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Pathology > Immunopathy > Flashcards

Immunopathy Flashcards

1
Q

Components of innate immunity

A
  1. Barriers (skin)
  2. Phagocytes
  3. NK cells
  4. Dendritic cells
  5. Complement
  6. Normal flora
  7. Chemicals (lysosyme, acute phase proteins, lactic acid,cytokines, complement)
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2
Q

Components of Adaptive Immunity

A
  1. T cells
  2. B cells
  3. APCs
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3
Q

Organs of innate vs adaptive immunity

A

Innate:

  • mucosa
  • skin
  • liver (source of acute phase proteins/complement)
  • bone marrow (source of cells)

Adaptive:

  • Bone marrow
  • THymus
  • Lymph nodes
  • Spleen
  • MALTs
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4
Q

Cytokines of Innate immunity

A

IL-1,6,8,10,12,17

TNF alpha and beta

IFN alpha and beta and gamma

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5
Q

Cytokines of Adaptive immunity and cells from which they are released

A

IL-1, 4, 5, 13

Produced by activated T cells

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6
Q

Cytokines of Hematopoiesis

A

IL-3, 7

GM-CSF

G-CSF

M-CSF

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7
Q

Function of IL-1

A

Innate immunity:

  • Cell activation
  • Fever
  • Acut phase proteins
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8
Q

Function of IL-6

A

Innate Immunity

  • Fever
  • Produce Actue phase proteins
  • Activates B and T cells

Released after IL-1 and TNF

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9
Q

Function of IL-8

A

Innate immunity

  • Neutorphil chemotaxis
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10
Q

Function of IL-10 and cells from which it is released

A

Innate immunity: **Induces Humoral Immunity**

  • Suppresses CMI-promoting cytokines (IL-2, 4, 5, 13 I think)
  • Stimulates Th2 differentiation (humoral immunity)

Released from:

  • Treg cells
  • APCs
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11
Q

Function of IL-12 and cells from which it is released

A

Innate Immunity: **Induces Cellular Immunity**

  • activates NK cells
  • Activates Tc cells
  • TH1 polarization (cellular immunity)

Released from:

  • APCs
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12
Q

Function of IL-17 and cells from which it is released

A

Innate Immunity **Proinflammatory**

  • activates neutrophils, fibroblasts, keratinocytes

Released from:

  • TH17 cells
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13
Q

Function of TNF alpha/beta

A

Innate Immunity

  • Like IL-1
  • Chemokine production from macrohpages and endothelial cells
  • Induces class I expression
  • Stimulates tissue factor expression (coagulation cascade)

Other: (TNF-alpha only)

  • Cachexia (mm wasting)
  • Inhibits myocardial contraction and smooth muscle tone –> Hypotension and possible shock
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14
Q

Function of IFN-alpha and -beta

A

Innate Immunity: **anti-viral**

  • Increase class I expression
  • Promotes Th1 polarization
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15
Q

Function of IFN-gamma and cells from which it is released

A

Innate Immunity: **promotes CMI**

  • Class I and II expression
  • Promotes IgG class switch
  • Promotes CMI

Released by:

  • Th1
  • NK cells
  • Tc cells
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16
Q

Function of IL-2 and cells from which it is released

A

Adaptive immunity

  • T cell maturation
  • Activates Tc and NK cells

Released by:

  • activated T cells
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17
Q

Function of IL-4 and cells from which it is released

A

Adaptive Immunity:

  • Promotes IgE isotype switching
  • Activates mast cells and eosinophils
  • Suppresses macrophages

Released by:

  • Activated T-cells
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18
Q

Function of IL-5 and cells from which it is released

A

Adaptive Immunity:

  • Promotes IgA isotype switching
  • Eosinophil maturation

Released by:

  • Activated T cells
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19
Q

Function of IL-13 and cells from which it is released

A

Adaptive Immunity:

  • Promotes IgE production
  • Inhibits proinflammatory cytokine synthesis
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20
Q

Function of IL-3 and cells from which it is released

A

Hematopoiesis:

  • B cell and granulocyte maturation

Released by:

  • T cells
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21
Q

Function of IL-7 and cells from which it is released

A

Hematopoiesis:

  • B and T cell maturation

Released by:

  • fibroblasts
  • bone marrow stromal cells
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22
Q

Function of GM-CSF

A

Hematopoiesis:

Maturation of:

  • Eosinophil
  • neutrophil
  • Monocyte
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23
Q

Function of G-CSF

A

Hematopoiesis:

  • Neutorphil maturation
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24
Q

Function of M-CSF

A

Hematopoiesis:

  • Monocyte maturation
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25
Q

Function of Regulatory cytokines: TGF-beta and IL-10

A

Inhibit activities of:

  • Th1
  • Th2
  • Th17
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26
Q

MHC Class I

  1. Cell types expressing it
  2. Gene clusters and chromosome
  3. Structure
  4. Size of peptide binding
  5. Endogenous/Exogenous pathogens
A
  1. Nucleated cells and platelets
  2. Chromosome 6; gene clusters A, B, C
  3. Polymorphic alpha chain (3 regions), non-polymorphic ß2 microglobulin
  4. binds peptides w/8-10 aa
  5. Endogenous
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27
Q

MHC Class II

  1. Cell types expressing it
  2. Gene clusters and chromosome
  3. Structure
  4. Size of peptide binding
  5. Endogenous/Exogenous pathogens
A
  1. APCs
  2. Chromosome 6, DP, DQ, DR
  3. Polymorphic Alpha and beta chain
  4. 10-35 aa
  5. Exogenous
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28
Q

What is the inheritance pattern of MHC?

A
  • They are inherited intact from each parent
  • Genes are codominant
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29
Q

What HLA alleles are expressed in SLE?

A

DR2 and DR3

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30
Q

What HLA alleles are expressed in RA?

A

DR4

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31
Q

What HLA alleles are expressed in MS?

A

DR3, DR4, B7

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32
Q

What HLA alleles are expressed in Type 1 Diabetes?

A

DR3, DR4, B8

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33
Q

What HLA alleles are expressed in Ankylosing Spondylitis?

A

B27

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34
Q

During antigen processing in the Class I pathway, where do Ag and receptor associate?

A

In the ER

(intracellular pathogens)

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35
Q

During antigen processing in the Class I pathway, where do Ag and receptor associate?

A

In Vesicles

(Exogenous pathogens)

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36
Q

Response of Ag binding to TLRs

A
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37
Q

Which TLR binds lipopeptides?

A

TLR 1

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38
Q

Which TLR binds peptidoglycan, lipoteichoic acid, fungal wall components?

A

TLR 2

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39
Q

Which TLR binds dsRNA from viruses?

