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T3 - patho unit 2 > Dani - immunopathology III > Flashcards

Dani - immunopathology III Flashcards

1
Q

____: autoimmune disease characterized by vasculitis and excessive deposition of collagen throughout the body, mainly in the skin ending in fibrosis and atrophy

A

scleroderma

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2
Q

scleroderma aka ___ ___ __

A

progressive systemic sclerosis

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3
Q

incidence of scleroderma

females to males

A

3:1

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4
Q

ages of scleroderma

__-__

A

30-50

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5
Q

PDGF =

A

platelet-derived growth factor

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6
Q

FGF =

A

fibroblast growth factor

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7
Q

2 passible mechanisms leading to systemic sclerosis

A

PDGF

FGF

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8
Q

scleroderma: vasculitis
- perivascular ___ infiltration
- ___ wall
- partial ___

A

lymphocytic
thickened
occlusion

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9
Q

scleroderm

- marked __ and ___

A

fibosis

atrophy

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10
Q

scleroderma

- restricted to the ___ first, later the ____

A

skin

viscera

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11
Q

scleroderma in the skin

- starts by __, then extends ___

A

fingers

proximally

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12
Q

scleroderma in the fingers

  • tapered
  • __ ROM
  • decreased blood supply –> ___ and ___
A

decreased

ulceration; gangrene

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13
Q

scleroderma in the face

- ___ movements

A

restricted

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14
Q

scleroderma: typical “__ __” necroses and ulcerations of the fingertips

A

rat bites

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15
Q

____ face: stretched, shiny skin and loss of normal facial lines giving a younger appearance than actual age

A

masklike

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16
Q

GI tract mucosa in scleroderma:

- ___, ___ and loss of ___

A

thin
ulcers
villi

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17
Q

GI tract submucosa in scleroderma: ___

A

collagenized

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18
Q

GI tract muscle layer in scleroderma:

- ___, replaced by ___, and deficient ___

A

atrophies
collagen
peristalsis

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19
Q

esophagus in scleroderma:

- in 50%, ___ due to narrowing of lower portion (___ ___ appearance)

A

dysphagia

rubber hose

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20
Q

intestines in scleroderma: ____

A

malabsorption

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21
Q

joints in scleroderma

- ___: proliferation of synovial membrane, later fibrosis and less motion

A

synovitis

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22
Q

muscles in scleroderma:

- focal inflammatory ___ and ___, followed by ____ and ____

A

infiltrates; edema

fibrosis; atrophy

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23
Q

kidneys in scleroderma;

- small arterioles: vasculitis, fibrosis and ischemia –> ____ ____

A

malignant hypertension

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24
Q

kidneys in scleroderma

- __ GFR and renal failure

A

decreased

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25
Q

kidneys in scleroderma

- ___ ___: depositon of immune complexes –> hypercellularity and basement membrane thickening

A

glomerular lesion

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26
Q

kidneys in scleroderma

  • diffuse interstitial ___ and ___
  • progressive ___ of the walls of small vessels
A

pneumonitis
fibrosis
thickening

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27
Q

___ disease: autoimmune disease characterized by immune-mediated destruction of salivary glands and lacrimal glands

A

sjogren’s

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28
Q

incidence of sjogren’s disease

females to males

A

9:1

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29
Q

age of sjogren’s

__-___

A

40-60

30
Q

primary sjogrens ___% and secondary __%

A

40

60

31
Q

sjogrens

- marked infiltration with ___ and ___ cells replacing the normal acinar cells

A

lymphocytes

plasma

32
Q

sjogrens

- ___ of duct epithelium –> lumen obstruction

A

hyperplasia

33
Q

dry eyes in sjogrens

  • ___ tears
  • ___ of vision
  • ___ eyes
  • corneal ___
A

decreased
blurring
itchy
ulceration

34
Q

dry mouth in sjogrens

  • ___ salivary secretion
  • ___ and ___ of oral mucosa
  • __ taste
  • ____
A

decrease
atrophy; ulceration
decreased
dysphagia

35
Q

keratoconjunctivitis sicca =

A

dry eyes

36
Q

xerostomia =

A

dry mouth

37
Q

arthritis occurs in __% of people with sjogrens

A

60

38
Q

___/____: autoimmune disease that affects primarily the skeletal muscles and skin

A

polymyositis

dermatomyositis

39
Q

incidence of polymyositis (f–>m)

