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PATHOMA > Ch. 2 - Inflammation, Inflammatory Disorders, and Wound Healing > Flashcards

Ch. 2 - Inflammation, Inflammatory Disorders, and Wound Healing Flashcards

1
Q

ANCA Associated Vasculitis involves the activation of {{c1::neutrophils}} by auto-antibodies leading to inflammation of the blood vessel wall.

A

neutrophils

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2
Q

Antiphospholipid Antibody Syndrome (APS) typically involves which 2 autoantibodies?

A

Anticardiolipin and Lupus Anticoagulant

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3
Q

which Ab can give you a false positive syphilis test?

A

Anticardiolipin

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4
Q

what Ab can give you a falsely elevated PTT?

A

lupus anticoag

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5
Q

Deficiency of which early complement proteins is associated with SLE?

A

C1q; C2; C4

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6
Q

What is the function of early complement proteins?

A

Early complement proteins are involved with opsonization of exposed nuclear material from apoptosis (poorly cleared apoptosis).

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7
Q

How does ESR change in inflammation?

A

Increase
Products of inflammation (e.g. acute phase reactants) coat RBCs and cause aggregation. This aggregation causes RBCs to fall at a faster rate within the test tube (i.e. increased rate of sedimentation).

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8
Q

How does the risk for B-cell (marginal zone) lymphoma change in Sjogren Syndrome?

A

Increased

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9
Q

what presents as unilateral enlargement of the parotid gland?

A

B cell marginal zone lymphoma in Sjogrens

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10
Q

How long after inflammation begins do macrophages predominate in the tissue?

A

Peak after 2-3 days

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11
Q

How long after inflammation do neutrophils undergo apoptosis?

A

Within 24 hrs

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12
Q

In leukocyte diapedesis during inflammation, (blank) on endothelium binds to (blank) on leukocytes.

A

PECAM-1

CD31

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13
Q

In leukocyte margination and rolling, (blank and blank) on endothelium binds to (blank) on leukocytes.

A

E-selectin and P-selectin bind to Sialyl Lewis X

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14
Q

In leukocyte margination and rolling, (blank) on endothelium binds to (blank) on leukocytes.

A

GlyCAM-1 and CD34 on endo

L-selectin

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15
Q

In leukocyte tight-binding during inflammation, (blank) on endothelium binds to (blank) on leukocytes.

A

ICAM CD54 on endo

CD11/18 integrins (LFA1/Mac1)

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16
Q

In leukocyte tight-binding during inflammation, (blank) on endothelium binds to (blank) on leukocytes.

A

VCAM CD106 on endo

VLA-4 integrin leuko

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17
Q

Polymorphisms in which cell surface protein on regulatory T cells are associated with autoimmunity (e.g. MS and T1DM)?

A

CD25

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18
Q

What ANA is indicative of diffuse Systemic Scleroderma?

A

Scl-70

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19
Q

What ANA is indicative of Drug-induced Lupus Erythematosus?

A

anti-histone

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20
Q

What ANA is indicative of limited Systemic Scleroderma (i.e. CREST Syndrome)?

A

anti-centrisome

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21
Q

What ANAs are indicative of Sjogren’s Syndrome?

A

SS-A/Ro and SS-B/La

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22
Q

What ANAs are indicative of Systemic Lupus Erythematosus (SLE)?

A

Anti-dsDNA or nonspecific ANA

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23
Q

What are the 2 complement components that function as anaphylatoxins?

A

C5a and C3a

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24
Q

What are the 4 major chemoattractants for Neutrophils?

A

“CILK”

  • C5a
  • IL-8
  • LTB4
  • Kinin
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25
Q

What do the letters mean in CREST?

A

CREST:

  • Calcinosis & anti-Centromere antibodies
  • Raynaud phenomenon
  • Esophageal dysmotility
  • Sclerodactyly
  • Telangiectasia of the skin
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26
Q

What is a colourless nitroblue tetrazolium (NBT) test indicative of?

A

NADPH Oxidase defect

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27
Q

What is a Malar Butterfly Rash a common clinical feature of?

A

Systemic Lupus Erythematosus

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28
Q

What is a possible primary cause of Sjogren Syndrome?

