Immunopathology II Flashcards

1
Q

Tolerance

A

incapability to mount an immune response against specific antigen

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2
Q

self tolerance

A

lack of responsiveness to self antigens

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3
Q

Central tolerance

A

referes to deletion of self-reactive B and T cells during their maturation inthe bone marrow and thymus

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4
Q

Peripheral tolerance

A

mechanism to eliminate self reactive T cells that escaped intrathymic delection (- selection)

  • anergy
  • suppression by reg. t cells
  • deletion by induced apoptosis
  • antigen sequestration
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5
Q

Anergy

A

T cell rendered useless after bad enconter

T cell requires two signals: MHCII and B7 (APCs)

  • secondary signal B7–>CD28 is absent
  • instead CTLA4 binds B7–>thus anergy
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6
Q

Suppression by regulatory T cells (T-regs)

A
  • T-regs develop in the thymus and express CD4 and CD25
  • mutation in Fox3, required for their dev and fx results in severe autoimmune
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7
Q

Deletion by Induced apoptosis

A

involves fas ligand system

mutaions can lead to autoimmune lyphoproliferative syndrome

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8
Q

antigen sequestration

A

some antigens are hidden from immune system due to lack of communication with blood and lymph (immune-privileged sites such as testis, eye, brain

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9
Q

Systemic Lupus Ertyhematosis (SLE)

A

unknown cause, but fundamental defect is failure to maintain self-tolerance

-characterized by presence of antinuclear antibodies

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10
Q

Criteria for diagnosis

A
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11
Q

Antinuclear antibodies (ANA)

A
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12
Q

Antinuclear Antibodies and Autoimmune Disease

A

Drug induced Lupus will have antihistones.

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13
Q

Antiphospholid antibody (lupus anticoagulat) is associated with?

A

thrombosis and recurrent miscarriages

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14
Q

Early complement deficiency (C3, C4, C1q) in SLE will?

A

impair removal by MO and favor tissue deposition

approx 5-10% patients

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15
Q

How are vesceral lesions mediated in SLE?

A

Hypersensitivity type III

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16
Q

What happens when cel nuclei are exposed to ANA in SLE?

A

cell damage occurs. those cells lose their chromatin pattern and become homogenous (hematoxylin bodies)

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17
Q

What does this figure represent?

A

Hematoxylin Bodies (SLE)

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18
Q

What does this figure represent?

A

Immune-complex deposition (Type III HS)

SLE

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19
Q

Class I of Lupus Nephritis:

A
20
Q

Class II of Lupus Nephritis:

A

Mesangial Glomerulonephritis (20%), mild symptoms

21
Q

Class III of Lupus Nephritis:

A

Focal prliferative (25%), moderate disease

22
Q

Class IV of Lupus Nephritis:

A

Diffuse proliferative (50%) most severe, characterized by wire loops

* active disease*

23
Q

Class V of Lupus Nephritis:

A

Membranous (15%), capillary wall thickening, nephrotic syndrome

24
Q
A

Normal Glomerulus (Class I)

25
Q
A

Class II

Glomerulonephritis

26
Q
A

Class III Focal Proliferative glomerulonephritis

27
Q
A

Class IV

Diffuse proliferative glomerulonephritis

*most active form

28
Q
A

Class IV “Wire loep”

29
Q
A

Class V: membranous glomerulonephritis

30
Q

Sjogrens Syndrome

A

Autiunnybe destruction of Lacrimal and salivary glands

  • dry eyes, dry mouth
  • about 75% patients have RA factors
  • 50-80% have +ANA
  • >90% antibodies against SS-A & SS-B present
  • 40x risk increase for non-Hodgkin’s lymphoma
  • women age 50
31
Q

What are these characteristic of?

A

Sjogens disease

32
Q

Systemic Sclerosisi (Scleroderma)

A
  • chronic disorder, abnormal accumulation fo firbous tissue in skin and other organs
  • common in 50-60yo F
  • diffuse and limited variants
  • Associated w/ anti-DNA topoisomerase 1 and anticentrome antiboides
  • diffuse variant: skin(most), GI (90%), lungs (50%), kidneys (65%) and SK (common)
33
Q

What autoimmune disorder is represented here?

A

Scleroderma

A. normal

B. fibroblasts, cell mediated process w/ lymphocytes

34
Q

What characteristic of limted type scleroderma does this figure show?

A

Calcinosis of soft tissues?

35
Q

What type of limited scleroderma does this figure represent?

A

Esophageal invovement-fibrosis of the lower 2/3 esophagus

36
Q

Rheumatoid arthritis

A

autoimmune disorder leads to chronic systemic inflammation affecting many tissue

  • arthritis- affects small joints of hand & feet and is non-suppurative that eventually leads to destruction of jts.
  • extra articular symptoms are fairly common and vascular/renal finding similar to SLE
  • incidence 1:100, F:M 3-5:1, 2nd-4th decades of life
37
Q

Rheumatoid factor

A

IgM attack B cell producing IgG

found in 80%

believed to be involved in extra articular pathology (due to similarities w/ SLE)

38
Q

What does this figure show?

A

ulnar deviation in arthritis

39
Q

What does this figure show?

A

Athritis: PIP joints (Swan neck deformity)

40
Q

What does this figure show?

A

Arthritis

Pannus formation – mass of inflammatory cells that begins destroying cartilage (B, T, Plasma cells)

41
Q

What does this figure show?

A
  1. Normal Synovium
  2. RA synovium
42
Q

What does this figure show?

A

Rheumatoid nodules- central zone of necrosis surrounded by epithelioid histiocytes

43
Q

What does this figure show?

A

Libman-Sacks Endocarditis

  • caused by lupus aswell
  • inflammatio and pus affecting valves
44
Q

Autoimmune disease and HLA

A

SLE–>HLA-DQ

RA–>HLA-DR4

JUVENILE RA–>HLA-B27

*SPONDYLOARTHROPATHIES–>HLA-B27

SJOGRENS SYNDROME–>HLA-DR3

45
Q

Seronegative Spondyloarthropathies

A

heterogenous group

  • characterized by
  • do not see RA factor (this is in RA)
  • pathological changes to ligaments
  • involvement of sacroiliac joints
  • association w/ HLA-B27
46
Q

What does this figure represent?

A

Ankylosing spondylitis: sacro-iliac involvement

-common in males