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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
LE cell in SLE SOAP-BRA**I**N-MED
26
identify picture, note important features
malar (butterfly) rash in SLE (SOAP-BRAIN-**M**D) * bilateral * spares nasolabial fold * photosensitive
27
identify picture, note important features
discoid rash in SLE (SOAP-BRAIN-M**D**) * gross appearance = plaques on face & scalp * **scary with follicular plugging** * surrounded by _erythematous, elevated_ border * often anti-dsDNA negative!!
28
identify picture, note important features
discoid rash in SLE (SOAP-BRAIN-M**D**) * microscopic * **flattened epidermis with hyperkeratosis,** + * perivascular inflammation
29
identify picture, note important features
IF of skin in SLE IC at dermal-intradermal junction
30
**rim/peripheral IF** highlights dsDNA - can be used to dx SLE
31
IF in crithidea lucilae highlights dsDNA - can be used to dx SLE
32
identify picture, not important pictures
vascular changes seen in SLE * small arteries / arterioles vessel * vessel walls: necrotizing vasculitis + fibrinoid deposits * perivascular: lymphoid infiltrate
33
identify picture(s) and note important features
nephritis in SLE (SOAP-B**R**AIN-MED) - microscopic * **IC complex deposition in the** **mesangium & basement membrane** * left: focal lupus nephritis * right: diffuse lupus nephritis ( \> 50% glomeruli affected)
34
what are the most common causes of drug induced lupus?
* anti-hypertensives * anti-fungals
35
rheumatoid arthritis - pathogenesis
* **T-cell mediated immune destruction** of _bone & cartilage_, d/t possible * genetic predisposition: **HLA-DR4** \> DR1, DR10, DR14 * infectious trigger * EBV * parvovirus B19
36
rheumatoid arthritis - gross morphology
* **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
rheumatoid arthritis - microscopic morphology
* 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
identify picture, note important features
rheumatoid arthritis * **papillary synovial hyperplasia** with _lymphoid nodules:_ lymphocytes, plasma cells
39
identify the picture, note important features
rheumatoid arthritis * **synovial cell hyperplasia** * lymphocytes * plasma cells
40
rheumatoid arthritis **rheumatoid nodule** occur almost exclusively RA, ACPA seropositive pts\*
41
rheumatoid arthritis * **rheumatoid nodule, microscopic:** * **palisade of activated macrophages** surrounding a zone of **fibrinoid necrosis** occur almost exclusively in RA, ACPA seropositive pts\*
42
rheumatoid arthritis - diagnosis
* 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
sjogren's syndrome - pathogenesis
* immune destruction - **CD4+ T-cells** \> B-cells, plasma cells - of, * lacrimal glands * salivary glands
44
sjogren's syndrome - microscopic morphology
* early: **_peri_**ductal / **_per_**ivascular infiltrate * late: * extensive lymphocytic (mostly CD4+ cells) infiltrate * hyperplasia of epithelium lining ducts → duct obstruction
45
sjogren's syndrome - clinical
* **dry mouth (xerostemia)** * difficulty swallowing / tasting * crackling of mucosa * **dry eyes (keratoconjunctivitis)** * blurring / burning / itching * thick secretions * **+/- parotid gland enlargement**
46
sjogren's syndrome significantly increases risk of?
lymphoma
47
sjogren's syndrome - diagnosis
* RF - nonspecific * ANA - nonspecific * **ribonucleic acid proteins:** * **_SS-A (Ro)_** * **_SS-B (La)_** * **speckled pattern on IF**
48
swollen parotid gland ## Footnote **sjogren's syndrome**
49
identify picture, note important features
sjogren's syndrome - microscopic * intense infiltrate - **lymphocytes & plasma cells** (mostly CD4+ cells), with **residual hyperplastic ductal epithelial cells**
50
identify picture, note important features
sjogren's syndrome - IF ## Footnote **“speckled pattern” IF**
51
systemic sclerosis - pathogenesis
* combination of autoimmunity + vascular damage + sclerosis * two major types * diffuse scleroderma * limited scleroderma
52
diffuse scleroderma - clinical presentation
* skin manifestations - extensive, widespread * hardening / thickening / “shiny appearance” of skin * morphea * reynaud's * visceral manifestations - early * cardiac / pulmonary fibrosis * HTN
53
limited scleroderma - clinical presentation
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
what is CREST synrome? in what condition is it seen?
CREST syndrome = * C: calcinosis * R: Raynaud phenomenon * E: esophageal dysmotility * S: scleroderma * T: Telangiectasi **seen in limited scleroderma**
55
skin manifestations of scleroderma - microscopic morphology
* extensive deposition of **dense collagen** **in the dermis** * **loss of adnexa** (hair follicles)
56
systemic sclerosis - diagnosis
* two ANAs: * **DNA-topoisomerase I** * **Anti-centromere antibody** in *limited systemic sclerosis* (crest syndrome)
57
identify the picture, note important features
shiny, hardened / thickened skin **systemic sclerosis/scleroderma** - both diffuse & limited
58
identify picture, note important features
reynaud's syndrome - episodic vasoconstriction of arteries after exposure to cold systemic sclerosis (scleroderma)
59
identify picture, note important features
morphea - pale sclerotic plaque surrounded by a violaceous peripheral rim systemic sclerosis (scleroderma)
60
identify picture, note important features
auto-imputation systemic sclerosis (scleroderma)
61
limited scleroderma (CREST syndrome) IF ## Footnote **anti-centromere IF**
62
mixed connective tissue disease is characterized by?
* 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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