A

Sicca syndrome

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29
Q

What is the etiology of Autoimmune Polyendocrine Syndrome?

A

AIRE mutation

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30
Q

What is the etiology of DiGeorge Syndrome?

A

22q11 chromosomal deletion

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31
Q

What is the etiology of Hyper IgM Syndrome?

A

Mutation in CD40L on helper T cells or CD40 on B cells

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32
Q

What is the etiology of Scleroderma?

A

Fibroblast activation leading to collagen deposition

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33
Q

In scleroderma, endothelial dsyfunction leads to inflammation via….

A

increased adhesion molecules

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34
Q

In scleroderma, endothelial dsyfunction leads to vasoconstriction via….

A

inc. endothelin

dec. NO

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35
Q

In scleroderma, endothelial dsyfunction leads to secretion of which two growth factors?

A

TGF-beta and PDGF

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36
Q

What gene is mutated in Wiskott-Aldrich Syndrome?

A

:WASP mutation

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37
Q

What is the etiology of X-linked (Bruton) Agammaglobulinemia?

A

Mutation in BTK, Bruton tyrosine kinase

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38
Q

What is the genetic inheritance of Chediak-Higashi Syndrome?

A

AR

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39
Q

What is the genetic inheritance of IPEX Syndrome?

A

XLR

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40
Q

what is IPEX syndrome?

A

IPEX = Immune dysregulation, Polyendocrinopathy, Enteropathy, X-linked

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41
Q

What is the genetic inheritance of Leukocyte Adhesion Deficiency?

A

Autosomal recessive

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42
Q

What is the genetic inheritance of Wiskott-Aldrich Syndrome?

A

XLR

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43
Q

What is the most common immunoglobulin deficiency?

A

IgA

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44
Q

IgA def is seen in what common and media-hyped GI disease?

A

celiac

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45
Q

What is the most common stimulus for chronic inflammation?

A

Persistent infection

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46
Q

What is the second activation signal in the activation of B-cells?

A

CD40L on CD4+ T cell to CD40R on B cells

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47
Q

Upon activation of the B cell, secretion by the Th cell of IL-4 leads to?

A

switching to IgE

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48
Q

Upon activation of the B cell, secretion by the Th cell of IL-5 leads ot?

A

switching to IgA

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49
Q

Upon activation of the B cell, secretion by the Th cell of IFNg leads to?

A

switch to IgG

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50
Q

What is the second activation signal in the activation of CD4+ helper T cells?

A

B7 on APCs to CD28 on CD4+ T cells

7 x 4 = 28

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51
Q

What is the second activation signal in the activation of CD8+ T cells?

A

IL-2 from Th1 cells

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52
Q

What is the treatment for iron poisoning?

A

Chelation via IV Deferoxamine or Oral Deferasirox; Dialysis

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53
Q

What nitroblue tetrazolium (NBT) test result is seen in Myeloperoxidase Deficiency?

A

Normal

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54
Q

What surface proteins on leukocytes binds to ICAM/VCAM on endothelium?

A

Integrins (LFA-1)

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55
Q

What type of collagen is found in scars?

A

type I

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56
Q

What type of collagen is seen in granulation tissue?

A

type III

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57
Q

What type of hypersensitivity is primarily involved in SLE?

A

Type III (antigen-antibody complex)

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58
Q

What type of hypersensitivity is seen in Sjogren Syndrome?

A

Type IV

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59
Q

Where along the capillary bed does extravasation predominantly occur?

A

Post-capillary venules

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60
Q

Where does vasodilation occur?

A

at the level of arterioles

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61
Q

Where are the stem cells of the bone marrow found?

A

Hematopoietic stem cells (CD34+)

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62
Q

Where are the stem cells of the skin found?

A

Basal layer of the skin

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63
Q

Where are the stem cells of the small and large bowels found?

A

Mucosal crypts

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64
Q

Where do T cells mature?

A

thymus

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65
Q

Anti-SSA and Anti-SSB abs in Sjogren are strongly associated with (blank) manifestions

A

extraglandular, like neuropathy

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66
Q

Which 2 anti-inflammatory cytokines are released by macrophages in the resolution and healing phase of inflammation?