A

TLR 3

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40
Q

Which TLR binds LPS?

A

TLR 4

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41
Q

How to distinguish NK cells from T cells?

A

NK don’t have TCR/CD3 complexes

NK has Fc receptor CD16

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42
Q

What is the role of CD16 in NK cells?

A

It is the Fc receptor

Function:

Antibody-dependent cell-mediated cytotoxicity

(ADCC)

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43
Q

What are the inhibitory receptors present on NK cells and what do they engage?

A

Receptors:

  • CD 94
  • Killer cell Ig-like receptors

Engage Clss I proteins on normal cells

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44
Q

What do activating receptors of NK cells bind?

A

Infected or tumor cells

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45
Q

Which cytokines enhance NK activity? From which cell type are they secreted?

A

Enhanced by:

  • IL-12 (promotes IFN-gamma)
  • IL-15 (proliferation)
  • IFN-gamma
  • IL-1
  • IL-2

IL-12 and -15 are secreted by macrophages

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46
Q

How do NK cells lyse targets?

A

Perforin and granzymes –> apoptosis

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47
Q

Function of NKT cells and Ag type recognized

A

Kill cells expressing lipid Ags and promote CMI/Ab production

Ags: lipid/glycolipid Ag

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48
Q

Cellular vs Humoral immunity summary

A
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49
Q

Th1 cells

  • What cytokine induces polarization?
  • What cytokines are released?
  • What type of immunity is stimulated?
A
  • What cytokine induces polarization?
    • IL-12
  • What cytokines are released?
    • IL-2
    • TNFß
    • IFN-gamma
  • What type of immunity is stimulated?
    • CMI
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50
Q

Th2 Cells

  • What cytokine induces polarization?
  • What cytokines are released?
  • What type of immunity is stimulated?
A
  • What cytokine induces polarization?
    • IL-10
  • What cytokines are released?
    • IL-4, IL-13 –> IgE
    • IL-5 –> IgA
    • IFN-gamma –> IgG
  • What type of immunity is stimulated?
    • Humoral Immunity
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51
Q

Ig that functions as the B cell Ag receptor

A

IgM

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52
Q

Ig that can cross the placenta and is found in breast milk

A

IgG

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53
Q

IgG isotype that cannot opsonize

A

IgG2

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54
Q

Ig that is monomeric or pentameric, can opsonize and activate complement

A

IgM

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55
Q

Ig that gives mucosal immunity

A

Dimeric IgA (secretory)

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56
Q

Ig in which most are bound to mast cells

A

IgE

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57
Q

Where is MALT located? (tissue levels and organs)

A

Tissues:

  • Lamina propria
  • Submucosa

Organs:

  • Tonsils
  • Adenoids, Peyer’s patches
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58
Q

Characteristic cells of MALT

A

M cells: mediate Ag entry

Plasma cells: secrete IgA

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59
Q

Free cells of MALT

A
  • Lamina propria lymphocytes (activated CD4 cells)
  • B cells/Plasma cells (secrete IgA)
  • Transepithelial lymphocytes (CD8)
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60
Q

What percentage of WBCs are lymphocytes?

A

25-30%

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61
Q

Where do NKT cells mature?

A

Thymus

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62
Q

Cell surface proteins on B cells:

  1. Adhesion
  2. Signal Transduction
  3. Ag Presenting
  4. Ag Processing
A
  1. Adhesion
    • ICAM 1 (bind LFA–1 on T cells)
    • LFA3 (binds CD2 on T cells
  2. Signal Transduction
    • CD40 (binds CD40L on T cells)
    • B7 (binds CD28 on T cells)
    • CD21 (complement receptor)
  3. Ag Presenting
    • MHC II
  4. Ag Processing
    • Fc receptors
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63
Q

Cytokines produced by Regulatory B cells

A
  • IL-10
    • Supress CMI cytokines
  • TGF-ß
    • Inhibit Th1, Th2, Th17

Function: Suppress CMI

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64
Q

Cytokines produced by Effector B cells (Be)

A
  • Be-2 cells
    • IL-2, 4, 6, and TNF-alpha
    • Activate Tc, NK cells, mast cells, eosinophils, B and T cells
  • Be-1 cells
    • IL-12, IFN-gamma, TNF-alpha
    • Activate Tc cells, Th1 polarization, promote CMI

Overall function: stimulate CMI

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65
Q

Cytokines that stimulate B cell maturation

A

IL-3

IL-7

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66
Q

Cytokines that stimulate T cell maturation

A

IL-2

IL-7

Thymic Hormones

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67
Q

TCR accessory molecules

A

CD3 and zeta

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68
Q

Cell surface proteins on T cells:

  1. TCR
  2. Adhesion molecules
A
  1. TCR
    • CD3
    • zeta
    • CD28 (costimulatory, binds CD80 (B7) on APCs)
    • CD154 (CD40 L) (binds CD40 on APC)
  2. Adhesion
    • CD2 (binds LFA-3 on APCs)
    • LFA-1 (binds ICAM-1 on APCs and endothelium)
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69
Q

Th1 cells

  1. Stimulatory cytokines
  2. Inhibitory Cytokines
  3. cytokines secreted
  4. Function
A
  1. Stimulatory cytokines
    • IL-12
    • IFN-gamma
  2. Inhibitory Cytokines:
    • IL-4 (stimulate Th2)
    • IL-10 (Stimulate Th2)
    • IL-13 (secreted by Th2)
  3. cytokines secreted
    • TNFß, IFN-gamma
  4. Function
    • Promote CMI and isotype switch to IgG 1 and 3 (opsonization)
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70
Q

Th2 cells

  1. Stimulatory cytokines
  2. Inhibitory cytokines
  3. cytokines secreted
  4. Function
A
  1. Stimulatory cytokines
    • ​​IL-4
    • IL-10
  2. Inhibitory cytokines
    • IL-12 (stimulates Th1)
  3. cytokines secreted
    • IL-4, 5, and 13
  4. Function
    • B cell proliferation
    • Isotype switch
      • IL-4 and 13: IgE
      • IL-5: IgA
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71
Q

Th17 cells

  1. Stimulatory cytokines
  2. cytokines secreted
  3. Function
A
  1. Stimulatory cytokines
    • ​​IL-1
    • IL-6
    • TGF-ß
  2. cytokines secreted
    • ​​IL-17
    • IL-22
  3. Function
    • IL-17: recruitment and survival of neutrophils
    • IL-22: activates keritinocytes/fibroblasts (secrete IL-6 and IL-8)
    • Tissue inflammation
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72
Q

Cytokines that stimulate Tc cell development

A
  • IL-2
  • IFN-gamma
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73
Q

