Scleroderma

Question 1

how is scleroderma characterized? (3)

Answer 1

skin fibrosis
vascular damage
internal organ dysfunction

Question 2

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

Answer 2

raynaud’s phenomenon
ANA +
skin thickening

Question 3

what is the most common cause of death in scleroderma?

Answer 3

cardiac involvement

Question 4

what is the 2nd most common death in scleroderma?

Answer 4

lung disease

Question 5

which gender is scleroderma most common in?

Answer 5

females

Question 6

which gender has a higher mortality with scleroderma?

Answer 6

males

Question 7

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

Answer 7

diffuse

Question 8

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

Answer 8

limited

Question 9

how does crest syndrome/limited scleroderma present?

Answer 9

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

Question 10

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

Answer 10

flexion contractures

Question 11

what causes the skin thickening to occur?

Answer 11

excessive collagen deposits

Question 12

what occurs on the face? (3)

Answer 12

thinning of lips
limited apeture
narrow nose bridge

Question 13

thickening and tightening of the fingers and toes

Answer 13

sclerodactyly

Question 14

how can telangiectasias be treated?

Answer 14

laser therapy

Question 15

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

Answer 15

raynaud phenomenon

Question 16

what is typically the first manifestation of scleroderma?

Answer 16

raynaud phenomenon

Question 17

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

Answer 17

fingertip ulcers
digital gangrene
finger loss

Question 18

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

Answer 18

CCB
surgery

Question 19

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

Answer 19

interstitial lung disease

Question 20

what 3 labs are indicative of interstitial lung disease?

Answer 20

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

Question 21

what 3 labs are associated with limited scleroderma/crest?

Answer 21

anticentromere
Anti-Th/To
Anti-PM Scl

Question 22

what 3 labs are associated with diffuse scleroderma?

Answer 22

Scl-70
Anti-RNA polymerase III
ANA

Question 23

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

Answer 23

fine inspiratory crackles

Question 24

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

Answer 24

PFT + DLCO
chest CT

Question 25

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

Answer 25

restrictive pattern

Question 26

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

Answer 26

ground glass opacities reticular fibrosis

Question 27

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

Answer 27

pulmonary arterial hypertension

Question 28

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

Answer 28

pulmonary arterial hypertension

Question 29

in which type of scleroderma can GI involvement occur?

Answer 29

diffuse + limited

Question 30

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

Answer 30

avoid late night meals H2 blockers PPIs

Question 31

in which type of scleroderma can renal involvement occur?

Answer 31

diffuse

Question 32

what should be ordered to confirm renal involvement in scleroderma?

Answer 32

renal biopsy

Question 33

what can treat renal involvement in scleroderma?

Answer 33

ACE inhibitors

Question 34

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

Answer 34

high doses of steroids

Question 35

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

Answer 35

NSAIDS/low dose steroids occupational therapy

Question 36

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

Answer 36

methotrexate

Question 37

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

Answer 37

heart lungs