A

IL-10; TGF-beta

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67
Q

Which adhesion molecules are upregulated on endothelium in inflammation by IL-1 and TNF?

A

ICAM; VCAM

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68
Q

Which arachidonic acid metabolite mediates the delayed response of mast cell degranulation?

A

Leukotrienes

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69
Q

Which autoantibodies are associated with CREST Syndrome?

A

Anti-centromere antibodies

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70
Q

Which autoantibodies are associated with Diffuse Scleroderma?

A

Anti-Scl-70 (Anti-DNA Topoisomerase I)

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71
Q

Which autoantibodies are seen in Mixed Connective Tissue Disease?

A

Anti-U1 RNP antibodies

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72
Q

Which bacteria is the most common cause of delayed would healing?

A

Staphylococcus aureus

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73
Q

Which cell surface protein on leukocytes binds to selectins on endothelium?

A

Sialyl Lewis X

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74
Q

Which cell surface protein on macrophages recognizes LPS on the outer membrane of gram-negative bacteria?

A

CD14 (co-receptor for TLR4)

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75
Q

Which coagulation factor is referred to as Hageman Factor?

A

factor XII

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76
Q

Which complement deficiency is associated with increased risk for Neisseria infection?

A

C5-C9

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77
Q

Which complement deficiency is most commonly associated with SLE?

A

C2

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78
Q

Which complement deficiency results in Hereditary Angioedema?

A

C1 inhibitor deficiency

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79
Q

Which complement pathway is activated by C1 binding to IgG or IgM bound to antigen?

A

classical

iGM makes classic cars

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80
Q

Which complement pathway is activated by Mannose-binding Lectin (MBL) binding to mannose on microorganisms?

A

Mannose-binding lectin pathway

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81
Q

Which complement pathway is activated by microbial products directly?

A

Alternative

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82
Q

Which complement protein complex lyses microbes by creating a hole in the cell membrane?

A

MAC

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83
Q

Which complement protein functions as an opsonin?

A

C3b

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84
Q

Which complement protein is an opsonin for neutrophils?

A

C3b

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85
Q

Which complement protein is chemotactic for neutrophils?

A

C5a

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86
Q

Which complement proteins are known to activate Mast Cells?

A

C3a, C5a

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87
Q

Which cytokine from macrophages induces helper CD4+ T cells to differentiate into Th1 cells?

A

IL-12

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88
Q

Describe the four steps in forming a granuloma

A
  1. Macrophage presents antigen to CD4+ T-cells via MHC II
  2. IL-12 release from the macrophage.
  3. CD4+ T cells become the Th1 subtype.
  4. Th1 cells secrete IFN-gamma which converts macrophages into epithelioid histiocytes and giant cells.
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89
Q

what is the hallmark cell of a granuloma?

A

epithelioid histiocytes

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90
Q

Which cytokine from Th1 cells converts macrophages into epithelioid histiocytes and giant cells (i.e. forms granulomas)?

A

IFN-gamma

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91
Q

Which cytokine from Th2 cells promotes B-cell class switching to IgE?

A

IL-4 (and IL-13)

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92
Q

Which cytokine released by macrophages recruits additional neutrophils?

A

IL-8

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93
Q

Which cytokines released from macrophages in response to pyrogens increase cyclooxygenase activity?

A

IL-1; TNF

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94
Q

Which enzyme converts Arachidonic Acid into Prostaglandins?

A

COX

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95
Q

Which enzyme in phagocytes converts H2O2 into HOCl (bleach)?

A

Myeloperoxidase

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96
Q

Which enzyme in phagocytes converts O2 into superoxide?

A

NADPH Oxidase

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97
Q

Which enzyme in phagocytes converts superoxide radicals into H2O2?

A

Superoxide dismutase (SOD)

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98
Q

Which enzyme involved in scar formation removed type III collagen?

A

Collagenase

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99
Q

what is the required cofactor for collagenase?

A

zinc

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100
Q

Which enzyme releases Arachidonic Acid from the phospholipid cell membrane?

A

Phospholipase A2

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101
Q

Which enzymes converts Arachidonic Acid into Leukotrienes?

