Systemic Sclerosis Flashcards

1
Q

what are the three classic findings of systemic sclerosis?

A

autoimmunity
vascular damage
tissue fibrosis

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2
Q

name the three subtypes of systemic sclerosis

A

limited cutaneous
diffuse cutaneous
systemic cutaneous sine scleroderma

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3
Q

what is systemic sclerosis sine scleroderma?

A

skin spared

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4
Q

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

A

CREST

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5
Q

what is the C in crest?

A

calcinosis

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6
Q

what is the R in crest?

A

raynauds

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7
Q

what is the E in crest?

A

esophageal dysfxn

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8
Q

what is the S in crest?

A

sclerodactyly

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9
Q

what is the T in crest?

A

telangiectasias

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10
Q

what two abs are found in diffuse cutaneous systemic sclerosis?

A

anti scl 70

anti RNA poly 1 and III

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11
Q

what ab is positive in limited cutaneous systemic sclerosis?

A

anti centromere

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12
Q

what are the four most common causes of raynauds?

A

SLE
systemic sclerosis
sjogrens
dermatomyositis

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13
Q

name three drug classes you can give for raynauds?

A

ca channel blockers
nitrates
PDE inhibitors

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14
Q

what does calcinosis look like?

A

white deposits on exteremities

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15
Q

what is leading cause of death in systemic sclerosis?

A

pulmonary fibrosis

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16
Q

what type of lung disease occurs in systemic sclerosis?

A

usually interstitial lung disease

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17
Q

what type of systemic sclerosis has pulmonary fibrosis more often?

A

diffuse cutaneous

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18
Q

who has more scleroderma renal crises?

A

people with diffuse cutaneous systemic sclerosis

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19
Q

explain what happens in scleroderma renal crisis

A

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

20
Q

what is a risk factor for scleroderma renal crisis?

A

previous high dose roids

21
Q

what is mainstay of rx for renal crisis with scleroderma?

A

ACEs

22
Q

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

A

topoisomerase I (scl-70)

23
Q

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

A

RNA polymerase III

24
Q

what meds should be avoided in systemic sclerosis? why?

A

avoid steroids because of risk of renal crisis

25
Q

what two drugs are often used insystemic sclerosis?

A

mycophenolate

cyclophosphamide

26
Q

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

A

trouble with solid or liquid food, chronic heartburn

27
Q

what happens in esophagus in systemic sclerosis that causes issue?

A

collagen replaces muscle…this happens throughout the GI tract

28
Q

what happens to LES pressure in systemic sclerosis?

A

decreases…reflux

29
Q

what happens to peristalsis insystemic sclerosis?

A

decreases

30
Q

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

A

esophagitis

31
Q

what can happen in stomach with systemic sclerosis?

A

gastroparesis

32
Q

what are symptoms of gastroparesis in systemic sclerosis?

A

N/V

33
Q

what can happen in SI with systemic sclerosis?

A

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

34
Q

what is GAVE?

A

gastric antral vascular ectasia

35
Q

what are the symptoms of GAVE in systemic sclerosis?

A

iron deficiency anemia heavy GI bleeding

36
Q

limited cutaneous systemic sclerosis is limited to where?

A

hands arms and face

37
Q

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

A

ILD and PHTN

38
Q

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

A

both decrease…but ratio stays about the same

39
Q

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

A

low

40
Q

what are symptoms of ILD in systemic sclerosis?

A

fatigue, exertional dyspnea and dry cough

41
Q

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

A

bi basilar fine inspiratory crackles

42
Q

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

A

PHTN

43
Q

what is mean pressure cut off for PHTN?

A

25mmHg

44
Q

how does scleroderma cause PHTN?

A

destruction of pulmonary vasculature from progressive fibrosis and inducing vascular remodeling

45
Q

how are PFTs in PHTN?

A

should be about normal

46
Q

what is DCLO in PHTN?

A

declining