitivity reaction

A

Pre-existiing IgE antibodies to allergen –> Ig E bound to Fc receptor on mast cells and basophils –> cell degranulation and release of inflammatory mediators–> increased vascular permeability, leukocyte chemotaxis and SM contraction

50
Q

2 mechanisms of type II hypersensitivity disease

A

Destruction or activation/blockage

51
Q

Give some examples of Type II diseases

A 
Grave's
MG
Goodpasture's
Pemphigus vulgaris
Membranous glomerulonephritis
52
Q

Affected antigen in pemphigus vulgaris?

A

EPidermal cadherin

53
Q

Antigen in goodpasture’s

A

Non-collagenous domain of BM collagen type IV

54
Q

c-ANCA against…

A

Proteinase 3

55
Q

p-ANCE against—

A

Myeloperoxidase

56
Q

Examples of type III hypersensitivity disease

A

SLE, Rheumatoid arthritis

57
Q

Tissue destruction by HLA Class I and II in type IV

A 
HLA class I present self-antigen to CD8 + T cells --> lysis
HLA class II present self-antigen to CD4+ T cells --> cytokine production, inflammation and tissue damage
58
Q

Examples of Type IV hypersensitivity disease

A

T1DM
Rheumatoid arthritis
MS

Leila's Yr 5 Pathology (100 decks)

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