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PATH Heme-Onc II > Autoimmune Diseases > Flashcards

Autoimmune Diseases Flashcards

1
Q

which diseases both

  • organ specific
  • antibody mediated
A
  • hemolytic anemia
  • thrombocytopenia
  • atrophic gastritis
  • goodpasture disease
  • graves disease
  • myasthenia gravis
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2
Q

which diseases both

  • systemic
  • antibody mediated
A

SLE

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3
Q

which diseases both

  • organ specific
  • T-cell mediated
A
  • Type I DM
  • MS
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4
Q

which diseases both

  • systemic
  • T-cell mediated
A
  • RA
  • systemic sclerosis
  • sjogren syndrome
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5
Q

which diseases both

  • systemic
  • T-cell mediated
A
  • RA
  • systemic sclerosis
  • sjogren syndrome
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6
Q

summarize the clinical presentation that is used to dx SLE

A

SOAP-BRAIN-MD

  • S - Serositis
  • O - Oral ulcers
  • A - Arthritis
  • P - Photosensitivity
  • B - Blood disorders
  • R - Renal involvement
  • A - Antinuclear antibodies
  • I - Immunologic phenomena
  • N - Neurologic disorder
  • M - malar rash
  • D - discoid rash
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7
Q

SOAP-BRAIN MD

A

S = serositis. includes

  • PERICARDITIS - valve disease, CAD
  • PLEURITIS - pleural effusion, tachypnea, cough
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8
Q

SOAP-BRAIN-MED

A

O = oral ulcers:

  • typically PAINLESS
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9
Q

SOAP-BRAIN-MD

A

A = arthritis

  • non-erosive
  • 2+ joints
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10
Q

SOAP-BRAIN-MD

A

P = photosensitivity

  • rash (malar, discoid) in response to exposure to sunlight
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11
Q

SOAP-BRAIN-MD

A

B = blood features

  • lymphocytopenia
  • neutropenia
  • thrombocytopenia
  • anemia
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12
Q

SOAP-BRAIN-MD

A

R = renal issues

  • aka nephritis
    • microscopic: IC complex deposition in the mesangium & basement membrane
    • dx by
      • persistent proteinuria
      • cellular casts
        *
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13
Q

SOAP-BRAIN-MD

A

A = anti-nuclear antibodies

  • ANA
  • ant-dsDNA - dx with w/ c. lucilae or rim/peripheral IF
  • anti-smith
  • ant-phospholipid
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14
Q

SOAP-BRAIN-MD

A

I = immunological phenomena

  • L-cells: phagocytic cells that have ingested damaged nuclei
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15
Q

SOAP-BRAIN-MD

A

N = neurological phenomena

  • seizure
  • psychosis
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16
Q

SOAP-BRAIN-MD

A

M = malar rash

  • aka “butterfly rash”
    • bilateral
    • spares nasolabial fold
    • photosensitivity (triggered by sunlight)
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17
Q

SOAP-BRAIN-MD

A

D = discoid rash

  • gross appearance = plaques on face & scalp
    • scary with follicular plugging
    • surrounded by erythematous, elevated border
  • microscopic
    • flattened epidermis with hyperkeratosis, +
      • perivascular inflammation
  • dx - tho a feature of SLE, RARELY have anti-dsDNA Abs
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18
Q

describe the microscopic morphology of blood vessels in SLE

A
  • small arteries / arterioles vessel contain IC (dsDNA-IgG-C3) d/t type III hypersensitivity, resulting in
    • necrotizing vasculitis + fibrinoid deposits in vessel walls
    • perivascular lymphoid infiltrate
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19
Q

describe the microscopic morphology of the skin changes in SLE

A
  • generally, characterized by
    • liquefactive degeneration of basal layer
    • dermis with vascular SLE changes
      • necrotizing vasculitis + fibrinoid deposits in vessel walls
      • perivascular lymphoid infiltrate
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20
Q

describe the IF of the skin changes in SLE

A

IC (Ab + IgG + complement) along dermal-intradermal junction

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21
Q

death from SLE is m/c dt?

