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Ortho-Rheum > Scleroderma > Flashcards

Scleroderma Flashcards

1
Q

how is scleroderma characterized? (3)

A

skin fibrosis
vascular damage
internal organ dysfunction

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2
Q

what are the 3 clinical findings of scleroderma that should be of alert?

A

raynaud’s phenomenon
ANA +
skin thickening

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3
Q

what is the most common cause of death in scleroderma?

A

cardiac involvement

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4
Q

what is the 2nd most common death in scleroderma?

A

lung disease

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5
Q

which gender is scleroderma most common in?

A

females

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6
Q

which gender has a higher mortality with scleroderma?

A

males

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7
Q

skin thickening extending to the proximal portion of the extremities/trunk

A

diffuse

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8
Q

skin thickening confined to extremities, distal to elbows and knees, and involves the face

A

limited

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9
Q

how does crest syndrome/limited scleroderma present?

A

Calcinosis on skin + digits
Raynaud’s
Esophageal motility dysfunction
Sclerodactyly of fingers
Telangiectasias on skin

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10
Q

severe and extensive thickening of the skin are at risk of what?

A

flexion contractures

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11
Q

what causes the skin thickening to occur?

A

excessive collagen deposits

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12
Q

what occurs on the face? (3)

A

thinning of lips
limited apeture
narrow nose bridge

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13
Q

thickening and tightening of the fingers and toes

A

sclerodactyly

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14
Q

how can telangiectasias be treated?

A

laser therapy

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15
Q

episodes of vasoconstriction of the digital arterial circulation in response to cold temperature or emotional stress

A

raynaud phenomenon

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16
Q

what is typically the first manifestation of scleroderma?

A

raynaud phenomenon

17
Q

what can recurrent raynaud phenomenon be associated with? (3)

A

fingertip ulcers
digital gangrene
finger loss

18
Q

what is the first line treatment for raynaud phenomenon? 2nd line if acute ischemic attacks?

A

CCB
surgery

19
Q

most common lung manifestation in scleroderma that is one of the most important determinants of morbidity and mortality

A

interstitial lung disease

20
Q

what 3 labs are indicative of interstitial lung disease?

A

Anti-U3-RNP
Scl-70
Anti-Th/To

21
Q

what 3 labs are associated with limited scleroderma/crest?

A

anticentromere
Anti-Th/To
Anti-PM Scl

22
Q

what 3 labs are associated with diffuse scleroderma?

A

Scl-70
Anti-RNA polymerase III
ANA

23
Q

what is the most common finding to suggest interstitial lung disease?

A

fine inspiratory crackles

24
Q

what are some tests to order to asses interstitial lung disease? (2)

A

PFT + DLCO
chest CT

25
Q

how will a PFT present in a patient with interstitial lung disease?

A

restrictive pattern

26
Q

what will be seen on chest CT of progressed interstitial lung disease? (2)

A

ground glass opacities
reticular fibrosis

27
Q

a potentially life-threatening manifestation that manifests later in the disease of scleroderma

A

pulmonary arterial hypertension

28
Q

a patient presents with dyspnea on exertion, fatigue, hypotension, and lower edema. what could they be experiencing?

A

pulmonary arterial hypertension

29
Q

in which type of scleroderma can GI involvement occur?

A

diffuse + limited

30
Q

what should be done/taken for GI involvement in scleroderma? (3)

A

avoid late night meals
H2 blockers
PPIs

31
Q

in which type of scleroderma can renal involvement occur?

A

diffuse

32
Q

what should be ordered to confirm renal involvement in scleroderma?

A

renal biopsy

33
Q

what can treat renal involvement in scleroderma?

A

ACE inhibitors

34
Q

what should be avoided in a patient with scleroderma has renal involvement?

A

high doses of steroids

35
Q

what can be used to treat MSK involvement in scleroderma? (2)

A

NSAIDS/low dose steroids
occupational therapy

36
Q

what can be used to treat MSK involvement in scleroderma if the patient has uncontrolled inflammatory joint disease?

A

methotrexate

37
Q

what 2 systems are most affected by scleroderma and thus must be monitored?

A

heart
lungs

Ortho-Rheum (27 decks)

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