A

5-Lipoxygenase

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102
Q

Which HLA subtype has the strongest association to autoimmune disorders?

A

HLA-B27

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103
Q

Which immunoglobulin isotype functions as an opsonin?

A

IgG

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104
Q

HLA-B27 is strongly associated with what disease?

A

ankylosing spondylitis

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105
Q

Which immunoglobulin isotypes are expressed by naive B-cells?

A

IgM

IgD (yes, D)

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106
Q

Which inflammatory cytokines induce E-Selectin expression in endothelium?

A

TNF; IL-1

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107
Q

Which inflammatory cytokines upregulate ICAM and VCAM adhesion molecules on endothelium in inflammation?

A

IL-1; TNF

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108
Q

Which inflammatory mediator triggers P-selectin upregulation from Weibel-Palade bodies?

A

Histamine

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109
Q

Which inflammatory mediators upregulate Integrin (LFA-1) expression on adhering leukocytes?

A

C5a; LTB4

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110
Q

Which Leukotriene functions to attract and activate neutrophils?

A

LTB4

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111
Q

Which lymphocytes are primarily affected in Autoimmune Polyendocrine Syndrome?

A

T cells

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112
Q

Which prostaglandin is known to mediate pain and fever?

A

PGE2

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113
Q

Which race is more commonly affected by Keloid?

A

African-americans

114
Q

Which Selectin protein in endothelium is induced by TNF and IL-1?

A

E-selectin

115
Q

Which Selectin protein is released from Weibel-Palade bodies in Endothelium?

A

P-selectin}

116
Q

how do you remember what Weibel Palade bodies release?

A

W and P
vWF
P-selectin

117
Q

Which sex is more commonly affected by autoimmune disorders?

A

womenz; Estrogen seems to decrease the apoptosis of self-reactive B cells.

118
Q

Which sex is more commonly affected by Scleroderma?

A

Women; esp of 30-50 years

119
Q

Which sex is more commonly affected by Sjogren Syndrome?

A

Women

120
Q

Which sex is more commonly affected by SLE?

A

Women; especially african americans

121
Q

Which substance released from Mast Cell granules mediates the immediate response of mast cell degranulation?

A

Histamine

122
Q

Which TLR on macrophages recognizes LPS on the outer membrane of gram-negative bacteria?

A

TLR4 (alongside CD14)

123
Q

Which type of collagen is made in excess in a hypertrophic scar?

A

Type I

124
Q

Which type of collagen is made in excess in a Keloid?

A

Type III

125
Q

Which type of granulomatous inflammation exhibits central necrosis?

A

Caseating

126
Q

Which type of granulomatous inflammation is associated with Beryllium exposure?

A

Noncaseating

127
Q

Which type of granulomatous inflammation is associated with cat-scratch disease?

A

Noncaseating

128
Q

Which type of granulomatous inflammation is associated with Crohn Disease?

A

Noncaseating

129
Q

Which type of granulomatous inflammation is associated with fungal infections?

A

Caseating

130
Q

Which type of granulomatous inflammation is associated with Sarcoidosis?

A

Noncaseating

131
Q

Which type of granulomatous inflammation is associated with tuberculosis?

A

Caseating

132
Q

Which type of granulomatous inflammation lacks central necrosis?

A

Noncaseating

133
Q

Which type of infection has an increased risk of manifesting in patients with Myeloperoxidase Deficiency?

A

Candida

134
Q

Which type of inflammation arises in response to tissue necrosis?

A

Acute inflammation

135
Q

Which type of inflammation is an immediate response and part of the innate immunity (i.e. limited specificity)?

A

Acute inflammation

136
Q

Which type of inflammation is characterized by the presence of edema and neutrophils in tissue?

A

Acute

137
Q

Which type of inflammation is characterized by the presence of lymphocytes and plasma cells in tissue?

A

Chronic

138
Q

Which type of inflammation presents as a delayed response but with more specificity as part of adaptive immunity?

A

Chronic inflammation

139
Q

Which type of lymphocyte is affected in Autoimmune Lymphoproliferative Syndrome (ALPS)?

A

T and B cells

140
Q

Which water soluble vitamin deficiency can delay wound healing?