A
  • concurrent infections
  • renal failures
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22
Q
A

pleural effusion (serositis) in SLE

SOAP-BRAIN-MD

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23
Q
A

painless oral uclers in SLE

SOAP-BRAIN-MD

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24
Q
A

LE cell

SLE (SOAP-BRAIN-MD)

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25
Q
A

LE cell in SLE

SOAP-BRAIN-MED

26
Q

identify picture, note important features

A

malar (butterfly) rash in SLE (SOAP-BRAIN-MD)

  • bilateral
  • spares nasolabial fold
  • photosensitive
27
Q

identify picture, note important features

A

discoid rash in SLE (SOAP-BRAIN-MD)

  • gross appearance = plaques on face & scalp
    • scary with follicular plugging
    • surrounded by erythematous, elevated border
  • often anti-dsDNA negative!!
28
Q

identify picture, note important features

A

discoid rash in SLE (SOAP-BRAIN-MD)

  • microscopic
    • flattened epidermis with hyperkeratosis, +
      • perivascular inflammation
29
Q

identify picture, note important features

A

IF of skin in SLE

IC at dermal-intradermal junction

30
Q
A

rim/peripheral IF

highlights dsDNA - can be used to dx SLE

31
Q
A

IF in crithidea lucilae

highlights dsDNA - can be used to dx SLE

32
Q

identify picture, not important pictures

A

vascular changes seen in SLE

  • small arteries / arterioles vessel
    • vessel walls: necrotizing vasculitis + fibrinoid deposits
    • perivascular: lymphoid infiltrate
33
Q

identify picture(s) and note important features

A

nephritis in SLE (SOAP-BRAIN-MED) - microscopic

  • IC complex deposition in the mesangium & basement membrane
    • left: focal lupus nephritis
    • right: diffuse lupus nephritis ( > 50% glomeruli affected)
34
Q

what are the most common causes of drug induced lupus?

A
  • anti-hypertensives
  • anti-fungals
35
Q

rheumatoid arthritis - pathogenesis

A
  • T-cell mediated immune destruction of bone & cartilage, d/t possible
    • genetic predisposition: HLA-DR4 > DR1, DR10, DR14
    • infectious trigger
      • EBV
      • parvovirus B19
36
Q

rheumatoid arthritis - gross morphology

A
  • bilateral, symmetrical polyarteritis of synovial joints - m/c the MCPs / PIPs of the hands & feet (hands first) that
    • is non-purulent
    • can result
      • development of rheumatoid nodules
      • destruction of articular cartilage
37
Q

rheumatoid arthritis - microscopic morphology

A
  • characterized by papillary synovial hyperplasia with dense inflammatory infiltrate of
    • lymphocytes
    • plasma cells
  • later on, rheumatoid nodules can develop:
    • fibrinoid necrosis surrounded by a
      • palisade of activated macrophages
38
Q

identify picture, note important features

A

rheumatoid arthritis

  • papillary synovial hyperplasia with lymphoid nodules: lymphocytes, plasma cells
39
Q

identify the picture, note important features

A

rheumatoid arthritis

  • synovial cell hyperplasia
    • lymphocytes
    • plasma cells
40
Q
A

rheumatoid arthritis

rheumatoid nodule

occur almost exclusively RA, ACPA seropositive pts*

41
Q
A

rheumatoid arthritis

  • rheumatoid nodule, microscopic:
    • palisade of activated macrophages surrounding a zone of fibrinoid necrosis

occur almost exclusively in RA, ACPA seropositive pts*

42
Q

rheumatoid arthritis - diagnosis

A
  • serology: both of the following are seen in about 80% of RA patients
    • RF - not specific for RF
    • ACPA / anti-CCP - highly specific (though not totally) for RA
43
Q

