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Test 1 IBI > B3.016 - Complement > Flashcards

B3.016 - Complement Flashcards

1
Q

What is the complement system

A

a group of interacting proteins that are cleaved in sequence during complement activation to yield biologically active peptides

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2
Q

How is the alternative pathway triggered

A

B and C3

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3
Q

Which pathway is part of innate immunity

A

Alternative and pectin

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4
Q

How is the classical pathway triggered

A

By antibodies bound to microbes

C2 and C4

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5
Q

How is the lectin pathway triggered

A

MBL binds to terminal mannose residues on microbial cell surface glycoproteins
C2 C4

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6
Q

What do the C3, C2 and C4 do?

A

Help with cleavage of C3 to C3a and C3b

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7
Q

What does C3a do

A

Stimulates inflammation

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8
Q

What does C3b do

A

Deposited on microbe. Helps with opsonization and phagocytosis

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9
Q

What does C5 do

A

Cleaved into C5a and C5b

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10
Q

What does C5a do

A

Helps with inflammation

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11
Q

What does C5b do

A

Combines with C6,C7,C8 and multiple C9s to make MAC

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12
Q

What are the anaphalotoxins

A

C3a and C5a

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13
Q

What do C3a and C5a do

A

Mediators of inflammation

Stimulate mast cells to release their granules and induces urticaria (hives) via histamine release

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14
Q

What is the most common complement related deficiency

A

C2 deficiency

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15
Q

What can be used as an indicator of a disease state with regards to complement components

A

C3 and C4 levels to see if they’re being consumed

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16
Q

What component of complement binds to microbes

A

C3b

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17
Q

What can C3b binding to the microbe cause

A
  1. ) Phagocytosis and killing of microbe
  2. ) Osmotic lysis of microbe by activating later stages of complement
  3. ) destruction of microbe by leukocytes by release of C3a and C5a to recruit leukocytes
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18
Q

What makes up the MAC

A

C5b, C6, C7, C8, many C9s

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19
Q

What pathway does C3d come from

A

Alternative

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20
Q

What does C3d do

A

Recognized by B cell complement receptor 2 (CR2)

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21
Q

Deficiency in late stage MAC production causes what disease

A

Neisseria infections

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22
Q

What are C3 deficiencies a cause of

A

Bacterial infections, may cause early childhood death

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23
Q

What does a deficiency in the MAC complex cause

A

Disseminated Neisseria infections (meningitis)

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24
Q

What does C5a do

A

Chemotaxis of neutrophils

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25
what do C3b and iC3b do
Opsonophagocytosis | Clearance of immune complexes
26
What do C3a, C4a, and C5a do
Danger signaling
27
What do C5b, C6, C7, C8, C9n do
Lysis of cell
28
What does C3d do?
Coactivation of B cells
29
What does C1 inhibitor (C1 esterase) do
Inhibits the classical pathway
30
What does MASP 1,2 do
Inhibits the MBL pathway
31
What does Anaphylatoxin inhibitor do
Degrades C3a, 4a, 5a to reduce inflammation
32
What does Factor 1 do
Cleaves C3b to iC3b to block the C3b part of pathway leading to opsonization of pathogens w
33
What does S protein do
Blocks insertion of MAC into host cell membrane
34
Normally C1q binds to what to cause what
Binds to antigen complexed antibodies resulting in activation of C1r2s2
35
What does C1INH do
Prevents C1r2s2 binding and becoming proteolytically active
36
What does C1 INH do
Prevents C1r2s2 from becoming proteolytically active
37
What among inflammatory mediators can cause cutaneous swelling
Histamine related from mast cells | Bradykinin produced from kallikrein cascade
38
How do histamine and bradykinin act
Increase vascular permeability and vasodilation
39
Itchiness is indicative that a reaction is mediated by what
Histamine
40
Non itchiness and non erythematous area indicates a reaction is mediated by what
Bradykinin
41
Longer attacks indicate what about the cause
Its non histamine related
42
True allergic reactions should what
Be reproducible and consistent
43
Allergic reactions are mediated by what
Mast cells
44
What labs do you get to check the components of Mast cell mediated allergic reactions
Tryptase | Histamine
45
What is tryptase
A protease released by mast cells that degrades in 4-6 hours
46
How quickly does histamine degrade
Within 15 minutes
47
What labs do you get to see if a reaction is bradykinin mediated
Complement studies: C2, C4, C1 esterase inhibitor level and fxn
48
What does factor 12 do
Activates kallikerian to eventually yield bradykinin | Activates plasmin and uses up complement components
49
A C1 esterase inhibitor defect means what
The complement system is going unopposed and increased bradykinin leading to angioedema
50
What therapeutic intervention could also increase bradykinin levels
ACE inhibitors
51
ACE inhibitors are prescribed for what
Hypertension
52
How long does it take for angioedema caused by bradykinin levels to resolve
3-5 days
53
Episodes of hereditary angioedema can happen where
Anywhere on the body
54
Are patients with hereditary angioedema at risk for infection?
No, complement cascade is redundant
55
What is the pathophys of hereditary angioedema
1. ) absence of C1 esterase inhibitor permits ongoing cleavage of C2 and C4 leading to overconsumption 2. ) C2b is further cleaved to a C2 kin in peptide which alters vascular permeability 3. ) C1 esterase inhibitor also regulates fibrinolytic and bradykinin systems which leads to vascular permeability and vasodilation
56
Describe type 1 HAE (low levels of what, age of onset, treatments)
Low or no C1 INH levels or fxn Low C2 or C4 from overconsumption Age of onset 0-20 Can be treated by C1 INH replacement, androgens, Bradykinin inhibitors
57
Type 2 HAE describe
Same as Type 1 except normal levels of C1 INH, but function is an issue
58
HAE type 3 describe
Everything is normal, runs in families Age of onset 1-63 Unsure of treatments We think factor 12a
59
Acquired angioedema describe
Low levels of everything due to autoimmune consumption/inhibition Older people with lymphoma or autoimmune disease Treat underlying defect Age of onset over 20
60
Therapeutic options for angioedema
Supportive care | Anti emetics for GI attacks, IV fluids for dehydration
61
What medications are given for acute settings
Fresh frozen plasma C1 esterase inhibitor replacement products Kallikrein inhibitors Bradykinin receptor antagonists
62
What is ecallantide
Kallekrein inhibitor
63
What is Icatibant
Bradykinin receptor antagonists
64
Would a bradykinin receptor antagonists work for ACE inhibitor angioedema
Yes it should

Test 1 IBI (8 decks)

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