A

Vitamin C

141
Q

what is the role of vitamin C in wound healing?

A

Vitamin C is a cofactor the hydroxylation of proline/lysine residues in collagen. Collagen is needed in fucking wound healing so.. yeah.

142
Q

(blank) is a cutaneous complication of Chediak-Higashi Syndrome that results from defective transport of Melanin from melanocytes to keratinocytes.

A

albinism

143
Q

what are 3 hematological complications of SLE that occur due to Type II hypersensitivity reactions via autoantibodies against cell surface proteins.

A

anemia
thrombocytopenia
leukopenia

144
Q

what are the two Abs that are specific for SLE?

A

Anti dsDNA

Anti Sm

145
Q

(blank) is an autoantibody seen in Antiphospholipid Antibody Syndrome that yields false-positive VDRL and RPR syphilis tests.

A

anticardiolipin

146
Q

what are the three abs seen in Antiphospholipid Antibody syndrome?

A

anticardiolipin
anti beta2-glycoprotein I
lupus anticoagulant

147
Q

what is an autoimmune disorder that involves autoantibodies directed against proteins bound to phopsholipids and is seen in 30% of SLE cases?

A

antiphospholipid Ab syndrome

148
Q

(blank) is an autoimmune disorder that is characterized by a hypercoagulable state due to antiphospholipid antibodies, especially lupus anticoagulant.

A

Antiphospholipid Antibody Syndrome

149
Q

Where do you normally get thrombosus in Antiphospholipid Antibody Syndrome?

A

DVTs
hepatic vein
placental
cerebral
(often a major complication in pregnancy)
ASK COLT ABOUT THE BLACK RESIDENT AT REGANTI’S OFFICE

150
Q

(blank) is a musculoskeletal feature of SLE that typically involves ≥ joints.

A

ARTHRITIS

151
Q

(blank) s an autoimmune disorder that results from a mutation in the FAS (CD95) apoptotic pathway.

A

Autoimmune Lymphoproliferative Syndrome (ALPS)

152
Q

The mutation in the apoptotic pathway in ALPS can be in which three proteins?

A

FAS
FASL
Caspase 10

153
Q

(blank) is an autoimmune disorder that results from a mutation in the Fas apoptotic pathway and involves an inability to induce anergy at the periphery.

A

Autoimmune Lymphoproliferative Syndrome (ALPS)

154
Q

Is the peripheral or central tolerance broken in ALPS?

A

peripheral; affects both T and B cells

155
Q

ALPS results in lymphoproliferation due to an impaired destruction of (b/t) cells

A

b cells

156
Q

what is the transcription factor in medullary epithelial cells that is involved with the expression of self-antigens?

A

AIRE

157
Q

(blank) is an autoimmune disorder due to a mutation in AIRE that presents with a triad of hypoparathyroidism, adrenal failure, and chronic candida infection of the skin or oral mucosa.

A

Autoimmune Polyendocrine Syndrome

158
Q

What three particular infections are the hallmark of generalized immunoglobulin def?

A
  1. bacterial
  2. enterovirus (mucosal immun)
  3. Giardia lamblia
159
Q

what is the product of HMW kininogen that mediates vasodilation and icnreased vascular permeability as well as pain?

A

bradykinin

160
Q

what is characterized by stellate-shaped, noncaseating granulomatous inflammation?

A

cat-scratch diz

161
Q

what dz presents with giant granules in leukocytes d/t fusion of granules from the Golgi?

A

Chediak-Higashi

162
Q

There is an impairment in formation what organelle in chediak-higashi?

A

phagolysosome

163
Q

(blank) is an immunological disorder that is characterized by poor O2-dependent killing due to an NADPH Oxidase deficiency.

A

CGD

164
Q

(blank) positive bugs cause recurrent infections and granulomas in CGD

A

catalase positive

165
Q

which trace element acts as a cofactor for Lysyl Oxidase, an enzyme which cross links collagen?

A

copper

166
Q

what is a type of Limited Scleroderma that involves anti-centromere antibodies?

A

CREST syndrome

167
Q

what is the word for the rupture of a wound?

