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MOD I Final > Chapter 6 Part 2 > Flashcards

Chapter 6 Part 2 Flashcards

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1
Q

Autoimmune diseases effect mostly?

A

Woman

-IgG4 diseases effect men

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2
Q

Do autoantibodies mean there is an autoimmune disease

A

no, could be something else

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3
Q

3 things need to be met for autoimmune

A
  1. Immune reaction for self
  2. Not secondary to tissue damage (needs to be primary)
  3. Absence of other causes
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4
Q

Systemic diseases discussed in this chapter?

A
  1. Systemic Lupus erythematosus
  2. Rheumatoid arthritis
  3. Systemic Sclerosis (Sjogren syndrome)
  4. Polyarteritis nodosa
  5. Inflammatory myopathies
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5
Q

How does the immune system generate autoanitbodies?

A

Somatic recombination remember?? We need to eliminate these

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6
Q

There are 2 types of immune tolerance, what are they?

A
  1. Central (thymus and bone marrow)

2. Peripheral

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7
Q

How does central tolerance eliminate autoanitbodies (t-cells)?

A
  1. T cells for self are apoptosed in thymus.

2. Some CD4 cells allowed to live as regulatory T-cells

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8
Q

Central tolerance for B-cells?

A
  1. RECEPTOR EDITING is how B-cells are fixed.

2. Apoptosed if not

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9
Q

What is another word for peripheral tolerance?

A
  1. Anergy
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10
Q

How does peripheral tolerance work?

A
  1. Lymphocytes for self rendered functionally unresponsive
    - eliminate co-stimulater after APC presentation.
  2. Without TH, B-cells don’t function adequately
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11
Q

What allows fetus to be accepted?

A

Regulatory T-cells

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12
Q

Where are immune privileged sites?

A
  1. Testes, eyes, Brain

2. Sites where Ags are hidden from immune system

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13
Q

Changes contributing to autoimmunity (3)

A
  1. Defective tolerance
  2. Abnormal Ag display
  3. Inflammation or initial innate immune response
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14
Q

What disease is associated with the HLA-B27?

A

Ankylosins spondylitis (greater liklihood of happening)

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15
Q

HLA -DQA1?

A

Celiac disease

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16
Q

Role of Infections leading to autoimmune (the dark side): (3)

A
  1. Infection provides co-stimulation needed to cause self-antigen
  2. Molecularmimicry: microbe that looks like self Ag
  3. Viruses: (EBV or HIV) can drive B-cell activation that causes problems
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17
Q

Role of infections (the good side):

A
  1. Infections can help develop immunity.

2. Infections can decrease IL-2 and reg T-cell

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18
Q

What common disease decreases the likelihood of autoimmune problems?

A

Type 1 diabetes

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19
Q

Gen features of Autoimmune (AI)

A
  1. Chronic
  2. Relapse
  3. Damage=progressive
  4. Epitope spreading (1 bad Ag causes tissue damage and then stim other Abs
  5. Abnormal/excessive Th1 and 17
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20
Q

This disease effects skin, joints, kidney and serosal membranes. Pt has a malar rash, oral ulcers, renal problems and tests positive for syphilis.

A

Systemic Lupus Erythematosus (SLE)

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21
Q

Specific Abs for SLE?

A
  1. Double stranded DNA

2. Smith (SM) Ag

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22
Q

Antinuclear ABs are found in all AI and can be for? (4)

A
  1. DNA
  2. Histones
  3. Non-histone proteins on RNA
  4. Nuclear Ags
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23
Q

What can cause a false-+ syphilis test in SLE?

A

Antiphospholipid Abs

VDRL

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24
Q

What else can Antiphospholipid Abs do?