Cytokines released by Treg cells

A
  • IL-10
    • inhibits IL-12 to block Th1 development and CMI
  • TGF-ß
    • Inhibits T cell and macrophage activation
    • IgA production
    • Stimulates tissue repair (angiogenesis, connective tissue)
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74
Q

Percent of WBCs that are Monocytes/macrophages

A

1-6%

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75
Q

Cell surface proteins on Macrophages:

  1. Ag processing
  2. Receptors
A
  1. Ag processing
    • MHC I and II
    • C3b receptor (opsonization)
    • LFA-1 (binds ICAM-1 on other cells)
  2. Receptors:
    • CD14 (binds LPS-binding protein)
    • TLRs
    • Mannose receptors (bind PAMPs)
    • Hormone receptors
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76
Q

Functions of Macrophages

A
  1. Ag processing
  2. Control Th1 and Th2 polarization
    • Secrete IL-12 for Th1
    • Secrete IL-10 for Th2
  3. CMI
  4. Delayed-type hypersensitivity (DTH)
  5. Tissue damage/reorganization/healing
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77
Q

Interdigitating Dendritic Cells

A
  • After Ag encounter, migrate to draining node where they mature
    • Express high MHC lvls for Ag presentation

Summary:

  1. MHC I and II
  2. APCs
  3. Migratory
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78
Q

Follicular Dendritic cells

A
  • Express only MHC I
  • Not APCs
  • Non-migratory (stay in 1º and 2º follicles of lymph nodes, spleen and mucosal tissue)
  • Function: hold immune complexes in mucosal tissue
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79
Q

Characteristics of Type I Hypersensitivity

  1. Mediated by which Ig?
  2. Response by which cell type?
  3. Which type of antigen?
A
  1. IgE
  2. Th2
  3. Soluble
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80
Q

In Type 1 sensitivity, which cytokines are released to activate the sequence?

A
  • IL-4:
  • IL-10: (-) CMI
  • IL-13: (-) CMI
  • IL-9: activates mast cells

IgE isotype switching, Ab production

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81
Q

Cell types associated with Local Type I Rxns and Diseases

A
  • Cells: Mast Cells
  • Diseases:
    1. Utricaria
    2. Allergic Rhinitis
    3. Asthma
    4. Atopic dermatitis
    5. Food allergy
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82
Q

Cell types associated with Systemic Type I Rxns and Diseases

A
  • Cell Types: Mast cells and Basophils
  • Diseases: anaphylaxis
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83
Q

Mucosal vs Connective Tissue Mast Cells

  • Products
  • Granule contents
  • Location
A

Mucosal Mast Cells

  • Products: LTs and PGs (mostly PGD2); Tryptase and chymase
  • Granules: Chondroitin sulfate
  • Location: Submucosa of Respiratory tract and Intestines

Connective Tissue Mast Cells

  • Products: PGD2, Tryptase
  • Granules: Heparin
  • Location: Skin and Intestines
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84
Q

Mast cell activation and Degranulation mechanisms (Picture)

A
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85
Q

Mast cell activation and Degranulation mechanisms (Explanation)

A
  1. PIP2 –> DAG and Ca2+ –> PKC –> Phosphorylates myosin –> granule-membrane fusion and release
  2. High Ca2+ lvls and MAP kinase –> PLA2 –> hydrolyses PC to arachidonic Acid
    1. AA + COX = PG
    2. AA + Lipoxygenase = LT
  3. MAP Kinase + G protein –> adenylate cyclase –> increased cAMP –> PKA –> (-) degranulation

Summary: Ca2+ causes degranulation and PG/LT synthesis; cAMP causes inhibition of degranulation

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86
Q

Primary Mediators of Type I Rxns

A
  1. Histamine
  2. Chemotactic factors (eosinophils and neutrophils)
  3. Heparin/Chondroitin sulfate (storage matrices)
  4. Proteases (tryptase and chymase)
  5. TNF-alpha
  6. IL-6
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87
Q

H1 vs H2 receptors

A
  1. H1 Receptors
    • Increased vascular permeability (bronchial endothelial cells)
    • Smooth mm contraction
    • Increased mucus production
  2. H2 Receptors
    • Smooth mm relaxation (vascular smooth mm)
    • Vasodilation
    • Increased vascular permeability
    • Increased mucus production
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88
Q

Function of tryptase and chymase

A

Tryptase

  • From both mucosal and connective tissue mast cells
  • Bronchial hyperresponsiveness

Chymase

  • Connective tissue mast cells
  • Increased mucus production
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89
Q

What are the secondary mediators of Type I Rxns?

A
  1. LTs: LTC4, LTD4, LTE4
  2. PGs:
    • PGD2
    • PGE2
  3. Cytokines
    • IL-1, IL-6, TNF-alpha: Anaphylaxis/inflammation
    • IL-4 and -13: Th2 activity
    • IL-3, -5, GM-CSF: Eosinophil activity
    • Platelet activating factor: increased venule permeability
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90
Q

LT receptors: LT1 vs LT2

A

LT1

  • Increased vascular permeability
  • Bronchoconstriction

LT2

  • Endothelial activation
  • Macrophage activation
  • Chemokine release
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91
Q

Biphasic response of Type I Hypersensitivity:

Components of each phase

A

Phase I: Primary mediators (Histamine, Heparin/Chondroitin Sulfate, Typtase, chymase)

Phase II: Secondary mediators, Esosinophils, neutrophils

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92
Q

Characteristics of Anaphylaxis

A

Systemic, non-atopic Type I Rxn

  • Skin: Pruritus, erythema, Urticaria, angioedema
  • Vacular: Hypotension and shock
  • Respiratory: Bronchial obstruction from edema, bronchospasm, hyperexcretion of mucus
  • Blood: DIC
93
Q

What is pictured? In what reaction can it be present?

A

Pulmonary Edema

Anaphylaxis, Type I Rxn

94
Q

What is pictured? What type of reaction is it?

A

Urticaria

Local non-atopic Type I Rxn

95
Q

What is pictured? What type of reaction is it?

