Systemic Sclerosis Flashcards
what are the three classic findings of systemic sclerosis?
autoimmunity
vascular damage
tissue fibrosis
name the three subtypes of systemic sclerosis
limited cutaneous
diffuse cutaneous
systemic cutaneous sine scleroderma
what is systemic sclerosis sine scleroderma?
skin spared
what is the mnemonic to remember sx of limited diffuse systemic sclerosis?
CREST
what is the C in crest?
calcinosis
what is the R in crest?
raynauds
what is the E in crest?
esophageal dysfxn
what is the S in crest?
sclerodactyly
what is the T in crest?
telangiectasias
what two abs are found in diffuse cutaneous systemic sclerosis?
anti scl 70
anti RNA poly 1 and III
what ab is positive in limited cutaneous systemic sclerosis?
anti centromere
what are the four most common causes of raynauds?
SLE
systemic sclerosis
sjogrens
dermatomyositis
name three drug classes you can give for raynauds?
ca channel blockers
nitrates
PDE inhibitors
what does calcinosis look like?
white deposits on exteremities
what is leading cause of death in systemic sclerosis?
pulmonary fibrosis
what type of lung disease occurs in systemic sclerosis?
usually interstitial lung disease
what type of systemic sclerosis has pulmonary fibrosis more often?
diffuse cutaneous
who has more scleroderma renal crises?
people with diffuse cutaneous systemic sclerosis
explain what happens in scleroderma renal crisis
malignant hypertension…high renin state…MAHA, thrombocytopenia, proteinuria
what is a risk factor for scleroderma renal crisis?
previous high dose roids
what is mainstay of rx for renal crisis with scleroderma?
ACEs
what antibody is heavily associated with ILD and diffuse systemic sclerosis?
topoisomerase I (scl-70)
what antibody is associated with renal crisis and diffuse cutaneous systemic sclerosis?
RNA polymerase III
what meds should be avoided in systemic sclerosis? why?
avoid steroids because of risk of renal crisis
what two drugs are often used insystemic sclerosis?
mycophenolate
cyclophosphamide
what are the main symptoms of esphageal dysmotility in systemic sclerosis?
trouble with solid or liquid food, chronic heartburn
what happens in esophagus in systemic sclerosis that causes issue?
collagen replaces muscle…this happens throughout the GI tract
what happens to LES pressure in systemic sclerosis?
decreases…reflux
what happens to peristalsis insystemic sclerosis?
decreases
what is there a risk for since increase reflux and decrease peristalsis in systemic sclerosis?
esophagitis
what can happen in stomach with systemic sclerosis?
gastroparesis
what are symptoms of gastroparesis in systemic sclerosis?
N/V
what can happen in SI with systemic sclerosis?
impaired motility so you get bloating and nausea and bacteria can overgrow
what is GAVE?
gastric antral vascular ectasia
what are the symptoms of GAVE in systemic sclerosis?
iron deficiency anemia heavy GI bleeding
limited cutaneous systemic sclerosis is limited to where?
hands arms and face
what are the two most common lung issues in systemic sclerosis?
ILD and PHTN
with ILD in systemic sclerosis, what is seen with FVC and FEV1?
both decrease…but ratio stays about the same
will the DLCO be high low or normal in ILD from systemic sclerosis?
low
what are symptoms of ILD in systemic sclerosis?
fatigue, exertional dyspnea and dry cough
what can be heard on ascultation in ILD in systemic sclerosis?
bi basilar fine inspiratory crackles
anti centromere antibodies are more of a predictor for which lung finding in systemic sclerosis?
PHTN
what is mean pressure cut off for PHTN?
25mmHg
how does scleroderma cause PHTN?
destruction of pulmonary vasculature from progressive fibrosis and inducing vascular remodeling
how are PFTs in PHTN?
should be about normal
what is DCLO in PHTN?
declining