sjogren’s syndrome - pathogenesis

A
  • immune destruction - CD4+ T-cells > B-cells, plasma cells - of,
    • lacrimal glands
    • salivary glands
44
Q

sjogren’s syndrome - microscopic morphology

A
  • early: periductal / perivascular infiltrate
  • late:
    • extensive lymphocytic (mostly CD4+ cells) infiltrate
    • hyperplasia of epithelium lining ducts → duct obstruction
45
Q

sjogren’s syndrome - clinical

A
  • dry mouth (xerostemia)
    • difficulty swallowing / tasting
    • crackling of mucosa
  • dry eyes (keratoconjunctivitis)
    • blurring / burning / itching
    • thick secretions
  • +/- parotid gland enlargement
46
Q

sjogren’s syndrome significantly increases risk of?

47
Q

sjogren’s syndrome - diagnosis

A
  • RF - nonspecific
  • ANA - nonspecific
  • ribonucleic acid proteins:
    • SS-A (Ro)
    • SS-B (La)
  • speckled pattern on IF
48
Q
A

swollen parotid gland

sjogren’s syndrome

49
Q

identify picture, note important features

A

sjogren’s syndrome - microscopic

  • intense infiltrate - lymphocytes & plasma cells (mostly CD4+ cells), with residual hyperplastic ductal epithelial cells
50
Q

identify picture, note important features

A

sjogren’s syndrome - IF

“speckled pattern” IF

51
Q

systemic sclerosis - pathogenesis

A
  • combination of autoimmunity + vascular damage + sclerosis
    • two major types
      • diffuse scleroderma
      • limited scleroderma
52
Q

diffuse scleroderma - clinical presentation

A
  • skin manifestations - extensive, widespread
    • hardening / thickening / “shiny appearance” of skin
    • morphea
    • reynaud’s
  • visceral manifestations - early
    • cardiac / pulmonary fibrosis
    • HTN
53
Q

limited scleroderma - clinical presentation

A

like diffuse scleroderma

  • skin manifestations (but only on fingers, forearms, face)
    • hardening / thickening / “shiny appearance” of skin
    • morphea - local
    • reynaud’s
  • visceral manifestations
    • cardiac / pulmonary fibrosis
    • HTN

unlike diffuse scleroderma, can result in CREST syndrome

54
Q

what is CREST synrome? in what condition is it seen?

A

CREST syndrome =

  • C: calcinosis
  • R: Raynaud phenomenon
  • E: esophageal dysmotility
  • S: scleroderma
  • T: Telangiectasi

seen in limited scleroderma

55
Q

skin manifestations of scleroderma - microscopic morphology

A
  • extensive deposition of dense collagen in the dermis
  • loss of adnexa (hair follicles)
56
Q

systemic sclerosis - diagnosis

A
  • two ANAs:
    • DNA-topoisomerase I
    • Anti-centromere antibody in limited systemic sclerosis (crest syndrome)
57
Q

identify the picture, note important features

A

shiny, hardened / thickened skin

systemic sclerosis/scleroderma - both diffuse & limited

58
Q

identify picture, note important features

A

reynaud’s syndrome - episodic vasoconstriction of arteries after exposure to cold

systemic sclerosis (scleroderma)

59
Q

identify picture, note important features

A

morphea - pale sclerotic plaque surrounded by a violaceous peripheral rim

systemic sclerosis (scleroderma)

60
Q

identify picture, note important features

A

auto-imputation

systemic sclerosis (scleroderma)

61
Q
A

limited scleroderma (CREST syndrome) IF

anti-centromere IF

62
Q

mixed connective tissue disease is characterized by?

A
  • combined presentation of other autoimmune diseases - SLA, RA, systemic sclerosis/scleroderma
  • high antibody titers to U1 ribonucleoprotein

PATH Heme-Onc II (6 decks)

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