A

Angioedema (usually involved deep dermis)

Local non-atopic Type I Rxn

96
Q

Diagnostic Tests for Type I rxn

A
  1. RIST (sandwich ELISA for IgE concentration)
  2. RAST (normal ELISA for specific IgE to allergens): in vitro
97
Q

Define Atopy

A

An inherited abnormal state of hypersensitivity to an allergen with hyper-reactive target tissues

Serum IgE often elevated but not diagnostic

98
Q

Define Peak expiratory flow rate (PEFR)

A

Brief, forceful exhalation

reduced PEFR indicates airway narrowing

99
Q

Define Spirometry

A

Measures Forced expelled volume (FEV)

Indicates extent of airway obstruction

100
Q

Location and Clinical Manifestation of Allergic Rhinitis

A

Locations: nasal mucosa and conjuctiva

Clinical Manifestations

  • Pale, swollen nasal mucosa with watery secretions
  • Pruritic, hyperemic conjuctivae
  • Paroxysmal sneezing
  • Allergic shiners (lwr eyelid ecchymoses from eye rubbing)
  • Nasal crease (from nose wiping)
101
Q

Extrinsic vs Intrinsic Asthma

  • Serum tests
  • Results of skin test
  • Family History
A

Extrinsic: response to allergens

  • IgE and allergen-related
  • Elevated serum IgE
  • Skin tests positive
  • Family history

Intrinsic: Vagal response to irritants

  • Normal serum IgE
  • Eosinophilia
  • Skin test negative
  • No family history
102
Q

Characteristics of Early bronchial reaction in asthma and mediator cells

A
  • Mast cells and eosinophils
  • Smooth mm contraction
  • Vasodilation
  • Edema
  • Increased mucous production
103
Q

Characteristics of Late bronchial reaction in asthma and mediator cells

A
  • Eosinophils and neutrophils
  • Cough produces thick sputum
  • Thick mucus plugs
  • smooth muscle hyperplasia and hypertrophy, eventual lumen obstruction (chronic)
104
Q

What is pictured? In which illness is it associated?

A

Inflammed bronchus of Late Asthmatic Reaction

  • Thickened basement membrane (left arrow)
  • Smooth muscle hyperplasia (Bottom arrow)
  • Increased mucus (top arrow)
105
Q

Characteristics of atopic dermatitis

A
  • Elevated serum IgE
  • Staph infections common
  • Acute lesions
    • vesicular, erythematous, pruritic
    • weeping and crusting with itching
    • On face and flexor regions
  • Chronic lesions
    • Dry and scaly
    • Epidermal hyperplasia and hyperkeratosis
106
Q

What is pictured?

A

Atopic Dermatitis

107
Q

In Type II Hypersensitivity,

  1. What types of Ig are involved?
  2. What type of antigen?
A
  1. IgG, IgM
  2. bound to cell or matrix

*Causes cellular dysfunction

108
Q

What is MCV?

A

Mean Corpuscular Volume: Size of RBCs

If high = Macrocytic

If low = Microcytic

109
Q

What does hemoglobin measure?

A

Oxygen carrying capacity of the blood

110
Q

What does hematocrit measure?

How is it calculated?

A

Oxygen carrying capacity of the blood

Hct% = (RBC count x MCV) / 10

111
Q

Characteristics of hemolytic anemia

(blood labs, symptoms)

A

Blood:

  1. Increased Hemoglobin
  2. Bilirubinemia
  3. reticulocytosis
  4. Spherocytosis

Clinical Symptoms:

  1. Jaundice
  2. Splenomegaly (filtering RBCs)
  3. Fever (platelet release of chemokines upon lysis)
112
Q

What is the process that leads to reticulocytosis in hemolytic anemia?

A

Decreased O2 in kidney –> Increased erythropoietin (EPO) –> erythroid hyperplasia –> reticulocytosis

113
Q

Erythroblastosis Fetalis

  1. Type of hemolytic anemia
  2. Associated Ig
  3. Treatment
  4. Prevention
A
  1. Warm
  2. IgG (can cross placenta)
  3. Transfusions during third trimester
  4. anti-Rh Ab to mother w/i 72 hours after birth of first child

Type II Hypersensitivity

114
Q

Ig associated with:

  1. Erythroblastosis Fetalis
  2. Autoimmune cold HAs
  3. Secondary cold HAs
  4. Paroxysmal cold HAs
A
  1. IgG
  2. IgM
  3. IgM
  4. IgG
115
Q

Characteristics of Immune thrombocytopenia

  1. Ig
  2. Counts
  3. Clinical features
A
  • Anti-platelet IgG
  • <100,000
  • Features:
    • Echymoses and petechiae
    • Nose bleeds
    • Hemorrhagic bullae
    • Anemia (blood loss)
    • Fever/chills (mediators released following lysis)

Type II Hypersensitivity

116
Q

Anti-GBM Nephritis

  • Type of Hypersensitivity
  • To what does the Ab bind?
  • What are the clinical manifestations?
  • What is the pathological change?
A
  • Type II Hypersensitivity
  • Ab to type IV collagen of GBM
  • Acute Nephritic Syndrome
    1. Macroscopic hematuria
    2. Proteinuria
    3. Retention of H2O and Na+
      • Edema
      • HTN
  • Rapidly Progressive (crescentic) Glomerulonephritis (RPGN)
    1. GBM ruptures
    2. Crescents of parietal cells
    3. Bowman space obliterated
117
Q

Goodpasture’s Syndrome

  • Type of Hypersensitivity
  • To what does the Ab bind?
  • What are the clinical manifestations?
  • What is the pathological change?
A
  • Type II
  • ABM and GBM
  • Pulmonary Symptoms/pathology
    • occurs first
    1. Hemoptysis (Pulmonary hemorrhage
    2. Dyspnea
  • Renal Symptoms/pathology
    • Rapidly Progressive Glomerulonephritis
      • GBM ruptures (hematuria, proteinuria)
      • Crescents of parietal cells
      • Bowman space obliterated
118
Q

Myasthenia Gravis

  • Type of Hypersensitivity
  • To what does the Ab bind?
  • What are the clinical manifestations?
  • What is the pathological change?
A
  • Type II
  • ACh receptors (increases endocytosis of receptors)
  • Clinical:
    • Muscle weakness
    • Occurs with Thymoma (thymic hyperplasia)
  • Path:
    • Damage to postsynaptic membrane
119
Q

Grave’s Disease

  • Type of Hypersensitivity
  • To what does the Ab bind?
  • What are the clinical manifestations?
  • What is the pathological change?
A
  • Type II
  • TSH receptor; stimulates production of thyroid hormone
  • Path/Clinical:
    • Epithelial hyperplasia (goiter)
    • Ophthalmopathy involving periorbital edema early followed by fibrosis (bug eyes)
120
Q

Bullous Pemphigoid

  • Type of Hypersensitivity
  • To what does the Ab bind?
  • What are the clinical manifestations?
  • What is the pathological change?
A
  • Type II
  • adhesion protiens at dermal-epidermal junction
  • Path/Clinical
    • Dermal-epidermal separation by eosinophils/lymphocytes
    • Fluid-filled blisters
121
Q

Pemphius Vulgaris

  • Type of Hypersensitivity
  • To what does the Ab bind?
  • What are the clinical manifestations?
  • What is the pathological change?
A
  • Type II
  • epidermal cell adhesion molecules
  • Path/Clinical
    • Acantholysis (lysis of intercellular adhesion sites)
    • Flaccid blisters that rupture and crust over
122
Q

Pernicious Anemia

  • Type of Hypersensitivity
  • To what does the Ab bind?
  • What are the clinical manifestations?
  • What is the pathological change?
A
  • Type II
  • Auto-Abs:
    • Block binding of B12 to intrinsic factor
    • Block binding of intrinsic factor to ileal cells
  • Path:
    • parietal cell-specific CD4 T cells
      • Loss of parietal cells
        • reduced IF
        • Reduced Acid
    • Auto-Abs:
      • reduced IF and B12
        • Megaloblastic anemia from imparied hemopoeisis
  • Clinical:
    • Bone marrow hypercellular
    • Gastritis
    • Demylination with spastic paraparesis
123
Q

What is pictured?

