#42 Scleroderma

Term
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Scleroderma is a disorder of ____, skleros means ____
it has an unknown etiology
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Terms in this set (25)
Scleroderma is a disorder of ____, skleros means ____
it has an unknown etiology
connective tissue
hard
Limited cutaneous systemic sclerosis is also called ___
CREST
Diffuse systemic cutaneous sclerosis: is it (slow or quick) progression, involves tightening of the skin and ____
with a charasteric skin appearance of ____
quick
visceral organs
poikeloderma
Image: Diffuse systemic cutaneous sclerosis: is it (slow or quick) progression, involves tightening of the skin and ____ with a charasteric skin appearance of ____
Limited cutaneous systemic sclerosis: is it a (slow or quick) progression, involved skin in ___ locations
slow
fingers
CREST stands for
C:____
R: ____
E: ____
S: ____
T: ____
calcinosis
raynaud's phenomenon
esophageal dysmotilits
sclerodactyly
telengectasias
calcinosis is ___
calcium deposit in the skin
Image: calcinosis is ___
raynaud's phenomenon is 3 color change: ____ then __ then ___, it is a ___ reaction
white then blue then red
vasospasm
Image: raynaud's phenomenon is 3 color change: ____ then __ then ___, it is a ___ reaction
vasospasm is caused by ____ factor in scleroderma which leads to vascular damage
granzyme A
esophageal dysmotilits loses ____ function
normal peristaltic function
Image: esophageal dysmotilits loses ____ function
sclerodactyly is skin ___
tightness
Image: sclerodactyly is skin ___
telengectasias are ____dilated peripheral blood vesselsmost likely other diseases associated with scleroderma ___ and ___polymyositis Lupusscleroderma epidemology most likely in: age and gender, ethnicitywomen of childbearing age native americansScleroderma is associated with ___ genes, may have a link to ___ dysfunction, like it is in miceHLA D (Q7 and R5) fibrillinskin biopsy in scleroderma will have increased ___collagenWhat drug can trigger scleroderma skin changebleomycinnon-specific marker that can be seen in scleroderma ___ANAscleroderma pathogenesis has B cells and Ig in circulation that are ____, which eventually induce ___autoantibodies fibrosisin scleroderma the vascular endothelium is ___ by destructive ___ factor which causes ___ proliforationdamaged platelet derived growth factor smooth muscleendothelial cells make endothelin which increases ___ which increases the synthesis of collagen which is unwantedfibroblastsnailfold capillaroscopy in sclerodermal will showincrease in dilated blood vesselsmost important sclerodermal vascular injury which is an emergency: ___, which leads to high ___ and reduced ___sclerodermal renal crisis blood pressure output of urinethe most important cytokine mediating transcriptional changes that lead to collagen over-synthesis is: ___ in sclerodermatransforming growth factor betaskin histology in scleroderma: thickened ___ and increased ___dermis collagenleading cause of death in scleroderma: ___ involvement, when you have this involvement, mean survival is 2 yearslung