A

dehiscence

168
Q

(diffuse proliferative GN, membranous GN) is a renal complication seen in SLE that is the most common and most severe form of renal injury.

A

diffuse proliferative GN

169
Q

what is a primary immunodeficiency that involves developmental failure of the 3rd and 4th pharyngeal pouches?

A

diGeorge

170
Q

what dz presents with T-cell def and hypocalcemia?

A

diGeorge

171
Q

what is the common erythematous, scaly rash typically seen on the arm or other areas exposed to sunlight in SLE?

A

discoid rash

172
Q

what is a mediator of wound healing that stimulates cell growth via Tyrosine kinases.

A

EGF

173
Q

(blank) is a cell surface protein expressed by CD8+ T cells that binds to Fas on target cells and causes apoptosis.

A

FASL

174
Q

fever results from the release of what two mediators from activated macs?

A

IL-1

TNF

175
Q

Fibroblast growth factor is weird because despite its name its two major functions are to…

A

increase angiogenesis and skeletal dev

176
Q

what are the three components of granulation tissue?

A
  1. fibroblasts
  2. capillaries
  3. myofibroblasts
177
Q

what is th enzyme made by CD8+ T cells that enters target cells and triggers apoptosis.

A

granzyme

178
Q

Hyper IgM syndrome is a primary immunodeficiency that presents with elevated IgM due to what two things?

A

mutated CD40L on T helpers and CD40 on B cells

179
Q

t/f: there is no secondary signal activation for B cells in hyper IgM syndrome

A

true

180
Q

Besides high IgM, what else is fucked up with the Ig’s in hyper IgM syndrome?

A

lack of IgA, E, and G (no class switching)

181
Q

what two adhesion molecules are upregulated during neutrophil adhesion?

A

VCAM and ICAM

182
Q

which cytokine is secreted by Th1 cells that activates macrophages, promotes B-cell class switching from IgM to IgG and inhibis th2 cells?

A

IFNg

183
Q

which cytokine released by Th2 blocks Th1 growth?

A

IL10

184
Q

which cytokine released by Th2 blocks Th1 growth?

A

IL10

185
Q

a

A

a

186
Q

IL1 and TNF act on what cell in the hypothal to cause fever?

A

perivascular cells

187
Q

What does the hypothal release to cause fever?

A

PGE2

188
Q

What does the hypothal release to cause fever?

A

PGE2

189
Q

IL5 causes chemotaxis of what cell type?

A

eosinophils

190
Q

IPEX is d/t a mutation in what gene?

A

FOXP3

191
Q

which enzyme cleaves high-molecular weight kininogen (HMWK) to Bradykinin.

A

kallikrein

192
Q

which enzyme cleaves high-molecular weight kininogen (HMWK) to Bradykinin.

A

kallikrein

193
Q

what is Keratoconjunctivitis sicca

A

complication of Sjogren, aka dry eyes

194
Q

what is labile tissue?

A

tissue that has stem cells

195
Q

What dz is characterized by delayed separation of the umbilical cord, increased circulating neutrophils and recurrent bacterial infections without pus formation?

A

leukocyte adhesion def

196
Q

What dz is characterized by delayed separation of the umbilical cord, increased circulating neutrophils and recurrent bacterial infections without pus formation?

A

leukocyte adhesion def

197
Q

(blank) is a type of endocarditis seen in SLE that is characterized by small, sterile deposits on both sides of the mitral valve.

A

Libman-Sacks endocarditis

198
Q

t/: libman-sacks endocarditis is aseptic

A

true

199
Q

which three leukotrienes cause vasoconstriction, bronchospasm and increased vascular permeability?

A

LTC4, D4, and E4

200
Q

what is a common diagnostic feature of Sjogren Syndrome that involves lymphocytic infiltration of minor salivary glands?

A

lymphocytic sialadenitis

201
Q

lysozyme kills in an O2 (dependent/independent) manner

A

independent

202
Q

Major basic protein is found int the granules of what cell?

A

eosinophils

203
Q

Major basic protein kills in an O2 (dependent/independent) manner

A

independent

204
Q

what is the toxin in bee venom that causes a type I reaction?

A

mellitin

205
Q

what is the toxin in bee venom that causes a type I reaction?