A

Megaloblastic anemia, Megaloblasts, associated with Pernicious anemia

124
Q

What is pictured?

A

Spherocytosis, associated with Hemolytic anemia

125
Q

Acute Rheumatic Fever/Heart disease

  • Type of Hypersensitivity
  • To what does the Ab bind?
  • What are the clinical manifestations?
  • What is the pathological change?
A
  • Type II
  • ABs to S pyogenes cross-react with heart
  • Aschoff’s nodules
    • inflammatory cells/giant cells surrounding fibrous necrosis
  • Rheumatic valvular disease (vegetations)
    • cause murmur
  • Exudative inflammation
    • pericarditis, myocarditis, endocarditis
  • Polyarthritis (neutrophils)
  • Sydenham’s Chorea (limbs, facial mm)
126
Q

What is pictured?

A

Aschoff body, associated with Acute Rheumatic fever

127
Q

Characteristics of Type III Hypersensitivity

A
  • Ab types: IgG, IgM
  • Immune complex formation, inflammation, tissue damage
128
Q

What is the Arthus reaction?

A
  • Model that explains how immune complexes initiate tissue damage
  • Immune complexes form
  • Acomplement activated
  • Mast cells degranulate
  • Result:
    • Vasculitis
    • Erythema
    • Edema
    • Hemorrages
129
Q

Polyarteritis Nodosa

  • Type of Hypersensitivity
  • Onset
  • What are the clinical manifestations?
  • What is the pathological change?
A
  • Type III
  • Onset: with infections (HBV, TB, Strep)
  • Systemic vasculitis that spares pulmonary circulation
  • Morphology
    • Necrotizing inflammation
    • Mixed infiltrate
    • Fibrinoid necrosis causes vessel wall thickening and occlusion of lumen –> ischemia
    • ***All stages of inflammation occur at same time***
  • Clinical manifestations: associated with low O2
130
Q

What is pictured?

A

Polyarteritis nodosa

vasculitis w/ occlusion and necrosis of wall

131
Q

Serum Sickness

  • Type of Hypersensitivity
  • Onset
  • What are the clinical manifestations?
A
  • Type III
  • Onset: follows large dose of high molecular weight compound
  • Symptoms:
    1. Pruritic rash first
    2. fever
    3. arthritis
    4. myalgia
    5. GN
    6. lymphadenopathy
    7. splenomegaly
132
Q

Immue Complex Glomerulonephritis

  • Type of Hypersensitivity
  • Common finding of group
  • Pathogenesis
  • Clinical Syndromes
A
  • Type III
  • Granular IC deposits
  • Path: Either
    • In situ rxn of Ab w/ Ag
    • Deposition of circulating IC
  • Clinical:
    • Nephritic syndrome (mild proteinuria (>150mg)
    • Nephrotic syndrome (extensive proteinuria (>3.5g) and hematuria
133
Q

Acute Diffuse Proliferative GN

  • Type of Hypersensitivity
  • Onset
  • What is the pathological change?
  • What are the clinical manifestations?
A
  • Type III, IC-GN
  • Onset
    • Post-infectious (strep)
    • Non-infectious (lupus)
  • Path:
    • Diffuse Hypercellularity
    • Subepithelial deposits = humps
    • Granular deposits w/immunofluorescence
  • Clinical:
    • Children, post-Inf: nephritic syndrome (hematuria, RBC casts, hypocomplementemia)
    • Others: RPGN/Chronic GN
134
Q

Rapidly Progressive (crescentic) GN

  • Type of Hypersensitivity
  • Onset
  • What is the pathological change?
  • What are the clinical manifestations?
A
  • Type III, IC-GN
  • immune-mediated
  • Path:
    • Subepithelial ICs
    • GBM thickened and ruptured
  • Clinical:
    • Allows inflammatory cells in urinary space (appear in urine?)
135
Q

Membranous GN

  • Type of Hypersensitivity
  • Onset
  • What is the pathological change?
  • What are the clinical manifestations?
A
  • Type III, IC-GN
  • Autoimmune, drug induced, tumor Ags, lupus
  • Path:
    • Subepithelial deposits as “spikes”
    • Thickened, irregular GM that grows over deposits
    • Universal GBM thickening = “wire loops”
  • Clinical:
    • Nephrotic syndrome
    • Hematuria
136
Q

Membranoproliferative GN

  • Type of Hypersensitivity
  • Onset
  • What is the pathological change?
  • What are the clinical manifestations?
A
  • Type III, IC-GN
  • Onset:
    • Type I Primary: Subendothelial immune deposit, mesangial immune deposit
    • Type II Primary: Intramembranous immune deposit
    • Secondary: Subendothelial deposits caused by infection/autoimmune disease/neoplasm
  • Path:
    • Hypercellularity
    • GBM thickened with “double contour”
    • Cell components occur between GBMs “interposed mesangial cell process”
  • Clinical:
    • Nephritic or nephrotic syndrome
137
Q

What is pictured?

A

Type II Primary Membranoproliferative GN

Intramembranous immune deposits

138
Q

What is pictured?

A

Type I Primary Membranoproliferative GN

Subendothelial IC deposits

139
Q

What is the difference between type I and type II primary membranoproliferative GN?

