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Year 1: 11. MOD > Chapter 6: Lecture 2 > Flashcards

Chapter 6: Lecture 2 Flashcards

1
Q

What is central tolerance?

A

Negative selection: process of eliminating any developing T or B lymphocytes that are reactive to self in the thymus or bone marrow

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2
Q

What is peripheral tolerance?

A

Ensure that self-reactive T and B cells which escaped central tolerance do not cause autoimmune disease

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3
Q

How is central tolerance mediated?

A

Thymus: Apoptosis of T cells that react w/ self antigens

Bone Marrow: Receptor editing and apoptosis

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4
Q

How is peripheral tolerance mediated?

A

Anergy

Suppression by T reg Cells

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5
Q

What is anergy?

A

Lack of reaction by the body’s defense mechanisms to foreign substances and consists of a direct induction of peripheral lymphocyte tolerance

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6
Q

What are T cell inhibitory receptors?

A

CTLA-4

PD-1

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7
Q

How can cancer cells utilize the T cell inhibitory receptors?

A

Downregulate them to avoid detection and killing

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8
Q

What induces the production of T reg cells?

A

TGF-beta

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9
Q

What do T reg cells express?

A

CD-25

FOXP3

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10
Q

What is the purpose of AIRE and where is it found?

A

Transcription factor expressed in the thymus to eliminate self-reactive T cells
-Exposes T cells self proteins from body and T cells that react to those proteins are destroyed

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11
Q

What is IPEX?

A

Defect in FOXP3 that can lead to an absence of Treg cells

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12
Q

What is an autoimmune disease?

A
  1. Immune reaction against self-antigen
  2. Immune reaction responsible for pathologic condition
  3. No other pathophysiology responsible
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13
Q

What contributes to an autoimmune disease?

A
  1. Genes
  2. Immune Regulation
  3. Environment
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14
Q

What is ankylosing spondylitis?

A

Hereditary inflammatory condition of the joints, especially in the spine
-can lead to degeneration and then fusion of vertebrae

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15
Q

What MHC/HLA allele is ankylosing spondylitis associated with?

A

B27

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16
Q

What is Crohn’s disease?

A

Inflammatory disease of GI tract, especially in the ileum and cecum

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17
Q

What causes Crohn’s disease?

A

Paneth cells in intestinal lining cannot effectively kill microbes, causing an accumulation of bacteria
-this causes an exaggerated immune response

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18
Q

Crohn’s disease has polymorphisms in what gene?

A

NOD-2

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19
Q

What is oral lichen planus?

A

Chronic inflammatory condition that affects mucous membranes inside mouth

1) T cell response against lesions in mucosa
2) Basement membrane is damaged and exposes brand new antigen proteins
3) Secondary B cell response occurs

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20
Q

Most people test positive for _____ if they have an autoimmune disease.

A

ANA

-exception: IgG4 related disease

21
Q

People with lupus test positive for what tests?

A

Anti DS DNA (homogenous)

Anti Smith

22
Q

People with Sjogren syndrome test positive for what tests?

A

Anti Ro/SS-A (speckled)

Anti La/SS-B (speckled)

23
Q

People with systemic sclerosis test positive for what tests?

A

Anti DNA topoisomerase/SCL-70 (speckled)

24
Q

What HLA is systemic lupus erythematosus associated with?

A

HLA-DQ

25
Q

What are some symptoms of systemic lupus erythematosus?

A
  • Malar rash (butterfly rash)
  • Discoid rash: severe rash worse with sun exposure
  • Photosensitivity
  • Oral ulcers
  • Arthritis
  • Serositis
  • Renal disorder
  • Neurologic disorders
  • Hematologic disorders
26
Q

When do you start to suspect systemic lupus erythematosus?

A
  • Arthritis
  • Skin rashes
  • Fever
  • Fatigue
  • Hematologic disorders
27
Q

What symptoms are seen in Diffuse Lupus Nephritis?

A
  • Proteinuria
  • Hematuria
  • Endothelium shows immune deposits
28
Q

What type of immunoglobulins are found in Diffuse Lupus Nephritis?

A

IgG

29
Q

How does skin change in systemic lupus erythematosus?

A

Basal layer degenerates leaving spaces

30
Q

What are some cardiovascular complications of systemic lupus erythematosus?

A

Libman-Sacks Endocarditis

  • warty valve deposits of fibrin
  • rarely can embolize
  • coronary artery disease
31
Q

What symptoms in systemic lupus erythematosus are seen in discoid lupus erythematosus?

A
  • Discoid Rash: face and scalp
  • Positive ANA
  • Positive immunofluorescence
32
Q

What symptoms in systemic lupus erythematosus are seen in drug induced lupus erythematosus?

A
  • Arthralgias
  • Discoid rash
  • Hematologic disorders
  • Positive ANA
  • Positive immunofluorescence
33
Q

What medications can cause drug induced lupus erythematosus?

A
  • Procainamide: higher risk in HLA-DR6

- Hydralazine: higher risk in HLA-DR4

34
Q

What test is drug induced lupus erythematosus positive for?

A

Anti-Histone Ab

35
Q

What is Sjogren syndrome?

A

Autoimmune disease that can destroy lacrimal and salivary gland tissue

  • B and T cell mediated destruction
  • Fibrotic destruction
36
Q

What are symptoms of Sjogren syndrome?

A
  • Dry eyes
  • Dry mouth (xerostomia)
  • Difficulty swallowing
  • Smooth tongue with predisposition to candida yeast infection
37
Q

What are complications of Sjogren syndrome?

A
  • Pulmonary Fibrosis

- Lymphoma

38
Q

What is systemic sclerosis (scleroderma)?

A

Fibrosis throughout body: collagenous deposition with subcutaneous fibrosis

  • Skin
  • GI tract
  • Kidneys
  • Heart
  • Lungs
39
Q

How do ANA patterns differ between systemic sclerosis and CREST syndrome?

A

Systemic sclerosis: speckled

CREST: centromere

40
Q

What test is CREST syndrome positive for?

A

Anticentromere antibodies

41
Q

What is CREST syndrome?

A

Unique form of limited sclerosis

  • Calcinosis: calcium deposits in skin
  • Raynaud’s Phenomenon
  • Esophageal dysfunction
  • Sclerodactyly
  • Telengiectasias: dilation of capillaries causing red marks on skin surface
42
Q

What is IgG4-related disease?

A

Chronic inflammatory condition characterized by:

  • tissue infiltration with lymphocytes and IgG4-secreting plasma cells and T lymphocytes
  • Various degrees of fibrosis
43
Q

What is allorecgonition?

A

Ability of an individual organism to distinguish its own tissues from those of another

44
Q

What causes hyperacute rejection?

A

Preformed ABO antibodies

-marked inflammation followed by thrombotic microvasculopathy

45
Q

What causes acute and chronic antibody rejection?

A

Inflammation with complement C4d breakdown product

-chronic: fibrosis on vessels

46
Q

What causes acute cellular rejection?

A

T cell mediated

-endotheliitis

47
Q

What are some treatments for transplant patietns?

A
  • Corticosteroids: immunosuppressive
  • T lymphocyte-mediated cellular rejection: Tacrolimus
  • Ab mediated rejection: Rituximab
48
Q

What issues can arise with graft vs host disease?

A
  • Skin: rash –> desquamation
  • Liver: jaundice –> cholestasis
  • Intestines: Bloody diarrhea –> strictures

Year 1: 11. MOD (8 decks)

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