Systemic Sclerosis

Question 1

what are the three classic findings of systemic sclerosis?

Answer 1

autoimmunity
vascular damage
tissue fibrosis

Question 2

name the three subtypes of systemic sclerosis

Answer 2

limited cutaneous
diffuse cutaneous
systemic cutaneous sine scleroderma

Question 3

what is systemic sclerosis sine scleroderma?

Answer 3

skin spared

Question 4

what is the mnemonic to remember sx of limited diffuse systemic sclerosis?

Answer 4

CREST

Question 5

what is the C in crest?

Answer 5

calcinosis

Question 6

what is the R in crest?

Answer 6

raynauds

Question 7

what is the E in crest?

Answer 7

esophageal dysfxn

Question 8

what is the S in crest?

Answer 8

sclerodactyly

Question 9

what is the T in crest?

Answer 9

telangiectasias

Question 10

what two abs are found in diffuse cutaneous systemic sclerosis?

Answer 10

anti scl 70

anti RNA poly 1 and III

Question 11

what ab is positive in limited cutaneous systemic sclerosis?

Answer 11

anti centromere

Question 12

what are the four most common causes of raynauds?

Answer 12

SLE
systemic sclerosis
sjogrens
dermatomyositis

Question 13

name three drug classes you can give for raynauds?

Answer 13

ca channel blockers
nitrates
PDE inhibitors

Question 14

what does calcinosis look like?

Answer 14

white deposits on exteremities

Question 15

what is leading cause of death in systemic sclerosis?

Answer 15

pulmonary fibrosis

Question 16

what type of lung disease occurs in systemic sclerosis?

Answer 16

usually interstitial lung disease

Question 17

what type of systemic sclerosis has pulmonary fibrosis more often?

Answer 17

diffuse cutaneous

Question 18

who has more scleroderma renal crises?

Answer 18

people with diffuse cutaneous systemic sclerosis

Question 19

explain what happens in scleroderma renal crisis

Answer 19

malignant hypertension…high renin state…MAHA, thrombocytopenia, proteinuria

Question 20

what is a risk factor for scleroderma renal crisis?

Answer 20

previous high dose roids

Question 21

what is mainstay of rx for renal crisis with scleroderma?

Answer 21

ACEs

Question 22

what antibody is heavily associated with ILD and diffuse systemic sclerosis?

Answer 22

topoisomerase I (scl-70)

Question 23

what antibody is associated with renal crisis and diffuse cutaneous systemic sclerosis?

Answer 23

RNA polymerase III

Question 24

what meds should be avoided in systemic sclerosis? why?

Answer 24

avoid steroids because of risk of renal crisis

Question 25

what two drugs are often used insystemic sclerosis?

Answer 25

mycophenolate | cyclophosphamide

Question 26

what are the main symptoms of esphageal dysmotility in systemic sclerosis?

Answer 26

trouble with solid or liquid food, chronic heartburn

Question 27

what happens in esophagus in systemic sclerosis that causes issue?

Answer 27

collagen replaces muscle...this happens throughout the GI tract

Question 28

what happens to LES pressure in systemic sclerosis?

Answer 28

decreases...reflux

Question 29

what happens to peristalsis insystemic sclerosis?

Answer 29

decreases

Question 30

what is there a risk for since increase reflux and decrease peristalsis in systemic sclerosis?

Answer 30

esophagitis

Question 31

what can happen in stomach with systemic sclerosis?

Answer 31

gastroparesis

Question 32

what are symptoms of gastroparesis in systemic sclerosis?

Answer 32

N/V

Question 33

what can happen in SI with systemic sclerosis?

Answer 33

impaired motility so you get bloating and nausea and bacteria can overgrow

Question 34

what is GAVE?

Answer 34

gastric antral vascular ectasia

Question 35

what are the symptoms of GAVE in systemic sclerosis?

Answer 35

iron deficiency anemia heavy GI bleeding

Question 36

limited cutaneous systemic sclerosis is limited to where?

Answer 36

hands arms and face

Question 37

what are the two most common lung issues in systemic sclerosis?

Answer 37

ILD and PHTN

Question 38

with ILD in systemic sclerosis, what is seen with FVC and FEV1?

Answer 38

both decrease...but ratio stays about the same

Question 39

will the DLCO be high low or normal in ILD from systemic sclerosis?

Answer 39

low

Question 40

what are symptoms of ILD in systemic sclerosis?

Answer 40

fatigue, exertional dyspnea and dry cough

Question 41

what can be heard on ascultation in ILD in systemic sclerosis?

Answer 41

bi basilar fine inspiratory crackles

Question 42

anti centromere antibodies are more of a predictor for which lung finding in systemic sclerosis?

Answer 42

PHTN

Question 43

what is mean pressure cut off for PHTN?

Answer 43

25mmHg

Question 44

how does scleroderma cause PHTN?

Answer 44

destruction of pulmonary vasculature from progressive fibrosis and inducing vascular remodeling

Question 45

how are PFTs in PHTN?

Answer 45

should be about normal

Question 46

what is DCLO in PHTN?

Answer 46

declining