A

Type I:

  • Subendothelial immune deposits; mesangial immune deposits

Type II:

  • Intramembranous immune deposits
140
Q

Two types of Type IV Hypersensitivity (explanation)

A

Delayed Type Hypersensitivity (DTH)

  • Kills extracellular microbes
  • Th1 secrete IFN-gamma to activate Macrophages
  • Th17 secrete IL-17 and -22 Stimulate neutrophils, fibroblasts, keratinocytes

Cell-Mediated Immunity (CMI)

  • Kills intracellular microbes (viruses) and tumor cells
  • Th1 secretes IFN-gamma and IL-2 –> activate Tc and NK cells
141
Q

DTH mechanism

A

Tuberculin-type Hypersensitivity reaction

  1. Sensitization: initial recognition, produces memory Th1 cells
  2. Elicitation phase: 2nd encounter, Induration (vasc. perm. and leakage of fibrinogen
  3. Resolution/Chronic inflammation
    • Granuloma formation (epithelioid) and healing by Fibrosis
142
Q

CMI mechanism

143
Q

Allergic Contact Dermatitis

A
  • Type IV DTH hypersensitivity
  • Elicitation: basophils, eosinophils, monocytes
  • Symptoms:
    • Acute: highly pruritic vesicular rash
    • Chronic: Pruritic, crusty rash
  • Diagnosis: Patch test
  • Treatment: corticosterioids
144
Q

Mechanisms of cytotoxicity (Tc cells)

A
  1. Granzyme/perforin - apoptosis or necrotic cell lysis
  2. Fas Ligand (Tc) - Fas - Apoptosis
  3. TNF-ß (lymphotoxin) (Tc) - Apoptosis
145
Q

Cytokines that induce Tc maturation

A
  1. Bind Class I MHC w/IL-2 and IFN-gamma: becomes cytolytic
  2. IL-12: Increases cytolytic activity
146
Q

Direct vs Indirect pathway of graft rejection

A

Direct:

  • react to MHC on donor cells
  • Donor dendritic cells most important (MHC I and II, B7)

Indirect:

  • Donor Ag presentation by recipient’s dendritic cells
147
Q

Characteristics of Hyperacute Graft rejection

  • Time frame
  • Cause
  • Result
A
  • w/i minutes to hours
  • Pre-existing Abs to MHC or mismatched blood type
  • Damaged endothelium
    • thrombosis
    • infarction
148
Q

Characteristics of Acute Humoral Graft rejection

  • Time frame
  • Cause
  • Result
A
  • Few days to a couple of weeks
  • Ab to endothelial Ags
  • Results:
    • necrotizing vasculitis (Thickened vessel wall)
    • narrowing of the lumen
    • infarction
149
Q

Characteristics of Acute Cellular Graft rejection

  • Time frame
  • Cause
  • Result
A
  • Not stated
  • T cell infiltrate responding to foreign Abs
  • Results:
    • Endothelial injury
    • Invasion of tubules
    • Necrosis
150
Q

Characteristics of Chronic Graft rejection

  • Time frame
  • Cause
  • Result
A
  • Months to years
  • Not stated
  • Results:
    • Kidney: fibrosis, tubular atrophy
    • Vasculature: Intimal fibrosis, smooth mm proliferation
151
Q

Characteristics of Graft vs Host Disease

  • Most common in which type of transplant?
  • Cause
  • Symptoms
  • Treatment
A
  • Hematopoietic cell transplant
  • Donor immune cells mount response to recipient
  • Symptoms:
    1. Rash
    2. Jaundice (destroys small bile ducts)
    3. Bloody diarrhea (mucosal ulceration)
  • Treatment:
    1. Corticosteroids and cyclosporin
    2. use of self stem cells
152
Q

Characteristics of Host vs Graft Disease

  • Most common in which type of transplant?
  • What is it?
A
  • Hematopoietic cell transplant
  • Residual NK and T cells in recipient attack transplant
153
Q

What are the most likely infections in Prolonged Imunodeficiency?

A
  1. CMV (overall)
  2. Adenovirus (children)
154
Q

What are the most common diseases that recur after a kidney transplant?

A

Membranous GN

Membranoproliferative GN

155
Q

Types of rejection in kidney transplant

A
  1. Acute Cellular
  2. Acute Humoral
  3. Chronic Rejection
    • fibrosis
    • Mononuclear infiltrate
    • Tubular atrophy
156
Q

Characteristics of Chronic rejection in Heart Transplant

A
  • Arteriopathy = narrowing of lumen
    • Intimal proliferation
    • Distal epicardial and intramyocardial coronary artery branches
157
Q

Common recurrent disease in heart transplants?

A

Accelerated atherosclerosis

158
Q

Types of Rejections in Liver transplants and Characteristics

A
  1. Acute cellular
    • Portal inflammation
    • Bile duct injury
    • Endothelial inflammation in portal/central veins
  2. Chronic rejection
    • Arteriopathy
159
Q

Common Recurrent disease in Liver Transplant

A
  1. Hep B
  2. Hep C
160
Q

Characteristics of Post-transplantation Lymphoproliferative Disorder (PTLD)

A
  • EBV-induced B cell Proliferation
  • Associated with immunosuppression
161
Q

What are long term complications in transplantation?

A
  1. Post-transplantation lymphoproliferative disorder
  2. leukemia
  3. Hodgkin’s and non-Hodgkin’s lymphomas
  4. Infection
162
Q

Function of AIRE

A

(Autoimmune Regulatory Protein)

  • stimulates expression of peripheral Ags in thymus for (+)/(-) selection (central tolerance)
163
Q

Characteristics of Autoimmune Polyendocrine Syndrome

  • Cause
  • Affected systems
A
  • Cause:
    • mutation of AIRE (central tolerance)
  • Affected systems:
    • Endocrine
    • Skin
164
Q

Mechanisms of Peripheral Tolerance in self-reactive cells

A
  1. Induced to be anergic by Ag-presentation w/o costimulatory signals
  2. Suppressed by T regs by cytokine secretion (IL-10, TGF-ß)
  3. Removed by apoptosis
    • Persistent activation
    • Fas-mediated (T regs)
165
Q

What is epitope spreading? What does it cause?

A
  • constant exposure of different, normally hidden, epitopes due to tissue damage
  • Causes autoimmune disease
166
Q

Types of auto-Abs present in Lupus, association with active disease

A
  1. Anti-nuclear
  2. Anti-dsDNA (Active disease)
  3. anti-RBC (+ COOMBS test)
  4. anti-phospholipid
167
Q

What is the pathogenesis of Lupus?

A

Auto-Abs from:

  1. Failure of peripheral tolerance
  2. Dysfunctional Tregs
168
Q

Locations of IC deposition and resulting symptoms

A
  1. Skin
    • Malar rash (butterfly, over nose)
    • Discoid rash (erythematous raised patches
    • Photosensitivity
  2. Joints
    • Arthritis (2 or more joints)
  3. Kidney
    • proteinuria
    • Cellular casts
    • Mesangial GN
    • Proliferative GN
    • Membranous GN
  4. Serosal Membranes
    • Pleruitis
    • Pericarditis
  5. Other: Hematological
    • Anemia
    • Leukopenia
    • Thrombocytopenia
    • Hypergammaglobulinemia
    • Hypocomplementemia
169
Q

Characteristics of Mesangial GN

A
  • Mesangial proliferation w/IC deposits
  • mild symptoms: Nephritic
170
Q

Focal vs Diffuse proliferative GN

A

Both:

  • Endothelial and mesangial proliferation

Focal:

  • <50% glomeruli affected
  • Variable symptoms (nephritic or nephrotic)

Diffuse:

  • Most glomeruliaffected
  • Nephrotic
  • Subendothelial deposits
171
Q

Characteristics of Chronic Discoid Lupus

A
  • Skin lesions w/o systemic symptoms
  • ANA w/o anti-dsDNA
172
Q

Characteristics of Subacute Cutaneous Lupus

A
  • Skin lesions w/o systemic symptoms
  • ANA w/o anti-dsDNA
173
Q

Drug-induced Lupus

A
  • Multiple organs affected
    • Not CNS or kidney
174
Q

Differential Diagnosis with positive Anti-nuclear Ab test?