A

Cause hypercoagulable PTT test

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25
What drugs are associated with SLE? (3)
1. Hydralazine 2. Procainamide 3. D-penecillamine
26
What is linked to anti-DS DNA and Anti smith?
HLA-DQ
27
what in the Env may cause SLE?
UV-light
28
Morphologic effects of SLE are seen where? (5)
1. Blood vessels 2. Joints 3. Kidneys 4. Skin 5. Pericardium/serosal surfaces
29
What are the morphologic changes of blood vessels?
Acute necrotizing vasculitis of small blood vessels
30
What are the morphologic changes of Joints?
Non-erosive synovitis, Just inflammed !Rheumatoid arthritis is deformed joints!
31
What are the morphologic changes of Kidneys
Glomerular lesions related to the immune complex
32
What are the morphologic changes of Skin
Butterfly rash on face!
33
What are the morphologic changes of Pericardium/serosal surfaces
Inflammation - Acute = fibrisous exudate - Later = thickened
34
SLE can cause what cardiac problems?
1. Tachycardia (due to myocarditis) 2. Coronary artery disease 3. Valvular abnormalaties
35
Other organs effected by SLE (3)
1. CNS (due to vasculitis of small blood vessels) 2. Splenomegaly 3. Liver: pleural effusion/pleuritis
36
Clinical features of SLE
1. Young woman 2. Renal involvement (blood/protein in urine) 3. Increased infections 4. Disease can flare (corticosteroids help)
37
What is associated with Raised skin lesions that are deep and promote scarring?
1. Chronic Discoid Lupus
38
What is associated with wide spread rash and no scars?
Subacute Cutaneous Lupus
39
Drug induced lupus
1. Hydralazine 2. Procainamide 3. D-penecillamine - Spare CNS and Kidneys
40
Pt comes in complaining of dry eyes (keratoconjuctivitis sicca) and Dry mouth (xerostomia): What is the disease and what causes these symptoms
1. Sjogren Syndrome | 2. Destruction of lacrimal and salivary glands
41
Will you find Sjogren syndrome on its own?
Generally not. Usually in conjunction with other diseases
42
How do you diagnose Sjogren syndrome?
1. Biopsy the lip and look for intense lymphocytic response 2. Early onset = T and B mix 3. Late = mostly B
43
Sjogren syndrome mostly effects who?
Women ages 50-60
44
This disease causes widespread damage to small blood vessels, progressive interstitial and perivascular Fibrosis in the skin and organs. Pt with thick skin and Raynaud phenomenon. What is the disease? What causes death?
1. Systemic Slerosis or Scleroderma | 2. Fibrosis of kidney, heart or pulm
45
Clinical features of Scleroderma? (6)
1. Skin Thickening 2. Raynaud phenomenon (vasoconstriction of extremities and discoleration in fingers) 3. Dysphasia from esophageal fibrosis 4. Resp diff 5. Myocardial fibrosis 6. Renal issues
46
Pt with calcinosis, Raymond phenomenon, esophagea and scleradoma issues?
CREST syndrome. -Limited form of scleroderma
47
AI more likely in men?
IgG4 related -Autoimune pancreatitis
48
What is the major factor in Human organ transplants?
HLAs
49
Rec of alloantigins in organ grafts, direct vs indirect?
direct: both CD8 and CD4 Indirect: CD4 only (IFN-gamma)
50
Acute cellular rejections include
1. Cytokines (CD4+ T-cells) | 2. Increase vasc. perm and local mononuclear cells
51
Chronic rejection includes?
1. Lymphocytes react against alloantigens in vessel walls | 2. Secrete cytokinse for local inflammation
52
Hyperacute rejection includes
1. Preformed antidonor Abs | 2. Extremely rapid
53
Acute ab-mediated rejections (2)
1. Antidonor abs produced after transplant | 2. graft vasculature is targeted
54
Chronic AB-mediated
1. Insidious | 2. Also vascular components
55
How to increase graft survival? (5)
1. HLA matching 2. Immunosuppressive Therapy 3. T + B cell depleting Abs 4. Pooled IgG 5. Plasma Pherisis (pull out plasma from blood)
56
HLA matching required for Kidney? (3)
HLA A, B and DR -not done for liver, heart or lungs
57
Immunosuppressive Therapies include? (2)
1. Steroids = decreased inflammation | 2. Mycophenolate mofetil = inhb lymphocytes
58
This virus can reactivate, infect renal tubules and cause graft failure
Polyoma Virus
59
Infections that increase AI risk? (3)
