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8-disorders of the immune system 3 Flashcards

1
Q

Other names for autoimmune diseases:

A

connective tissue diesase or collagen vascular disease

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2
Q

clonal deletion:

A

loss of T cell clones during maturation

-it is part of self tolerance

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3
Q

clonal anergy:

A

inactivation induced by antigens

-part of self tolerance, the last part of self tolerance is “peripheral suppression by T cells”

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4
Q

Mechanisms of Autoimmune disease (loss of self tolerance)

A
  • bypass of helper T-cell tolerance (modified by drugs or costimulatory molecules/infection)
  • molecular mimicry (microbes share epitopes with self antigens)
  • polyclonal lymphocyte activation (endotoxin, EBV)
  • Imbalance of suppressor/helper function
  • emergence of sequestered antigens (eye, brain, not normally in contact with circulating immune system)
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5
Q

What is the greatest example of molecular mimicry?

A

Streptococci and rheumatic heart disease

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6
Q

Examples of systemic, multi-organ autoimmune diseases (many antibodies)

A
  • Systemic Lupus Erythematosus
  • Rheumatoid Arthritis
  • Spondyloarthropathies
  • Sjogren’s Syndrome
  • Scleroderma
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7
Q

Examples of Organ-specific disorders (antibodies against organ)

A
  • Thyroiditis; adrenal failure
  • autoimmune hepatitis
  • Type I diabetes Mellitus
  • Pernicious Anemia
  • multiple sclerosis
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8
Q

symptoms of Systemic Lupus Erythematosus

A
  • butterfuly rahs
  • discoid lupus (disc shaped rash)
  • photosensitivity
  • oral ulcers
  • arthritis
  • serositis
  • neurologic disease
  • hematologic disease
  • immunologic disease: LE cell, anti-DNA, anti-Sm, false positive for STS
  • anti-nuclear antibody
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9
Q

What is the incidence of SLE?

A

1:2500

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10
Q

what is the proportion of Female:male incidence?

A

10:1

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11
Q

When does SLE start?

A

2nd/3rd decade of life (teens and 20’s)

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12
Q

What ethnicity does SLE affect most?

A

Blacks

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13
Q

What areas of the body of SLE affect most?

A

skin, kidney, serosal membranes, joints, heart

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14
Q

ANA=?

A

Anti-Nuclear Antibodies

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15
Q

What disease is ANA associated with?

A

SLE

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16
Q

What method is used to test ANA?

A

indirect immunofluorescent method

-Hep2 cells+patient serum+fluor anti-human Ig

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17
Q

What are the different antibodies associated with ANA?

A

-Abs to: DNA, histone, non-histone proteins bound to RNA, nucleolar antigens

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18
Q

ANA Patterns:

A
  • Homogeneous: DNA, histone
  • Speckled: Sm, RNP, SS-A, SS-B
  • Nucleolar
  • patterns are NOT DIAGNOSTIC
  • antibodies against RBCs, WBCs, platelets, phospholipids
  • even centromere ANA
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19
Q

what monitoring test is used for SLE

A

erythrocyte sedimentation rate

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20
Q

Genetic factors of SLE

A
  • 30% concordance in monosygotic twins
  • increased risk in family members
  • HLA-DQ locus and SLE association
  • inherited deficiency of complement and SLE
21
Q

Non-Genetic factors of SLE

A
  • drugs: procainamide, hydralazine
  • sex hormones (estrogens>androgens)
  • UV light
22
Q

***fundamental cause of disease in SLE

A
  • Anti-DNA Ab forms complexes with DNA normally released into blood from cells turning over
  • These circulating immune complexes (CIC) deposit in small blood vessels
  • complement binds to CIC deposits causing tissue damage and attracting WBCs to cause more damage
23
Q

The fundamental disease in SLE happens in small blood vessels all over the body in many different organs, but especially in:

A

the glomeruli of the kidney

24
Q

What serosa does SLE affect?

A

pericardium and pleura

25
What affect does SLE have of the heart?
valvular endocarditis
26
SLE in kidneys is called?
glomerulonephritis
27
SLE in the central nervous system:
microinfarcts
28
What drugs are used to treat SLE?
steroids; immunosuppressive drus | -for the kidneys, it may progress to end stage renal disease=transplantation
29
Rheumatoid Arthritis
- proliferative synovitis - destruction of articular cartilage: disabling - extra-articular lesions of skin, heart, blood vessels, lungs, muscles (similar to SLE, SS)
30
Prevalence of RA
1%,
31
When does RA usually start?
4th/5th decade of life
32
RA for female:male
3-5:1
33
Predisposition for RA:
genetic predisposition with microbial initiation
34
blood tests for RA
- rheumatoid factor: anti-IgG Fc | - Anti-CCP
35
Spondyloarthropathies
- ligamentous attachments to bone affected - sacroiliac joints; uveitis (inflam of eye) - absence of RF - HLA-B27 association
36
how is spondyloarthropathies distinguished from rheumatoid arthritis?
spondyloarthropathies=absence of RF
37
Which HLA is associated with spondyloarthropathies?
HLA-B27
38
infectious triggers of spondyloarthropathies:
-Yersinia, shigella, salmonella, helicobacter, campylobacter
39
ankylosing spondylitis; reactive arthritis (arthritis, conjunctivitis, urethritis/cervicitis)
spondyloarthropathies
40
Sjogren's Syndrome
-immune-mediated destruction of lacrimal and salivary glands, (with swelling) =keratoconjunctivitis sicca (dry eyes) and xerostomia (dry mouth) -pseudolymphoma in 10% -B cell lymphoma in 1%
41
HLA-DR3
associated with the primary disease state of SjS | -"sicca syndrome"
42
Secondary disease state of SjS
HLA-DR4, RA, SLE
43
who does SjS affect the most?
Women over 40
44
Systemic Sclerosis (scleroderma)
- inflammation/fibrosis of interstitium of organs (thickening) - skin: fingers, upper extremities, shoulders, neck, face - GI: esophagus, difficulty swallowing - Musculoskeletal: joints and muscles - lungs: diffuse interstitial fibrosis - kidneys, heart - raynaud's phenomenon: reversible vasospasm
45
Raynaud's phenomenon:
reversible vasospasm, systemic sclerosis
46
inflammatory myopathies: (affect muscles)
- dermatomyosistis | - polymyositis
47
Mixed connective tissue disease
- features not characteristic of other disease - anti-RNP - little renal disease
48
Amyloidosis
- amyloid replaces normal functioning tissue - intercellular pink translucent material on H&E - chemical nautre: beta-pleated fibrillar protein - organ deposition: kidney, spleen, liver, heart, endocrine - prognosis poor
49
diagnosis of amyloidosis:
- congo red stain - rectal/gingival biopsy - serum/urine protein electrophoresis

General Pathology (9 decks)

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