A

CREST:

  • Calcinosis
  • Raynaud’s
  • Esophageal motility disorder
  • Sclerodactyly (scleroderma)
  • Telangiectasia (dilation of vessels)

And LUPUS

175
Q

What auto-Ab causes a positive Coombs test?

A

Anti-RBC Ab

176
Q

What allele increases risk of developing rheumatoid arthritis?

177
Q

Symptoms of Rhematoid arthritis

A
  1. Symmetric stiffness (starts in small joints)
  2. Rheumatoid nodules
  3. Vasculitis
  4. Lung: pneumonitis/pleurisy
178
Q

Pathology of Rheumatoid Arthritis

A
  1. Rheumatoid factors initiate IC formation in synovium
    • IgMs to Fc of IgG or anti-citrullinated peptide Ab
    • due to defective CD4+CD25+ Tregs
  2. Th17 cells release IL-17: neutrophil recruitment
  3. Th1 cells activate macrophages
    • by secretion of IL-1 and TNF-alpha, PGE2 and LTB4
  4. IL-1 and TNF-alpha activate synovial cell proliferation
  5. Osteoclasts resporb bone
  6. Synovium thickens and connective tissue pannus replaces cartilege
179
Q

Sjogren’s Syndrome

  • Pathology
  • Clinical Symptoms
A
  • Pathology
    • CD4 cells and Igs reactive to glands
    • Lymphocytic infiltrate (B cells, plasma cells, CD4)
    • Hyperplasia of duct lining occludes duct
    • Fibrosis, atrophyof acini, replacement of parynchyma with fat
  • Clinical Symptoms
    • Chronic dry eyes (assess with Schirmer test)
    • Dry mouth (difficulty swallowing, fissures, caries)
    • enlargement of salivary glands
180
Q

What is Mixed Connective Tissue Disease?

A

Coexistence of SLE, polymyositis, RA and systemic sclerosis

181
Q

Progressive Systemic Sclerosis (scleroderma)

  • Pathology
  • Clinical Symptoms
A
  • Pathology
    • Collagen-vascular disease
    • fibrosis in skin and other organs
  • Clinical Symptoms
    • Raynaud’s
    • Cutaneous fibrosis
  • Symptoms of DIffuse Scleroderma
    • Skin
    • Connective dissue infiltration
      • GI = malabsorption syndrome
      • joints = arthritis
      • Lungs = pulmonary fibrosis
      • Heart = cardiac arrhythmia
    • CREST Syndrome
182
Q

What is CREST syndrome?

A

Calcinosis

Raynauds

Esophageal dysmotility

Sclerodactyly

Telangiectasia (vessel dilation)

183
Q

Ab present in Limited scleroderma

A

anti-centromere Ab

184
Q

Antibody present in Diffuse Scleroderma

A

anti-Scl 70 (DNA topoisomerase)

185
Q

Polymyositis-Dermatomyositis

  • Pathology
  • Clinical Symptoms
A
  • Pathology
    • Increased MHC I and II on skeletal muscle cells
      • atrophy and necrosis
    • Dermatitis from IC-mediated vasculitis
  • Clinical Symptoms
    1. Symmetric proximal mm weakness/pain
    2. Muscle enzymes in blood
    3. Creatine in Urine
    4. Rash and photosensitivity
    5. Hypergammaglobulinemia
      • Rheumatoid factor or anti-nuclear Abs
186
Q

Primary Immunologic Deficiency Syndromes

  • Cause
  • Common symptoms
A
  • Cause: Genetic
  • Common symptoms:
    1. skin lesions
    2. chronic diarrhea
    3. impaired growth
    4. failure to thrive
187
Q

Markers for B and T cells in counting

A

B cells: CD 19, 20, sIg

T cells: CD 2, 3, 5

188
Q

What does a positive DTH skin test mean?

A

CMI anergy

189
Q

What does a positive NBT test mean? What does a negative and positive result look like?

A

Positive test:

  • blue-black
  • functional NADPH oxidase

Negative test:

  • yellow
190
Q

What does the flow cytometric assay of dihydrorhodamine test?

A

NADPH oxidase activity (replaces NBT test)

191
Q

Defect and genetic cause of X-linked SCID

A
  • Defect
    • No T or NK cells
    • Non-functional B cells
  • Cause:
    • IL2 receptor gamma chain
      • **IL-7 receptor (T cell maturation)
192
Q

Chronic Granulomatous Disease

  • Type of Immunodeficiency
  • Molecular defect
  • Symptoms
  • Treatment
A
  • Intrinsic Phagocyte deficiency
  • Molecular defect:
    • Defective NADPH oxidase complex
    • No ROIs
  • Symptoms:
    • lymphadenopathy
    • skin lesions
    • sinopulmonary infections
  • Treatment:
    • IFN-gamma
193
Q

Defect and cause of Wiskott-Aldrich syndrome

A
  • Defect:
    • defective B and T cells
  • Cause:
    • WAS gene defect
      • codes for signal transduction in hematopoietic stem cells
194
Q

X-linked agammaglobulinemia

  • Type of Immunodeficiency
  • Defect
  • Cause
  • Symptoms
A
  • B cell immunodeficiency
  • Defect
    • Failure of B cells to mature
    • **No B, Normal T
  • Cause
    • defective B cell tyrosine kinase gene (BTK)
  • Symptoms:
    • Recurrent infections: eye, ear, teeth, rash
    • Ig < 250
  • Treatment: IV Ig
195
Q

Cause of MHC Class I deficiency

A

defect in TAP2

196
Q

Type 1 leukocyte adhesion deficiency

  • Type of Immunodeficiency
  • Defective molecules
  • Symptoms
A
  • Phagocyte deficiency
  • Defective molecules
    • LFA-1
    • Mac-1 (CR3)
  • Symptoms:
    • Defective chemotaxis/cytotoxicity
    • recurrent skin, vaginal, sinopulmonary infections
197
Q

Autosomal SCID

  • Causes
  • Cells absent
A
  • Causes:
    1. Adenosine deaminase deficiency
      • ​​(-) ribonucleotide reductase –> (-) lymphoid stem cell development
    2. Purine nucleoside phosphorylase deficiency
    3. Recombination-activating gene (RAG) mutations
    4. Jak3 defects
  • Cells absent:
    • B and T cell maturation inhibited
198
Q

What is the cause of Common variable immunodeficiency?

