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COM - MOD 1 > Ch 6 Slides Part 2 - Guerin > Flashcards

Ch 6 Slides Part 2 - Guerin Flashcards

1
Q

What is the mechanism of central tolerance for T cells?

A

Negative selection or deletion

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2
Q

What are the immune-privileged sites?

A

Testis
Eye
Brain

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3
Q

What HLA type is associated with Ankylosing spondylitis

A

B27

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4
Q

What are 2 methods by which infections induce autoimmunity?

A

Costimulation on APSc

Molecular mimicry

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5
Q

What is the classic example of molecular mimicry?

Causes what?

A

Rheumatic heart disease

Myocarditis

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6
Q

Most autoimmune disease are caused by what?

A

Excessive Th1 and Th17 responses

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7
Q

What group of people is most common to develop SLE?

A

Women

African American and Hispanic > White

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8
Q

What ANAs are specific for SLE?

A

dsDNA

Sm antigen

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9
Q

What antibodies are diagnostic for Sjogren syndrome?

A

Ro/SS-A

La/SS-B

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10
Q

Antiphospholipid antibodies are found in what % of lupus patients?

Has what false positive?

Induces what state?

A

30-40%

Syphilis

Hypercoagulable state (INC PTT)

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11
Q

What environmental factors induce SLE?

A

UV light
Sex chromosomes
Hydralazine, procainamide, D-penicillamine

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12
Q

What are the blood vessels like in patients with SLE?

A

Acute necrotizing vasculitis involving capillaries, small arteries, and arterioles

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13
Q

What are the joints like in patients with SLE?

Opposite of what?

A

Non-erosive synovitis with little deformity

RA

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14
Q

Glomerular lesions are the result of what?

A

Immune complex deposition

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15
Q

What is the most common and severe form of lupus nephritis?

A

Diffuse lupus nephritis (Class IV)

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16
Q

What kind of effusions may be present with SLE?

A

Pleural and pericardial effusions

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17
Q

What cardiovascular system effects are present with SLE?

A

Myocarditis
CAD
Valvular (Libman-Sacks) endocarditis

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18
Q

What other organs are involved in SLE?

A

CNS
Spleen
Lungs (effusions), fibrosis secondary pulmonary HTN

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19
Q

What are signs of renal involvement in SLE?

A

Hematuria, proteinuria, nephrotic syndrome

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20
Q

What are the causes of death in SLE?

A

Renal failure
Infections
CAD

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21
Q

How does chronic discoid lupus Erythematosus present?

A

Skin manifestations, rarely systemic
Skin plaques with erythema, scaliness, edema
Face and scalp usually

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22
Q

How is subacute cutaneous Lupus distinguished from chronic discoid LE?

A

Skin rash is widespread, superficial, non-scarring

Mild symptoms

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23
Q

What drugs induce lupus syndrome?

A 
HIPD
hydralazine
Isoniazid
Procainamide 
D-Penicillamine
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24
Q

What organs are NOT affected in drug-induced LE?

A

Renal

CNS

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25
Q

What is Sjorgren characterized by?

Occurs bc of what?

A 
Dry eyes
Dry mouth (xerostomia)

Immunologically mediated destruction of lacrimal and salivary glands

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26
Q

What are some clinical features of Sjogren?

A

Blurring of vision, burning, itching
Difficulty in swallowing solid foods, Dec ability in taste
Parotid gland enlargement
Epistaxis

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27
Q

What is the best way to diagnose Sjogren syndrome?

A

Biopsy of the lip to examine minor salivary glands

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28
Q

What kind of pathology may develop from Sjorgren syndrome?

A

Dominant B-cell clone and marginal zone lymphoma

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29
Q

What characterizes systemic sclerosis (Scleroderma)?

A

Chronic inflammation via autoimmunity
Widespread damage to blood vessels
Progressive interstitial and perivascular fibrosis in the skin

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30
Q

What are the Clinical features of scleroderma?

A

F to M ratio of 3:1
Peak incidence of 50-60 y/o
Cutaneous changes
Raynaud, dysphagia, respiratory difficulty, myocardial fibrosis, mild proteinuria

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31
Q

What are the symptoms of patients with limited scleroderma?

