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BPS Exam Study > Ch 6 Immune system diseases > Flashcards

Ch 6 Immune system diseases Flashcards

1
Q

What are the primary target organs of acute GVHD?

A

The primary targets are skin, gut epithelium, bile ducts, and lymphoid tissue.

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2
Q

What are the main cytokines involved in a Type 1 hypersensitivity reaction?

A

IL-4, IL-5, IL-13

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3
Q

What activates mast cell in a type 1 hypersensitivity reaction?

A

Cross-linking of high affinity IgE Fc receptors
Also triggered by C5a and C3a

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4
Q

Localised area of tissue necrosis (fibrinoid necrosis) resulting from acute immune complex vasculitis usually elicited in the skin.
An example of a Type III hypersensitivity reaction

A

Arthus reaction

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5
Q

Main cells involved in a Type IV hypersensitivity reaction

A

Th1 and Th17

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6
Q

Autoimmune haemolytic anaemia and Goodpasture syndrome are examples of which type of hypersensitivity reaction?

A

Type II

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7
Q

Most immediate hypersensitivity disorders are caused by excessive responses from which cell type

A

Th2 cells

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8
Q

In a type 1 hypersensitivity reaction, IgE binds to which receptor on mast cells

A

Fc receptors (FcER1)

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9
Q

What mediators are released by mast cells in a type 1 hypersensitivity reaction?

A
  • Granule contents: Histamine, Proteases (e.g. tryptase), Proteoglycans
  • Lipid mediators: Leukotrienes (B4, C4, D4), Prostaglandin D2; Platelet-activating factor (PAF)
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10
Q

3 mechanisms of antibody-mediated injury in type 2 hypersensitivity reactions?

A
  • Opsonisation and phagyocytosis
  • Complement and Fc receptor mediated inflammation
  • Anti-receptor antibodies disturb the normal function of receptors
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11
Q

Cytokine that activates macrophages in type IV hypersensitivity reactions

A

IFN-gamma

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12
Q

Which complement components can activate mast cells?

A

C3a and C5a

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13
Q

What are the most potent vasoactive and spasmogenic agents released by mast cells?

A

Leukotrienes C4 and D4

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14
Q

Which mediators released by mast cells are lipid derived?

A

Prostaglandins and Leukotrienes (from arachidonic acid)
Platelet activating factor

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15
Q

Can non-antigenic stimuli cause type 1 hypersensitivity reactions?

A

Yes; it is estimated 20-30% of immediate hypersensitivity reactions are triggered by non-antigenic stimuli such as temperature extremes and exercise and do not involve Th2 cells or IgE (sometimes called nonatopic allergy)

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16
Q

Process involved in contact dermatitis / drug reactions?

A

Environmental chemical binds to and structurally modifies self proteins
Modified proteins are recognised by T cells and elicit the reaction

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17
Q

Differences between central and peripheral immune tolerance

A

Central: immature lymphocytes that recognise self antigens in the central (generative) lymphoid organs are killed by apoptosis

Peripheral: mature lymphocytes that recognise self antigens in peripheral tissues become functionally inactive, are suppressed by regulatory T cells or die by apoptosis

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18
Q

What is FOXP3 and what happens if it is defective?

A

A transcription factor required for the development and maintenance of functional CD4+ regulatory T cells

Mutations in FOXP3 result in severe autoimmunity

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19
Q

What are the patterns of nuclear staining by indirect immunofluorescence? With which diseases can each often be associated?

A
  • Homogenous e.g. SLE
  • Speckled - least specific staining. Seen with antibodies against various nuclear antigens including Sm and RNPs
  • Centromeric e.g. Sjogrens syndrome, some cases of systemic sclerosis
  • Nucleolar e.g. systemic sclerosis (most often)
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20
Q

What are antiphospholipid antibodies and against what can they be directed? Why are they sometimes referred to as lupus anticoagulant?

A
  • antibodies specific for epitopes of plasma proteins that are revealed when the proteins are in complex with phospholipids e.g. prothrombin, protein S, protein C, B2 glycoprotein
  • some of these antibodies interfere with in vitro clotting tests such as partial thromboplastin time (therefore referred to as lupus anticoagulant)
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21
Q

What proportion of patients with SLE have antiphospholipid antibodies?

A

30-40%

22
Q

What is chronic discoid lupus erythematosus? Does the antibody profile differ at all from SLE?

