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General Pathology > 8-disorders of the immune system 3 > Flashcards

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
Q

What affect does SLE have of the heart?

A

valvular endocarditis

26
Q

SLE in kidneys is called?

A

glomerulonephritis

27
Q

SLE in the central nervous system:

A

microinfarcts

28
Q

What drugs are used to treat SLE?

A

steroids; immunosuppressive drus

-for the kidneys, it may progress to end stage renal disease=transplantation

29
Q

Rheumatoid Arthritis

A
  • proliferative synovitis
  • destruction of articular cartilage: disabling
  • extra-articular lesions of skin, heart, blood vessels, lungs, muscles (similar to SLE, SS)
30
Q

Prevalence of RA

A

1%,

31
Q

When does RA usually start?

A

4th/5th decade of life

32
Q

RA for female:male

A

3-5:1

33
Q

Predisposition for RA:

A

genetic predisposition with microbial initiation

34
Q

blood tests for RA

A
  • rheumatoid factor: anti-IgG Fc

- Anti-CCP

35
Q

Spondyloarthropathies

A
  • ligamentous attachments to bone affected
  • sacroiliac joints; uveitis (inflam of eye)
  • absence of RF
  • HLA-B27 association
36
Q

how is spondyloarthropathies distinguished from rheumatoid arthritis?

A

spondyloarthropathies=absence of RF

37
Q

Which HLA is associated with spondyloarthropathies?

A

HLA-B27

38
Q

infectious triggers of spondyloarthropathies:

A

-Yersinia, shigella, salmonella, helicobacter, campylobacter

39
Q

ankylosing spondylitis; reactive arthritis (arthritis, conjunctivitis, urethritis/cervicitis)

A

spondyloarthropathies

40
Q

Sjogren’s Syndrome

A

-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
Q

HLA-DR3

A

associated with the primary disease state of SjS

-“sicca syndrome”

42
Q

Secondary disease state of SjS

A

HLA-DR4, RA, SLE

43
Q

who does SjS affect the most?

A

Women over 40

44
Q

Systemic Sclerosis (scleroderma)

A
  • 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
Q

Raynaud’s phenomenon:

A

reversible vasospasm, systemic sclerosis

46
Q

inflammatory myopathies: (affect muscles)

A
  • dermatomyosistis

- polymyositis

47
Q

Mixed connective tissue disease

A
  • features not characteristic of other disease
  • anti-RNP
  • little renal disease
48
Q

Amyloidosis

A
  • 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
Q

diagnosis of amyloidosis:

A
  • congo red stain
  • rectal/gingival biopsy
  • serum/urine protein electrophoresis

General Pathology (9 decks)

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