A 
CREST
Calcinosis
Raynaud
Esophageal dysmotility
Sclerodactyly
Telangiectasia
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32
Q

What disease often affects middle-aged and older men and is 1st characterized in autoimmune pancreatitis?

A

IgG4 Related Disease

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33
Q

What is Mikulicz syndrome?

Associated with what?

A

Enlargement and fibrosis of salivary and lacrimal glands

IgG4

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34
Q

What characterizes IgG4 disease?

A

Storiform fibrosis
Obliterative phlebitis
INC serum IgG4

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35
Q

The Direct pathway uses what molecules?

A

Class I MHC

Class II MHC

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36
Q

The indirect pathway of recognition of alloantigens involves what cells?

A

B cells -> Abs -> endothelial injury

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37
Q

What characterizes acute cellular rejection?

Commonly seen when?

A

Increased vascular permeability, local accumulation of mononuclear cells

With initial months after transplantation

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38
Q

What characterizes chronic rejection?

A

Lymphocytes react against alloantigens in the vessel wall

Secrete cytokines that induce local inflammation

39
Q

What mediates acute cellular and chronic rejection?

40
Q

Hyperacute rejection occurs when?

Develops in who?

A

Preformed antidonor antibodies are present

Previous transplant, prior blood transfusions, multiparous women

41
Q

Acute antibody-mediated rejection occurs when?

What is the initial targets of these antibodies?

A

Antidonor antibodies produced after transplantation

Graft vasculature

42
Q

Chronic antibody-mediated rejection develops how?

Affects what?

A

Insidiously

Vascular components

43
Q

What alleles are matched for kidney transplants?

A

HLA-A, HLA-B, HLA-DR

44
Q

What is allele matching not done for?

A

Liver, heart, lungs

45
Q

What drugs are given for immunosuppressive therapy?

A

Steroids
Mycophenoalte mofetil
Tacrolimus (FK506) - inhibits T cell functions

46
Q

In kidney transplant recipients what virus can reactivate and infect renal tubules, may even cause graft failure?

How to stop?

A

Polyoma virus

Reduce immunosuppresion

47
Q

Immunosuppressed individuals are susceptible to what latent viruses?

A

EBV-induced lymphomas
HPV-induced squamous cell carcinomas
Kaposi sarcoma (HHV8)

48
Q

What organs can be transplanted?

A

Kidney is the most

Also liver, heart, lungs, pancreas

49
Q

When is GVHD most commonly seen?

A

HSC transplantation

50
Q

Patient presents with a rash and diarrhea after weeks of allogenic bone marrow transplantation, what is going on?

A

Acute GVHD

51
Q

What are the major organs affected from acute GVHD?

A

Immune system, skin, liver, and intestines

52
Q

What characterizes chronic GVHD?

A

Extensive cutaneous injury

Chronic liver disease

53
Q

What mediates GVHD?

A

T lymphocytes from the donor

54
Q

Depletion of donor T cells before transfusion eliminates GVHD but leads to what?

A

Recurrence of tumor in leukemia patients
Increased incidence of graft failure
Increased rates of EBV-related B-cell lymphoma

55
Q

SCID affects what immune responses?

Presents in who with what?

A

Humoral and cell-mediated

Infants present with thrush, extensive diaper rash, failure to thrive, rash (GVHD)

56
Q

What severe infections are SCID patients susceptible to?

A

PPV w/your CC

Pneumocystis jiroveci
Pseudomonas
Varicella

Candida albicans
CMV

57
Q

What inheritance is most common in SCID?

58
Q

What is the major defect in X-linked Agammaglobulinemia?

Aka what?

A

Failure of B-cell precursors (pro and pre) to develop into mature B cells

Bruton

59
Q

What are the characteristics of X-linked Agammaglobulinemia?

A

B cells absent or very low in circulation
Serum levels of all Igs low
NO plasma cells
Apparent after 6 months

60
Q

What bacterial infections are common in X-linked Agammaglobulinemia?

A

HSS
Haemophilus influenzae Streptococcus pneumoniae
Staphylococcus aureus

61
Q

What viruses are common in X-linked Agammaglobulinemia?

A 
PECG
Poliovirus
Enterovirus (echovirus)
Coxsackievirus
Giardia lamblia
62
Q

35% people with X-linked Agammaglobulinemia develop what?

A

Arthritis and dermatomyositis

63
Q

DiGeorge Syndrome is due to what?