A

mellitin

206
Q

mixed connective tissue dz has characteristics of what three dzs?

A

SLE
scleroderma
polymyositis

207
Q

in mixed connective tissue disease, there is a lack of involvement of what two systems?

A

CNS

renal

208
Q

what is the major cause of death in MCTD?

A

pHTN

209
Q

MPO def is a problem in what pathway?

A

oxidative burst

210
Q

MPO def leads to an inability to make (blank) from H2O2

A

HOCl

211
Q

MPO def leads to an inability to make (blank) from H2O2

A

HOCl

212
Q

What are the two complications of a baby born to a woman pos for Anti-SSA abs?

A

neonatal lupus

congenital heart block

213
Q

why is there neutropenia in chediak higashi?

A

intramedullary death of neutrophils

214
Q

what is upregulated by TLR activation?

A

NF-kB

215
Q

what two things mediate pain?

A

PGE2

bradykinin

216
Q

(blank) is a Type II Hypersensitivity disorder where the epithelium unzips and antibodies target the desmoglein protein in desmosomes, thereby forming blisters.

A

pemphigus vulgaris

217
Q

what causes peripheral neuropathy in chediak-higashi?

A

defective protein trafficking leading to atrophy of nerve terminals

218
Q

What are the three prostaglandins that mediate vasodilation and vascular permeability?

A

PGI2, PGD2, PGE2

219
Q

what two cell types release platelet derived growth factor PDGF?

A

platelets

macs

220
Q

What does PDGF stimulate?

A

vascular remodeling, smooth muscle migratino and fibroblast growth.

221
Q

what are two types of serositis seen in SLE?

A

pleuritis

pericarditis

222
Q

what is healing by primary intention?

A

cutaneous wound healing that involves wound edges being brought together.

223
Q

what is healing by primary intention?

A

cutaneous wound healing that involves wound edges being brought together.

224
Q

what are three drugs that commonly cause drug induced SLE?

A

procainamide
hydralazine
isoniazid

225
Q

t/f: removal of the drug can stop lupus

A

true

226
Q

describe renal and CNS involvment in drug induced lupus

A

RARE

227
Q

the gain of function in the PTPN22 gene has what role in autoimmunity?

A

decreases signalling that would result in self tolerance

228
Q

This Phenomenon is characterized by an excessively reduced blood flow in response to cold or emotional stress, thereby causing discoloration of the fingers, toes or other areas.

A

Raynaud’s

229
Q

This Phenomenon is characterized by an excessively reduced blood flow in response to cold or emotional stress, thereby causing discoloration of the fingers, toes or other areas.

A

Raynaud’s

230
Q

what problem do you have with your teeth in Sjogrens?

A

lotsa cavities

231
Q

what is the word to describe healing via replacement with native tissue?

A

regeneration

232
Q

what is the word to describe healing via formation of a fibrous scar?

A

repair

233
Q

t/f: rheumatoid factor is present in Sjogrens as well as in RA

A

true

234
Q

Scleroderma is caused by the activation of (blanks) that lay down collagen

A

fibroblasts

235
Q

what is healing by second intention?

A

cutaneous wound healing that involves a lack of approximation of wound edges and filling with granulation tissue

236
Q

what are the ell surface proteins upregulated on endothelium in Neutrophil margination that act as “speed bumps”?

A

selectins

237
Q

SCID can result from what enzyme def?

A

Adenosine Deaminase (ADA) deficiency.

238
Q

why does a lack of Adenosine Deaminase (ADA) lead to SCID?

A

ADA is necessary to deaminate adenosine and deoxyadenosine; buildup of both of them is toxic to lymphocytes.

239
Q

SCID can develop from MHC (I/II) def

A

MHC II

240
Q

t/f: SCID can develop from cytokine receptor defects

A

true

241
Q

which glands are destroyed in sjogrens?

A

lacrimal and salivary

242
Q

what cell destroys the glands in Sjogrens?

A

t cells

243
Q

what is a type II hypersensitivity rxn?

A

Hypersensitivity involves the binding of antibodies to cell surface antigens causing damage through classical complement activation or cell cytotoxicity.

244
Q

what is a type III hypersensitivity rxn?

