Systemic Sclerosis and Sjogren's Flashcards
limited scleroderma affects
extremities + head
classifications of scleroderma
morphea
limited scleroderma
diffuse scleroderma
diffuse scleroderma affects
limited + trunk
early skin changes in scleroderma
swelling of fingers/hands–>erythema, pruritis
later skin changes in scleroderma
shiny, tight, thick pigment changes (hypo and hyperpigmented areas), sclerodactyly, joint contractures, digital ulcers, pitting of fingertips
scleroderma sine sclerosis
dx later in life
raynaud, GI issues, autoabs, telangiectasias
limited scleroderma aka
CREST
calcinosis, raynaud’s, esophageal dysmotility, sclerodactyly, telengectasia
calcinosis is
only seen in limited (not diffuse)
dilated nailfod capilaries important..
to tell primary raynaud’s from secondary which helps predict whether the patient will develop CT abnormalities or not
sclerodactyly
resorption of tufts of terminal phalanges
diffuse scelroderma involves (4)
early organ involvment
renal crisis
pulmonary fibrosis
topo 1 (scl-70)
limited scleroderma
CREST
calcinosis
pulmonary HTN
centromere
4 changes in pathophys of scleroderma
fibroblasts–>tissue fibrosis
endothelial cells–>vasculopathy
b cells–>produce autoabs
t cells–>cytokine produx
characteristics of diffuse scleroderma
vasculopathy autoimmunity fibrosis pulmonary ILD GI** MSK Skeletal Muscle
how does vasculopathy happen
not a product of inflammation
actually oblierate vessels via thickeninging of intima and dibrosis of adventitia