A

2:1

40
Q

two peaks of polymyositis

A

5-15

50-60

41
Q

in early stage of polymyositis, muscle show

  • ____
  • ____
  • ____ change
  • ____
A

edema
lymphocytosis
fatty
necrosis

42
Q

skin in polymyositis

  • ___ with edema and cellular infiltration
  • later on, 3 more things
A

vasculitis
atrophy
fibrosis
calcification

43
Q

muscles in plymyositis

  • symmetrical ___, ___ and ___
  • stiff ___
  • ___ due to affection of pharyngeal muscles
A

weakness, pain and tenderness
fingers
dysphagia

44
Q

skin in polymyositis

  • ____ rash on malar area of face
  • ___ area of neck
  • forehead
  • shoulders
  • chest
  • ___ rash of upper eyelids
  • ____
A

butterfly
heliotrope
atophy

45
Q

visceral involvement is ___ in polymyositis

A

uncommon

46
Q

___ phenomenon occurs in 30% of patients with polymyositis

A

Raynaud’s

47
Q

increased risk of malignancy in ___ patients with polymyositis

A

adult

48
Q

___: extracellular accumulation of fibrillar proteins –> pressure atrophy of adjacent parenchyma

A

amyloidosis

49
Q

etiology of amyloidosis

- ___ production or __ utilization

A

increase

decrease

50
Q

____ amyloidosis

- associated with multiple myeloma, the most common form in the US

A

primary amyloidosis

51
Q

primary amyloidosis

- malignant plasma cells synthsize excessive light ____ chains

A

immunoglobulin

52
Q

primary amyloidosis

- amyloid proteins: type ___ accumulate in the tissue

A

AL (amyloid light chain)

53
Q

secondary (reactive) amyloidosis is associated with 3 things

A

chronic inflammatory disease
autoimmune disease
neoplasms

54
Q

secondary (reactive) amyloidosis

- associated with chronic inflammatory diseases like… 5

A 
TB
leprosy
bronchiectasis
chronic osteomyelitis
chronic pyelonephritis
55
Q

secondary (reactive) amyloidosis

- associated with autoimmune diseases like… 4

A

Crohn’s
ulcerative colitis
scleroderma
RA

56
Q

secondary (reactive) amyloidosis

- associated with neoplasms like…2

A

hodgkin’s lymphoma

renal cell carcinoma

57
Q

in secondary (reactive) amyloidosis, there is systemic distribution of amyloid protein, type ___ protein which accumulates in 5 places

A 
AA
liver
spleen
thyroid
kidney
lymph nodes
58
Q

hemodialysis- related to amylodiosis

  • associated with renal failure –> affects 70% of patients maintained on hemdialysis
  • amyloid protein (___-____) accumulates mainly in joints and synovium
A

B2- microglobulin

59
Q

hereditary amyloidosis

  • present in certain ___ locations
  • amyloid protein: type ___
A

geographical

AA

60
Q

___ ____ ____: recurrent inflammation of serosal linings and fever

A

familial mediterranean fever

61
Q

2 types of localized amyloidosis

A

senile amyloidosis

amyloid in neoplasms

62
Q

senile amyloidosis

  • occurs in ___ age
  • protein accumulates in ___ and ___
A

old
heart
brain

63
Q

gross morphology of amylodosis

- the organ is ___, tissue appears ___, ___, ___ and ___ in consistency

A

enlarged

pale, waxy, gray and firm

64
Q

microscopic morphology of amyloidosis

- ___ atrophy of neighboring cells

A

pressure

65
Q

liver in amyloidosis

  • amyloid deposits begins in the ____ __ ___ –> enlarge to encroach on sinusoids and liver cells
  • pressure atrophy and replacement of ____ by sheets of amyloid
A

space of disse

hepatocytes

66
Q

kidneys in amyloidosis

  • ___ in size
  • ___ in color and ___ in consistency
  • involvement of renal artery –> ___, ___ and __ in size
A 
larger
pale
firm
ischemia
atrophy
decrease
67
Q

glomeruli in amyloidosis: amyloid deposit in ___ matrix and basement membrane of capillaries –> ___ or ___ lumen

A

mesangial
narrowed
oblliterated

68
Q

renal tubules: peritubular deposit ___ the lumen

A

compressing

69
Q

renal artery and arterioles: thickened walls and narrowed lumen –> __, ___ and ___

A

ischemia, fibrosis and atrophy

70
Q

heart in amyloidosis

- subendocardial deposits –> localized __

A

elevation

71
Q

heart in amyloidosis

  • amyloid deposits in ___ layers
  • ___ of muscle fibers, ___ atrophy
  • subendocardial deposits affect the conducting system –> ___
A

all
separation
pressure
arrhythmias

72
Q

clinical features of amyloidosis
generally
- weight ___
- _____ and ____

A

loss

weakness and fatigue

T3 - patho unit 2 (8 decks)

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