Developmental and Immune-mediated Mucocutaneous Conditions - part IV

Question 1

T/F: systemic sclerosis is common

Answer 1

false, rare

Question 2

systemic sclerosis

Answer 2

characterized by inappropriate deposition of dense collagen

Question 3

etiology of systemic sclerosis

Answer 3

probably immunologically mediated

Question 4

systemic sclerosis is also termed what?

Answer 4

scleroderma

sclero = hard
derma = skin

Question 5

clinical features of systemic sclerosis

Answer 5

1. diffuse, hard, smooth texture of skin
2. Raynaud phenomenon
3. sclerodactyly
4. acro-osteolysis ± ulceration
5. “mask-like” face
6. atrophy of alae
7. mouse facies
8. microstomia “purse-string” appearance
9. dysphagia with esophageal involvement
10. pulmonary, renal, cardiac and GI fibrosis may be seen, with pulmonary hypertension

Question 6

sclerodactyly

Answer 6

claw-like deformity

Question 7

acro-osteolysis

Answer 7

resorption terminal phalanges…

destruction of digit tips, including bone

Question 8

mouse facies

Answer 8

pinched appearance of nose

Question 9

which sex is 3x more frequently affected by systemic sclerosis?

Answer 9

women 3x more than men

Question 10

Raynaud’s phenomenon

Answer 10

discoloraton of fingers and/or toes after exposure to changes in temperature or emotional events causing spasm of blood vessels

Question 11

T/F: Raynaud’s phenomenon is specific for systemic sclerosis

Answer 11

false, non specific

Question 12

white discoloration of fingers and/or toes due to Raynaud’s phenomenon is caused by what?

Answer 12

spasm of blood vessels causes diminished blood supply

Question 13

blue discoloration of fingers and/or toes due to Raynaud’s phenomenon is caused by what?

Answer 13

due to lack of oxygen

Question 14

red discoloration of fingers and/or toes due to Raynaud’s phenomenon is caused by what?

Answer 14

as blood rushes back in

Question 15

sclerodactyly is due to what?

Answer 15

collagen deposition, ulceration of fingertips

Question 16

radiographic features of systemic sclerosis

Answer 16

1. diffuse widening of periodontal ligament
2. resorption of posterior ramus, condyle, coronoid process or chin
3. may see root resorption

Question 17

resorption of posterior ramus, condyle, coronoid process or chin is seen in what percent of systemic sclerosis pts?

Answer 17

10-20%

Question 18

histo features of systemic sclerosis

Answer 18

diffuse deposition of collagen throughout the superficial CT

Question 19

what is suggestive in dx’ing systemic sclerosis?

Answer 19

generally stiffened skin with development of Raynaud’s phenomenon suggestive of dx

Question 20

how is systemic sclerosis dx’ed?

Answer 20

1. skin biopsy
2. lab studies
3. serologic studies

Question 21

serologic studies show that pts with systemic sclerosis have autoantibodies directed against what?

Answer 21

Scl-70

Question 22

serologic studies show what for pts with limited cutaneous systemic sclerosis?

Answer 22

anticentromere antibodies

Question 23

T/F: tx for systemic sclerosis is difficult and mainly symptomatic

Answer 23

true

Question 24

several drugs are used to do what in tx’ing symptoms of Raynaud’s phenomenon?

Answer 24

1. used to inhibit collagen production

2. increase peripheral blood flow

Question 25

T/F: steroids are indicated for tx'ing Raynaud's phenomenon

Answer 25

false, doesn't seem to help

Question 26

what is used to tx hypertension especially renal in pts with systemic sclerosis?

Answer 26

ACE inhibitors

Question 27

tx for systemic sclerosis

Answer 27

1. esophageal dilatation 2. oral hygiene instruction 3. fabricate prostheses with design to accommodate microstomia

Question 28

death from systemic sclerosis is due to what?

Answer 28

internal organ deposition of collagen

Question 29

T/F: cardiac involvement in pts with systemic sclerosis has a worse prognosis

Answer 29

true

Question 30

most deaths from systemic sclerosis is due to what involvement?

Answer 30

pulmonary

Question 31

what is the 10-year survival rate for limited cutaneous involvement in systemic sclerosis pts?

Answer 31

75-80%

Question 32

what is the 10-year survival rate for diffuse cutaneous involvement in systemic sclerosis pts?

Answer 32

55-60%

Question 33

CREST syndrome

Answer 33

``` Calcinosis cutis Raynaud's phenomenon Esophageal dysfunction Sclerodactyly Telangiectasia ```

Question 34

what is a milder variant of systemic sclerosis?

Answer 34

CREST syndrome

Question 35

who is affected by CREST syndrome?

Answer 35

women, 6-7th decade

Question 36

calcinotis cutis

Answer 36

subcutaneous nodules are deposition of calcium salts

Question 37

tx of CREST syndrome if symptomatic

Answer 37

similar to systemic sclerosis

Question 38

T/F: px of CREST syndrome is worse than systemic sclerosis

Answer 38

false, better than systemic sclerosis

Question 39

what is the 6-year survival rate for pts with CREST syndrome?

Answer 39

80%

Question 40

what is the 12-year survival rate for pts with CREST syndrome?

Answer 40

50%