Deficiency in what?

What chromosome abnormality?

A

Failure of 3rd and 4th pouches

T-cells

22q11 deletion

64
Q

Sx of DiGeorge Syndrome?

A

Poor defense against fungal and viral infections
Hypocalcemia -> tetany
Congenital defects of heart and great vessels
Abnormal facial and ear appearance

65
Q

What characterizes Hyper-IgM syndrome?

Defect in what?

A

Make IgM, cannot make any others

T helper cells cannot deliver activating signals to B cells and macrophages

66
Q

What kind of inheritance with Hyper-IgM syndrome?

Patients present with what type of infections?

A

X-linked (70%) or autosomal recessive

Pyogenic infections or Pneumocystis jiroveci pneumonia (CD40L mutation)

67
Q

CVID is characterized by what?

A

Normal B cells that are unable to differentiate into plasma cells

68
Q

Isolated IgA deficiency is common in whom?

Susceptible to what problems?

A

European descent (1 in 600)

Sinopulmonary infections and diarrhea

69
Q

Patients transfused with IgA-containing blood can develop what?

A

Anaphylactic reactions because the IgA behaves like foreign

70
Q

What disease is associated with thrombocytopenia, eczema, and recurrent infection?

Is also X-linked

A

Wiskott-Aldrich syndrome

71
Q

What autosomal-recessive disease is associated with Telangiectasia, neurologic deficits and increased incidence of tumors?

A

Ataxia Telangiectasia

72
Q

What can cause secondary immunodeficiency?

A 
Malnutrition
Chemotherapy
Irradiation
Infections
Cancers
73
Q

Globally what is the most common mode of transmission of HIV?

A

Heterosexual contacts

74
Q

What major capsid protein is the most abundant viral protein and what test is used to diagnose HIV infection?

75
Q

What is contained in the virus core?

A

P24
2 copies viral RNA
Protease, reverse transcriptase, integrase

76
Q

What is the virus core of HIV surrounded by?

A

Matrix protein p17

77
Q

What studs the viral envelope?

A

gp120 and gp41

78
Q

What does HIV first infect?

What do they all have?

A

T cells
DCs
Marcophages

CD4

79
Q

The M-tropic strain preferentially infects what?

What strain and chemokine receptor?

A

Monocyte/macrophage

R5 strain
CCR5

80
Q

The T-tropic strain uses what strain and chemokine receptor?

81
Q

HIV infects what?

A

Memory and activated T cells

82
Q

What is the active form of enzyme in the HIV genome?

What viral protein binds this?

A

APOBEC3G

Vif

83
Q

What viral gene in HIV-1 infects macrophages?

84
Q

HIV infects what in the brain?

A

Macrophages and microglial cells

85
Q

Acute retroviral syndrome occurs when?

What are the symptoms?

A

3-6 weeks after infection

Sore throat, myalgias, fever, weight loss, fatigue

Rash, cervical adenopathy, diarrhea, vomiting

86
Q

Where are the sites of continuous HIV replication and cell destruction?

A

LNs

Spleen

87
Q

How does HIV evade immune detection?

A

Destroys CD4+ T Cells
Antigenic variation
Down-modulates class I MHC
Can use CXCR4 or CCR5 to enter cells

88
Q

Immune reconstitution inflammatory syndrome may occur with what?

A

HAART treatment

89
Q

What are the adverse side effects of HAART?

A 
Lipoatrophy 
Lipoaccumulation
Elevated lipids
Insulin resistance
Peripheral neuropathy 
Premature cardiovascular, kidney, liver disease
90
Q

Hemodialysis associated Amyloidosis as what major fibril protein?

What precursor protein?

What disease?

A

Abeta2m

Beta2-microglobulin (Class I MHC)

Chronic renal failure

91
Q

Reactive systemic Amyloidosis has what major fibril protein associated with it?

What precursor protein?

What associated disease?

A

AA

SAA

Chronic inflammatory condition

92
Q

Primary Amyloidosis has what major fibril protein?

What precursor protein?

What associated diseases?

A

AL

Ig light chains (lambda)

Multiple myeloma
Monoclonal plasma cell proliferates

93
Q

Type 1 Diabetes and M.S. Are what kind of diseases?

A

Organ-specific

COM - MOD 1 (7 decks)

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