Scleroderma Flashcards
thick and indurated skin is the hallmark of
systemic sclerosis
extensive skin induration starting in the fingers and ascending from distal to proximal limbs and the trunk
diffuse cutaneous SSc (dcSSc)
skin involvement remains confined to the finger, distal limbs and face, while trunk is spared
limited cutaneous SSc (lcSSc)
raynaud’s phenomenon + clinical and laboratory features of SSc; absent detectable skin thickening
Ssc sine scleroderma
3 manifestations of SSc
diffuse microangipathy
inflammation and autoimmunity
visceral and vascular fibrosis in multiple organs
early and possibly primary pathogenic event in SSc that leads to protean clinical manifestations of small vessels vasculopathy
vascular injury
cytokines that induce production of TGB & promote collagen synthesis
Th2 cytokines
cytokines that inhibits collagen synthesis & blocks cytokine-mediated fibroblast activation
Th1 cytokines (IFNy)
sclerodactyly appearance
mauskopf appearance
most common lung involvement in SSc
nonspecific interstitial pneumonia (NSIP)
stricture formation in GIT of SSc
Barrett’s metaplasia
common GIT involvement in SSc
reflux disease
common heart involvement in SSc
subclinical cardiac pathology
which part of the heart is fibrosis common
SA node
localized scleroderma in more common in
children
