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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
APECED pathophysiology
Defect in central tolerance AIRE gene (Autoimmune regulator) Autosomal recessive
26
Function of AIRE gene
Upregulates the expression of self-antigens bythymic cells which T cells are selected against --> auto-reactive T and B-cells
27
Autoimmune diseases associated with APECED
Hypothyroidism Addisons Candidiasis
28
Why candidiasis with APECED?
Auto-antibodies to IL-17 and il-22
29
IPEX pathophysiolpogy
X-linked Impaired Tregs, mutation in FOXP3 Auto-reactive B cells
30
Symptoms of IPEX
Diarrhoea (enteropathy), dermatitis, diabetes
31
What does ALPS stand for?
Autoimmune lymphoproliferative syndrome
32
ALPS pathogenesis
Abnormality of lymphocyte apoptosis due to mutations in FAS pathway --> failure of tolerance as autoreactive T cells do not die
33
Symptoms of ALPS
``` Lymphocytosis Splenomegaly Lymph nodes AI diseases e.g. cytopaenias Increased risk of lymphoma ```
34
Flow cytometry feature of ALPS
Double negative T cells (CD4 - CD8-)
35
Major mutation often seen in Crohn's?
NOD2 (IBD1 gene on chr16)
36
What other diseases have NOD2 mutation?
Blau syndrome and some types of sarcoidosis
37
Where is NOD2 expressed?
Cytoplasm of myeloid cells e.g. macropahges, neutrophils, DCs
38
Function of NOD2
Cytoplasmic microbial sensor (muramyl dipeptide)
39
Normal function of HLA-B27
Ag presentation to CD8+ T cells
40
Normal function of IL-23R
Differentiation of TH17 cells
41
Where does AS tend to occur?
insertion point of ligaments + tendons (entheses)
42
Are autoantibodies usually a feature of mixed pattern disease and polygenic autoimmune disease?
Mixed disease - NO | Polygenic autoimmune - YES
43
HLA-DR15 which disease?
Goodpastures
44
HLA DR-3 which diseases?
T1DM Graves SLE
45
2 GENETIC Polymorphisms involved in T cell activation
``` PTPN 22 (suppresses T cell activation normally) CTLA4 (Inhibitory signal to control T-cell activation) ```
46
3 aspects of peripheral toelranceq
T-cells need co-stimulation Tregs Sites of immune privelege
47
What are 3 sites of immune privelege?
Eyes Testes CNS
48
Gel and coombs classification
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
Outline Type I hypersensitivity reaction
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
2 mechanisms of type II hypersensitivity disease
Destruction or activation/blockage
51
Give some examples of Type II diseases
``` Grave's MG Goodpasture's Pemphigus vulgaris Membranous glomerulonephritis ```
52
Affected antigen in pemphigus vulgaris?
EPidermal cadherin
53
Antigen in goodpasture's
Non-collagenous domain of BM collagen type IV
54
c-ANCA against...
Proteinase 3
55
p-ANCE against---
Myeloperoxidase
56
Examples of type III hypersensitivity disease
SLE, Rheumatoid arthritis
57
Tissue destruction by HLA Class I and II in type IV
``` 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
Examples of Type IV hypersensitivity disease
T1DM Rheumatoid arthritis MS

Leila's Yr 5 Pathology (100 decks)

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