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
kidneys in scleroderma | - ___ ___: depositon of immune complexes --> hypercellularity and basement membrane thickening
glomerular lesion
26
kidneys in scleroderma - diffuse interstitial ___ and ___ - progressive ___ of the walls of small vessels
pneumonitis fibrosis thickening
27
___ disease: autoimmune disease characterized by immune-mediated destruction of salivary glands and lacrimal glands
sjogren's
28
incidence of sjogren's disease | females to males
9:1
29
age of sjogren's | __-___
40-60
30
primary sjogrens ___% and secondary __%
40 | 60
31
sjogrens | - marked infiltration with ___ and ___ cells replacing the normal acinar cells
lymphocytes | plasma
32
sjogrens | - ___ of duct epithelium --> lumen obstruction
hyperplasia
33
dry eyes in sjogrens - ___ tears - ___ of vision - ___ eyes - corneal ___
decreased blurring itchy ulceration
34
dry mouth in sjogrens - ___ salivary secretion - ___ and ___ of oral mucosa - __ taste - ____
decrease atrophy; ulceration decreased dysphagia
35
keratoconjunctivitis sicca =
dry eyes
36
xerostomia =
dry mouth
37
arthritis occurs in __% of people with sjogrens
60
38
___/____: autoimmune disease that affects primarily the skeletal muscles and skin
polymyositis | dermatomyositis
39
incidence of polymyositis (f-->m)
2:1
40
two peaks of polymyositis
5-15 | 50-60
41
in early stage of polymyositis, muscle show - ____ - ____ - ____ change - ____
edema lymphocytosis fatty necrosis
42
skin in polymyositis - ___ with edema and cellular infiltration - later on, 3 more things
vasculitis atrophy fibrosis calcification
43
muscles in plymyositis - symmetrical ___, ___ and ___ - stiff ___ - ___ due to affection of pharyngeal muscles
weakness, pain and tenderness fingers dysphagia
44
skin in polymyositis - ____ rash on malar area of face - ___ area of neck - forehead - shoulders - chest - ___ rash of upper eyelids - ____
butterfly heliotrope atophy
45
visceral involvement is ___ in polymyositis
uncommon
46
___ phenomenon occurs in 30% of patients with polymyositis
Raynaud's
47
increased risk of malignancy in ___ patients with polymyositis
adult
48
___: extracellular accumulation of fibrillar proteins --> pressure atrophy of adjacent parenchyma
amyloidosis
49
etiology of amyloidosis | - ___ production or __ utilization
increase | decrease
50
____ amyloidosis | - associated with multiple myeloma, the most common form in the US
primary amyloidosis
51
primary amyloidosis | - malignant plasma cells synthsize excessive light ____ chains
immunoglobulin
52
primary amyloidosis | - amyloid proteins: type ___ accumulate in the tissue
AL (amyloid light chain)
53
secondary (reactive) amyloidosis is associated with 3 things
chronic inflammatory disease autoimmune disease neoplasms
54
secondary (reactive) amyloidosis | - associated with chronic inflammatory diseases like... 5
``` TB leprosy bronchiectasis chronic osteomyelitis chronic pyelonephritis ```
55
secondary (reactive) amyloidosis | - associated with autoimmune diseases like... 4
Crohn's ulcerative colitis scleroderma RA
56
secondary (reactive) amyloidosis | - associated with neoplasms like...2
hodgkin's lymphoma | renal cell carcinoma
57
in secondary (reactive) amyloidosis, there is systemic distribution of amyloid protein, type ___ protein which accumulates in 5 places
``` AA liver spleen thyroid kidney lymph nodes ```
58
hemodialysis- related to amylodiosis - associated with renal failure --> affects 70% of patients maintained on hemdialysis - amyloid protein (___-____) accumulates mainly in joints and synovium
B2- microglobulin
59
hereditary amyloidosis - present in certain ___ locations - amyloid protein: type ___
geographical | AA
60
___ ____ ____: recurrent inflammation of serosal linings and fever
familial mediterranean fever
61
2 types of localized amyloidosis
senile amyloidosis | amyloid in neoplasms
62
senile amyloidosis - occurs in ___ age - protein accumulates in ___ and ___
old heart brain
63
gross morphology of amylodosis | - the organ is ___, tissue appears ___, ___, ___ and ___ in consistency
enlarged | pale, waxy, gray and firm
64
microscopic morphology of amyloidosis | - ___ atrophy of neighboring cells
pressure
65
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
space of disse | hepatocytes
66
kidneys in amyloidosis - ___ in size - ___ in color and ___ in consistency - involvement of renal artery --> ___, ___ and __ in size
``` larger pale firm ischemia atrophy decrease ```
67
glomeruli in amyloidosis: amyloid deposit in ___ matrix and basement membrane of capillaries --> ___ or ___ lumen
mesangial narrowed oblliterated
68
renal tubules: peritubular deposit ___ the lumen
compressing
69
renal artery and arterioles: thickened walls and narrowed lumen --> __, ___ and ___
ischemia, fibrosis and atrophy
70
heart in amyloidosis | - subendocardial deposits --> localized __
elevation
71
heart in amyloidosis - amyloid deposits in ___ layers - ___ of muscle fibers, ___ atrophy - subendocardial deposits affect the conducting system --> ___
all separation pressure arrhythmias
72
clinical features of amyloidosis generally - weight ___ - _____ and ____
loss | weakness and fatigue