1. EBV-induced lymphomas 2. HPV 3. Kaposi Sarcoma (HHV8)
60
Where are hematopoitic cells taken from?
Peripheral blood (use colony stimulating factor to generate more stem cells)
61
What is required for a bone marrow transplant?
Need to destroy immune system of recipient
62
Graft vs host disease: Acute (2)
1. Days to weeks. | 2. Effects Immune system, skin, liver and intestines
63
Graft vs host disease: Chronic
1. Involution of thymus (some autoimmunity) | - gen same as acute but worse
64
Graft vs host disease: is mediated through? (2)
1. T-lymphocytes (if donor t-lymph killed than GVHD removed) | 2. If you remove T-cells, Leukemia could come back and increase risk of graft failure
65
B-cell related Immunodeficiency syndromes? (4)
1. X-linked Agammaglobulinemia 2. CVID 3. Hyper-IgM syndrome (CD40L) 4. IgA deficiency
66
T-Cell Related Immunodeficieny syndromes (4)
1. APA deficiency 2. X-linked SCID (gamma chain) 3. DiGeorge Syndrome 4. MHC Class 2 deficiency
67
What disease is a bubble boy? What happens to da bboy?
1. Severe combined Immunodeficiency (SCID) | 2. Pro T-cell can't become Immature T-cell thus effecting T-cell development
68
Features of SCID?
1. X-linked 2. Infants w/early thrus 3. Pt dies w/out bone marrow transplant
69
Disease that is due to fail of B-cell precurssors and when do you see it?
1. X-linked Agammaglobulinemia | 2. At 6 months (when mothers Abs are gone)
70
X-linked Agammaglobulinemia: do pt have Igs?
NOOOOO!
71
X-linked Agammaglobulinemia: what is associated (3)
1. giardia 2. Blood stream viruses 3. AI arthritis
72
X-linked Agammaglobulinemia: Tx
Provide Immunoglobins
73
Disease associated with a failure of 3rd and 4th pharyngeal pouches to close, t-cell deficiency and tetany.
DiGeorge syndrome
74
DiGeorge syndrome features (4)
1. Fail of 3rd and 4th pouch to close 2. T-cell deficiency 3. Hypoparathyroid = Hypocalcemia and tetany 4. abnormal mouth and ears
75
Can't produce IgE, IgG or IgA
Hyper IgM syndrome
76
Hyper IgM syndrome Features:
1. CD40L mutation (Th to B messed up) 2. X-linked 3. Pyogenic infections
77
What happens if IgM reacts w/blood cells?
hemolytic anemia
78
Hypogamma globulinemia (blocking IgG) is a result of what disease?
Common Variable Immunodeficiency (CVID)
79
CVID-do B-cells become plasma cells?
No, but they are normal
80
Find this disease with blood transfusions
Isolated IgA
81
IgA is usually
asymptomatic which is why you find it in transfusion pts.
82
Immunodeficiencies Associated w/systemic diseases (2)
1. Wiskoff-Aldrich syndrome | 2. Ataxia Telangiectasia
83
Wiskoff-Aldrich syndrome: facts (3)
1. X-linked 2. Thrombocytopenia 3. Hematopoietic stem cell transplant = Tx
84
Ataxia Telangiectasia: facts (3)
1. Autosomal Recessive 2. Abnormal gait and vascular malformations 3. neurologic issues and increased tumors
85
Acquired Immunodeficiency syndrome (AIDS): caused by?
HIV and transported by sexual, needles, mother to infant
86
2 forms of HIV
1. HIV 1 (Developed nations and Central america) | 2. HIV 2 (3rd world)
87
Virus core proteins of HIV
1. Major Capsid Proteins-P24 (ELIZA test for this) 2. copies of RNA 3. Viral Enzymes
88
HIV Matrix protein?
p17
89
Proteins studding HIV envelope?
Gp120 and GP41
90
HIV infection involves what cells?
1. T cells 2. Dendritic Cells 3. Macrophages
91
Chemokine receptors for HIV isolates (3)
1. R5 => CCR5 2. X4 => CXCR4 3. R5X4 => dual tropic
92
R5 => CCR5?
1. Monocytes | 2. Acute strain
93
X4 => CXCR4
1. T-cells | 2. gradually accumulates
94
HIV infects...
1. Memory and active T-cells | 2. Can't infect Naive T-cells
95
Why can't HIV infect Naive T-cells?
ApoBEC36 gene/enzyme blocks it -HIV's VIF is learning to counteract this
96
HIV thrives when...
1. Host T-cell and macrophages activated | - Stim NF-kB and increase monocytes
97
Polyclonal B-cells can
cause problems
98
Macrophages from HIV1... (2)
1. depend on VPR gene | 2. Resevoirs of infection
99
Dendritic Cells...
1. Bring mucosal dendritic cells to lymph and CD4+
100
Follicular cells...
1. HIV resevoir | 2. Trap virions and maintain CD4+ ability to infect

MOD I Final (4 decks)

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