A

Hypersensitivity involves local or circulating antibody-antigen complexes that cause damage by getting trapped in tissues and activating complement proteins.

245
Q

what type of Hypersensitivity involves the binding of antibodies to cell surface surface receptors causing stimulation, blockage or destruction of that receptor.

A

type II

246
Q

what type of Hypersensitivity involves the activation of T cells resulting in CD4+ mediated macrophage recruitment or CD8+ mediated cytotoxicity.

A

type IV

247
Q

what is type I hypersensitivity?

A

Hypersensitivity involves IgE and mast cell-mediated liberation of Histamine causing local and systemic anaphylaxis.

248
Q

what are the three manifestations of Wiskott-Aldrich?

A

Thrombocytopenia
eczema
recurrent infections

249
Q

what are the three manifestations of Wiskott-Aldrich?

A

Thrombocytopenia
eczema
recurrent infections

250
Q

what cells have TLRs?

A

dendritic cells and macs and lymphocytes

251
Q

what activates TLRs?

A

PAMPs from microbes

252
Q

what is the coreceptor for TLR?

A

CD14

253
Q

LPS comes from gram (pos/neg) bacteria

A

neg

254
Q

what enzyme produces PGs?

A

COX

255
Q

what enzyme produces LTs?

A

5-lipoxygenase

256
Q

Which leukotriene attracts and activates neutrophils?

A

LTB4

257
Q

What are the three ways that mast cells are activated?

A
  1. tissue trauma
  2. C3a and C5a
  3. cross linking of surface IgE by Ag
258
Q

In acute inflammation, what is released immediately and what is released as a delayed response?

A

immediately: histamine

delayed; leukotrienes

259
Q

Factor XII is activated upon exposure to..

A

subendothelial collagen

260
Q

What affect does histamine have on endothelial cells?

A

contraction

261
Q

P-selectin that is released from the Weibel Palade bodies is activated by…

A

histamine

262
Q

E-selectin is induced by what two cytokines?

A

TNF

IL1

263
Q

what is odd about the recurrent infections in LAD?

A

NO PUS FORMATION; b/c the lymphocytes can’t diapedese!

264
Q

What bugs cause recurrent infection in CGD?

A 
PSEUDOMONAS CEPACIA
staph aureus
Serratia marcesens
Nocardia
Aspergillus
(CATALASE POSITIVE)
265
Q

MPO def leads may lead to a propensity for what type of infections?

A

candida

266
Q

How long do neutrophils disappear after the resolution of the inflamm stimulus?

A

24 hours

267
Q

Is persistent pus formation 6 weeks after the start of an illness considered chronic inflamm?

A

NO; pus means acute, so it is called continued acute!

268
Q

what dz presents with T-cell def, abnl of the heart and face, and hypocalcemia?

A

diGeorge

269
Q

Is persistent pus formation 6 weeks after the start of an illness considered chronic inflamm?

A

NO; pus means acute, so it is called continued acute!

270
Q

what two receptors make up the TCR complex?

A

TCR

CD3

271
Q

B7 on APC binds CD(blank) on CD4 T cells for signal 2

A

CD28

272
Q

CD40 on B cells binds CD(blank) on T cells for 2nd signal

A

CD40L

273
Q

What is the second signal for CD8 T cells?

A

IL2 from Th1 cells

274
Q

what is the clinical presentation of autoimmune polyendocrine syndrome?

A
  1. hypoPTH
  2. Adrenal insuff.
  3. Candida infx
275
Q

Tregs are positive for what three cellular markers?

A

CD4, CD25, and FOXP3

276
Q

CD25 is aka (blank) receptor

A

IL2R

277
Q

Fas is aka CD(blank)

A

Fas: CD95

278
Q

CD25 mutations are asssociated with what two autoimmune dzs?

A

MS and DM type I

279
Q

what can B cells do in the bone marrow during negative selection to increase self tolerance?

A

receptor editing

280
Q

anti-SSA and SSB in SJogren are anit-(blank) abs

A

anti-ribonucleoprotein

281
Q

what causes the wound to contract in healing by second intention?

A

myofibroblasts

PATHOMA (11 decks)

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