A

failure of B cell differentiation into plasma cells

199
Q

What are selective immunodeficiencies? What is the most common type?

A

Defects in isotype switching

IgA

200
Q

Selective IgA Deficiency

  • Type of immunodeficiency
  • Cause
  • Symptoms
A
  • B cell immunodeficiency
  • Cause:
    • Isotype switch failure
    • Secretion failure
  • Symptoms:
    • recurrent infections:
      • Sinopulmonary
      • GI
    • IgA lvls <5-7mg/dl
    • No secretory IgA
201
Q

Hyper-IgM Syndrome

  • Type of immunodeficiency\
  • Cause
  • Cell types present
  • Symptoms
A
  • B cell immunodeficiency
  • Cause:
    • T cells fail to produce cytokines for isotype switch
    • defects in CD40 (B cells)
    • Defects in CD40L (T cells)
  • Cells:
    • Th1 and Th2 defect
  • Symptoms:
    • Ab and CMI defeciency
    • Pyogenic infections
    • Intracellular microbe infections
202
Q

Common Variable ID

  • Type of immunodeficiency
  • Cells present
  • Symptoms
  • Treatment
A
  • B cell immunodeficiency
  • No plasma cells
  • Symptoms:
    • Recurrent sinopulmonary infection
    • Ig lvl < 300
203
Q

DiGeorge Syndrome

  • Type of immunodeficiency
  • Cause
  • Symptoms
  • Treatment
A
  • T cell Immunodeficiency
  • Cause:
    • deletion of 22q11
    • failure of development of 3rd and 4th pharyngeal pouches
      • thyroid and parathyroid hypoplasia
  • Symptoms:
    • hypocalcemia
    • congenital heart disease
    • Chronic infection
    • T cell deficiency
  • Treatment:
    • marrow or stem cell transplant
204
Q

Paroxysmal Nocturnal Hemoglobinuria

  • Type of immunodeficiency
  • Cause
  • Symptoms
A
  • Complement deficiency
  • Cause:
    • Deficiency in DAF and CD59 (Mac inhibitor)
  • Symptoms:
    • pancytopenia: reduction in the number of red and white blood cells, as well as platelets
205
Q

Hereditary angioedema

  • Type of immunodeficiency
  • Deficiency
A
  • Complement Deficiency
  • C1INH deficiency
206
Q

What type of infections are commonly associated with Complement Immunodeficiency? What is the specific defect involved?

A

Neisseria infection

defects in MAC

207
Q

How is HIV transmitted from mother to fetus?

A
  1. Transplacentally
  2. During delivery (most common in US)
    • give anti-retroviral therapy to mother
  3. Breast milk
208
Q

What are the envelope proteins of HIV? What are their functions?

A
  1. gp120
    • binds CD4 and CCR5 or CXCR4 (initiate entry)
  2. gp41
    • Induce HIV-host membrane fusion
209
Q

Pathogenesis of HIV infection

A

Primary target: mucosal CD4 T cells

210
Q

Mechanisms of CD4 loss in HIV

211
Q

Patient presents with fever, lymphadenopathy, and rash. CD4 counts <800. What does he have?

A

Stage I HIV: Acute infection

212
Q

Patient presents with lymphadenopathy and splenomegaly. Has had a fever and oral infections. CD4 count 200-499/microL. What does he have?

A

Stage 2 HIV: Chronic phase

213
Q

Patient presents with opportunistic infections and CD4 count <200. What does he have?

A

Stage 3 AIDS: Crisis phase

214
Q

What opportunistic infections occur in stage 2 (chronic phase) HIV?

A

Oral Infections

  1. Oral Candidiasis
  2. Oral Hairy cell leukoplakia
  3. EBV infection
215
Q

What serologic test can diagnose HIV?

A

ELISA for IgM to gp24 and gp120

develop 2-8 weeks after infection

216
Q

Staining techniques and results for amyloidosis

A
  1. Congo red stain = RED
  2. Polarized light = yellow/green birefringence
217
Q

Characteristics of Primary (immunocytic) amyloidosis

  • Amyloid protein
  • Major organ affected
A
  • Ig/AL light chains (AL amyloid)
    • from neoplastic plasma cells
  • Affects heart
218
Q

Amyloid protein associated with Secondary (reactive) amyloidosis

A

Serum amyloid-associated protein A

(AA amyloid)

Secondary to chronic inflammation

219
Q

Characteristics of Heriditary (familial) amyloidosis

  • Amyloid protein
  • Major organ affected
A
  • Mutant Transthyretin (TTR)
  • Affects Heart
220
Q

What disease is associated with deposition of a mutant form of transthyretin (TTR)? Deposition of normal TTR?

A

Mutant TTR: Hereditary (familial) amyloidosis

Normal TTR: senile amyloidosis

221
Q

Characteristics of Senile amyloidosis

  • Deposit
  • Major organ affected
A
  • Normal transthyretin (TTR)
  • Major organ: Heart
    • CHF
    • Ventricular dysfunction
    • Valvular insufficiency
    • Ischemia
222
Q

Amyloid protein build-up associated with long-term dialysis?

A

ß2 microglobulin

223
Q

Amyloid protein associated with Alzheimer’s disease

A

ß-amyloid (Aß)

224
Q

Name the disease:

Bence Jones proteins

AL protein

A

Primary (immunocytic) amyloidosis

associated with plasma cell dyscrasias, such as multiple myloma

225
Q

Disease associated with secondary (reactive) amyloidosis

A

RA

(chronic inflammation, cellular necrosis)

226
Q

Where does ß2 microglobulin deposit in hemodialysis-associated amyloidosis?

A

Joints and bones

227
Q

Characteristics of Familial Mediterranean Fever

  • amyloid deposit
  • symptoms
A
  • Deposit
    • AA protein
  • Symptoms
    • Fever
    • serosal inflammation (pleura, peritoneum, synovial membranes)
228
Q

Characteristics of Familial amyloid polyneuropathies

  • Deposit
  • Symptoms
A
  • Deposit:
    • Mutant transthyretin deposits in nerves
  • Symptoms:
    • progressive peripheral and autonomic neuropathy
229
Q

Amyloid protein associated with Isolated atrial amyloidosis

Pathology (27 decks)

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