A
  • Skin manifestations may mimic SLE but the systemic manifestations are rare
  • Approx 35% of patients test positive for generic ANAs but antibodies to dsDNA are rarely present
23
Q

What are the 3 interrelated processes thought to be involved in systemic sclerosis?

A
  • Autoimmunity
  • Vascular damage
  • Collagen deposition
24
Q

What morphological change dominates chronic rejection of transplants?

A

Vascular changes - arteriosclerosis

Parenchymal fibrosis also present

25
Q

What are the best known pattern recognition receptors in host defence

A

Toll like receptors

26
Q

All TLRs signal by a common pathway that culminates in the activation of 2 sets of transcription factors

A

(1) NF-kB (which stimulates the synthesis and secretion of cytokines and expression of adhesion molecules)
(2) Interferon regulatory factors (stimulate production of type 1 interferons)

27
Q

Where are TLRs located?

A

Plasma membrane and endosomal vesicles

28
Q

What are the 3 most common forms of amyloid?

A
  1. Amyloid light chain (AL) protein
  2. Amyloid-associated (AA) protein
  3. Beta-amyloid protein (AB)
29
Q

Where do fibrillar proteins deposit in amyloidosis?

A

Extracellularly

30
Q

What is the difference between primary and secondary amyloidosis?

A

Primary - associated with plasma cell proliferates

Secondary - when occurs as complication as part of a chronic inflammatory process

31
Q

Congo red staining shows apple-green birefringence under polarized light is diagnosis of..

A

Amyloidosis

32
Q

Loss of function mutations in this gene involved in central tolerance can lead to autoimmune polyendocrine syndrome

A

AIRE (autoimmune regulator)

33
Q

This protein stimulates expression of some peripheral tissue-restricted self antigens in the thymus and is critical for detection of immature T cells specific for these antigens as part of central tolerance

A

AIRE

34
Q

Term for lymphocytes that recognise self antigens being rendered functionally unresponsive

A

Anergy

35
Q

Molecule expressed on CD4+ T cells that is important for co-stimulatory signal in activation

A

CD28

36
Q

Structurally similar molecule to CD28 but provides an inhibitory signal to CD4+ T cells that recognise self antigens

A

CTLA-4

37
Q

Mutations in this transcription factor (normally expressed in high levels by T regulatory cells) results in severe autoimmunity

A

FOXP3

38
Q

Term for sites such as testis, eye, brain where antigens introduced into these sites tend to elicit weak or no immune response

A

Immune-privileged sites

39
Q

What are the two main types of HIV called and where are they distributed geographically?

A

HIV-1: more common in US, Europe and central Africa

HIV-2: West Africa; India

40
Q

What are the 3 viral enzymes of HIV?

A
  • Protease
  • Reverse transcriptase
  • Integrase
41
Q

What is the major capsid protein of HIV detectable by ELISA?

A

p24

42
Q

What is the more clinically important marker for decision making – HIV viral load or CD4+ cell count?

A

CD4+ cell count

Used as the primary clinical measurement of when to start antiretroviral therapy

43
Q

Inherited defect in phagolysosome function

A

Chediak-Higashi syndrome

44
Q

Congenital disorder characterised by defects in bacterial killing that render patients susceptible to recurrent bacterial infection

(defects in genes encoding phagocyte oxidase)

A

Chronic granulomatous disease

45
Q

The most common complement protein deficiency

A

C2

46
Q

Deficiency of this complement proteins is associated with increased risk of immune complex mediated glomerulonephritis

A

C3

47
Q

Deficiency of this gives rise to hereditary angioedema

A

C1 inhibitor

48
Q

What is the most common form of SCID?

A

X-linked

  • the genetic defect in the common gamma chain subunit of cytokine receptors
  • T cell development particularly affected
49
Q

Inherited immune deficiency characterised by the failure of B cell precursors to develop into mature B cells

A

X-linked agammaglobulinemia

50
Q

Syndrome where the third and fourth pharyngeal pouches (thymus) do not develop normally resulting in T cell deficiency

Deletion of chromosome 22q11

A

Di George syndrome

51
Q

Immunodeficiency characterised by the inability to eliminate EBV, eventually leading to fulminant infectious mononucleosis and the development of B cell tumours

A

X-linked lymphoproliferative disease

52
Q

X-linked disease characterised by thrombocytopenia, eczema, and a marked vulnerability to infection

Progressive loss of T cells in the peripheral blood

A

Wiskott-Aldrich syndrome

BPS Exam Study (12 decks)

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